Comprehensive Neurology Board Review Flash Cards

Colen Publishing

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Colen Publishing Colen Publishing, L.L.C. PO Box 36536 Grosse Pointe Woods, MI 48236 Author and Editor: Chaim B. Colen, M.D., Ph.D. Editorial Assistant: Roxanne E. Colen, PA PA-C C COPYRIGHT © 2008 by Colen Publishing, L.L.C. This book, including all parts thereof, is legally protected by copyright. Any use, exploitation, or commercialization outside the narrow limits set by copyright legislation without the author’s consent if illegal and liable to prosecution. This applies in particular to photostat reproduction, copying, mimeographing or duplication of any kind, translating, preparation of microfilms, and electronic data processing and storage. Some of the product names, patents, and registered designs referred to in this book are in fact registered trademarks or proprietary names even though specific reference to this fact is not always made in the text. Therefore, the appearance of a name without designation as proprietary is not to be construed as a representation by the publisher that it is in the public domain. Permissions may be sought directly from Colen Publishing, L.L.C. by writing to the above address. PakPal: Printed in China 9781935345091 1935345095 Colen FlashReview: NPath 2009 ISBN 9781935345107 1935345109 Colen FlashReview: Nlgy 2009 Colen Flash-Review: Neurology 9781935345114 1935345117 Colen FlashReview: NAn 2009 Volume 1: 0-9788502-4-6 9781935345121 1935345125 Colen FlashReview: NCC 2009 Volume 2: 0-9788502-5-4 9781935345138 1935345133 Colen FlashReview: NRad 2009 9781935345145 1935345141 Colen FlashReview: NBio 2009 2 Volume Set: 0-9788502-9-7 9781935345152 193534515X Colen FlashReview: NBeh 2009 Note: Knowledge in medicine is constantly changing. The author has consulted sources believed to be reliable in the effort to provide information that is complete and in accord with the standards at the time of publication. However, in view of the possibility of human error by the author in preparation of this work, warrants that the information contained herein is in every respect accurate and complete, and that the author is not responsible for any errors or omissions or for the results obtained from use of such information. The reader is advised to confirm the information contained herein with other sources sources. This is especially important in connection with new or infrequently used drugs. drugs In such instances, instances the product information sheet included in the package with each drug should be reviewed.

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Glossary COPYRIGHT-------------------------------------------------PREFACE -----------------------------------------------------HOW TO USE THIS CARD REVIEW-------------------CONTRIBUTORS-------------------------------------------GLOSSARY--------------------------------------------------NEUROSURGERY-----------------------------------------NEUROLOGY -----------------------------------------------NEUROPATHOLOGY-------------------------------------NEUROANATOMY----------------------------------------NEUROCRITICAL CARE---------------------------------NEURORADIOLOGY--------------------------------------NEUROBIOLOGY NEUROBIOLOGY------------------------------------------BONUS BIOSTATISTICS----------------------------------

1 1 1 4 1 110 86 238 57 80 73 64 6

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Preface •

The idea to undertake such a large Flashcard review spawned from watching my wife Roxanne study for her Physician Assistant Boards. Diligently every day she would create a set of 7-10 flashcards from her studyy material that she would take with her to work. Later on,, when I was studying for my written Neurosurgery Board examination, I gleaned information from various texts and other study guides and wrote down the most relevant material on cards for quick review while at work. It was amazing how much time during the day would be available to review these cards. If there was a delay in a OR case, a long lunch-line, a traffic jam (especially the i94 on a Friday afternoon) or waiting for my wife at her OB/GYN appointment -these little cards were specially handy. Always ambitious in life, the thought of giving this study tool to the busy neurosurgery resident was captivating. My expectation is to enable the resident with a quick yet informative review of basic neuroscience principles. With positive encouragement from my fellow residents on the 1st edition, I cautiously proceed here with updating information, adding new images, improved illustrations and clarification of neuroscience concepts. May this endeavor serve to better our wonderful science inherited through the legacy of Harvey Cushing, Neurosurgery. Chaim

September 9, 2008

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The Colen Flash-Review Flash Review Author and Editor Chaim B. Colen, M.D., Ph.D. Department of Neurological Surgery Wayne State University School of Medicine Detroit, Michigan Assistant Editor Roxanne E. Colen, M.S., PA-C Colen Publishing, LLC Grosse Pointe, Michigan

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Acknowledgements I would like to give thanks to a great many wonderful persons whose efforts, although not inscribed in these cards, were instrumental in making this monumental task possible. One exceptional individual to whom I owe special thanks is my mother in-in-law, Colleen Johns, who babysat my daughter Emily and son Joshua for hours on end, end while my wife and I toiled through hundreds of pages of various textbooks and journal articles articles, formatted questions, and drew computer illustrations. To my daughter Emily Rivka, who incessantly tugged at my pants trying to get my attention to the squirrel in our backyard ;and that big bright smile from my son Joshua that continually sent me optimism. To Mahmoud and Abhi who spent hours at my home assisting with typing, researching and editing; Naomi whose positive attitude in life is exceptionally brightening and uplifted the group’s 2 am brainstorming sessions when I still had to wake up early to work the next day, all the pathologists, especially Doha, who assisted in taking photographs, Dr. William Kupsky, for allowing us access to his collection of unique neuropathology, and to all the medical students especially Kristyn, whose hard work is admirable. There are those whose names are not here but did assist in some way, thank you. I am forever indebted to my training program, the Wayne State University neurosurgery program, my Chairman Dr. Murali Guthikonda, and Associate Chairman Dr. Setti S. Rengachary whose moral support over the last five years has kept me on this educational drive. For this second edition, there were fellow residents that gave me input and new insight that has helped to improve this edition over the first. To my parents Joseph and Leila, educators of true dedicated quality, and to whom I owe my homeschooling education and self-motivation. Lastly to my wife Roxanne, whose patience with my ambitiousness biti kknows no b boundaries. d i ©™ Thank you All, Chaim September 9, 2008

How to use this Flashcard review •

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These cards are intended to cover most of the aspects of the Neurosurgery g y Board Examination. They y are not a COMPLETE review and therefore they are not intended to replace textbooks. We would advise using these cards during the last couple of weeks before your board exam except for the pathology section which you should go through all year to better remember the photographs in it ((heavilyy encountered during g the boards!). ) BOARD FAVORITE questions are of extreme importance and most likely to bump into during the boards, so make you sure you know how to answer them right. Good luck! Chaim B. B Colen, Colen M.D., M D Ph Ph.D. D ©™

Faculty Reviewers Murali Guthikonda, MD Professor and Chairman D Department t t off Neurological N l i lS Surgery Wayne State University School of Medicine Detroit, Michigan Setti Rengachary, MD Associate Chairman D Department t t off Neurological N l i lS Surgery Wayne State University School of Medicine Detroit, Michigan William, J. Kupsky, MD Department of Neuropathology W Wayne State St t University U i it School S h l off Medicine M di i Detroit, Michigan

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Forward •

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With ever increasing scope and complexity of knowledge base, the current day trainee or practitioner of neurosurgery finds it difficult to keep up with the explosion of neurosurgical information. This is compounded by a healthy growth in specialization in various branches of neurosurgery. Chaim has made an attempt to make life simpler by incorporating small quanta of knowledge on flashcards accompanied by clear and simple illustrations. The user may review as few or as many cards as his/her time will allow. Although not meant to be substitutes for standard comprehensive t t and texts d atlases, tl these th cards d help h l tto refresh f h th the information i f ti learned l d ffrom the bedside, operating room and standard books. Each card represents a mini-examination with instant access to appropriate answers. This is a fun way to recall neurosurgical information especially before an upcoming test. Setti S. Rengachary, M.D. Department of Neurological Surgery

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Physician Contributing Authors Rivka R. Colen,, MD Department of Radiology The Massachusetts General Hospital Harvard Medical School Boston, Massachusetts Doha Itani, Itani MD Department of Pathology WSU School of Medicine Detroit, Michigan

Mahmoud Rayes, MD Department of Neurological Surgery WSU School of Medicine Erika Peterson, MD UT Southwestern,, Department of Neurological Surgery Dallas, Texas

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Contributing Medical Students Darmafall, Kristyn Wayne State University School of Medicine Class of 2012

Galinato, Anthony Wayne State University School of Medicine Class of 2012

Larson, Sarah Wayne State University School of Medicine Class of 2012

Davis, Naomi Wayne State University School of Medicine Class of 2011

Gotlib, Dorothy Wayne State University School of Medicine Class of 2009

Martinez, Derek Wayne State University School of Medicine Class of 2011

Dub, Larissa Dub Wayne State University School of Medicine Class of 2012

Kozma, Bonita Kozma Wayne State University School of Medicine Class of 2008

Matthew Smith Wayne State University School of Medicine Class of 2011

Faulkiner, Rodney Wayne State University School of Medicine Class of 2012

Lai, Christopher Wayne State University School of Medicine Class of 2010

Matto, Shereen Wayne State University School of Medicine Class of 2012 ©™

Contributing Undergraduates Jeffrey P. Kallas Wayne State University Class of 2010 Abhinav Krishnan Wayne State University Class of 2010 Peter Paximadis Wayne State University Class of 2008

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Q? •

Neuropathology

This cerebellar specimen is consistent with: A. Lewy body B. Neuritic plaque C. Kuru plaque D. Hirano body

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Neuropathology Section A Neuropathology A. • •

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The correct answer is C, Kuru plaque. Pathology of variant Creutzfeldt-Jakob disease – Kuru demonstrating a “kuru plaque” in cerebellum. Accumulation of prion protein PrP in the form of amyloid plaques occurs in some forms of pprion disease. Hirano bodies (rod-shaped eosin inclusions) are seen in Alzheimer’s disease.

Kuru Plaque

PAS Liberski PP. Amyloid plaques in transmissible spongiform encephalopathies (prion diseases). Folia Neuropathol. 2004;42 Suppl B:109-19. Review.

Cerebellum: granular cell layer

Cerebellar Infection, Prion, CreutzfeldtJakob disease, disease Kuru, Kuru amyloid

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Neuropathology

This brain specimen is most consistent with: A. Lewy body B. Neuritic plaque C. Kuru plaque D. Hirano body

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A A. • •

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Neuropathology

The correct answer is D, Hirano body. Hirano bodies (rod-shaped eosin inclusions of mostly actin filaments) are seen in Alzheimer’s disease. Accumulation of prion protein PrP in the form of amyloid plaques occurs in the ppathology gy of variant CreutzfeldtJakob disease – Kuru demonstrates “kuru plaques” in the cerebellum. Lewy bodies are seen in Parkinson’s disease and diffuse Lewy body disease These are formed from αdisease. synuclein.

BOARD FAVORITE!

Hirano body

Section of hippocampus

Kuljis RO: Modular corticocerebral pathology in Alzheimer's disease. In: Mangone CA, Allegri RF, Ariza, eds. Dementia: A Multidisciplinary Approach. 1997: 143-55.

Degenerative disease, Alzheimer’s disease Hirano bodies disease,

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Q? •

Neuropathology

What is the most likely diagnosis seen in this fundoscopic examination? A. Drusen bodies B. Retinopathy of diabetes C. Retinal hemorrhage D. Pseudopapilledema

Used with permission from Handbook of Neurosurgery and Neurology in Pediatrics; By Chaim B. Colen, MD, PhD.

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A A. • • •

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Neuropathology

The correct answer is C, retinal hemorrhages. Shaken baby syndrome was described by Caffey as a clinical triad: subdural hematomas, subarachnoid hemorrhages, and retinal hemorrhages. "Shaken baby syndrome" (SBS) results in intracranial and intraocular hemorrhages with no evidence of external trauma. The cause of these injuries is vigorous shaking of an infant being held by the chest, shoulders, or extremities. Physicians are mandated to report cases of child abuse, abuse elder abuse, abuse sexual abuse abuse, domestic violence, and assault. Physicians are also required to notify the authorities if anyone has a specific plan to commit suicide or homicide. Drusen bodies aka “pseudopapilledema” are colloid or hyaline bodies lying beneath the retinal pigment epithelium. They may occur either secondary to changes in the choroid that affect the pigment epithelium or as an autosomal dominant disorder of ©™ the retinal pigment epithelium.

Martin HA, Woodson A, Christian CW, Helfaer MA, Raghupathi R, Huh JW. Shaken baby syndrome. Crit Care Nurs Clin North Am. 2006 Sep;18(3):279-86. Review.

Shaken baby syndrome syndrome, Pediatrics Pediatrics, Retinal hemorrhages, subdural hematomas, subarachnoid hemorrhages

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Neuropathology

Which pairing is most accurate for the localization of medulloblastomas in the pediatric and adult population? A. Children : Midline, Adults : Midline B. Children : Lateral, Adults : Lateral C. Children : Lateral, Adults : Midline D. Children : Midline, Adults : Lateral

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A A. •

Neuropathology

BOARD FAVORITE!

The correct answer is D, children : midline, adults: lateral

Homer-Wright rosette –(pseudorosette) radially arranged nuclei surrounding central fib ill fibrillary material t i l ((red d arrow); ) hi histopathological t th l i l features of medulloblastoma.

MRI b brain i -contrast t t enhanced h d sagittal image showing the midline location of the tumor in a pediatric patient.

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Neoplasm, p Cerebellum, PNET, medulloblastoma, location of medulloblastoma, Homer-Wright rosette

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Neuropathology

Compared to pilocytic astrocytoma: A. This tumor has a better prognosis B. This tumor has a similar prognosis. C. This tumor has a worse prognosis D. Prognosis is irrelevant

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A A. • • •

Neuropathology

BOARD FAVORITE!

The correct answer is C, this tumor has a worse prognosis.. Gemistocytic astrocytoma is WHO grade II and is distinctive in its appearance, having astrocytes t t with ith generous eosinophilic i hili cytoplasm t l with ith a ““stuffed” t ff d” stellate t ll t appearance. Low-grade astrocytomas with a significant fraction of gemistocytes progress more rapidly and typically carry a p53 mutation. Gemistocytic astrocytoma

PDGFR increase

Progenitor cell

Grade II astro

Secondary GBM

Grade III astro

Secondary GBM

P53 loss pRB loss, p oss, NK4α α increase, c ease, PTEN,, loss oss Krouwer HG, Davis RL, Silver P, Prados M. Gemistocytic astrocytomas: a reappraisal. J Neurosurg. 1991 Mar;74(3):399-406.

neoplasm, astrocytoma, gemistocytic astrocytoma, WHO grade III

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Neuropathology

This muscle specimen (Gomori stain) is consistent with ith which hi h di disorder? d ? A. Myoclonic seizures B. Disuse atrophy C. Steroid atrophy D Denervation atrophy D.

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A A. • •

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Neuropathology

The correct answer is A, myoclonic seizures. Mitochondrial disorders (e.g. (e g MELAS, MERRF) clinically are associated with seizures and muscle biopsy shows ragged-red fibers and rimmed vacuoles. Type 1 fiber atrophy- think muscle di disease! ! -myotonic t i ddystrophy t h andd congenital myopathy Type 2 muscle atrophy- think neurogenic or muscle disease! – denervation, disuse, myasthenia gravis, steroids and paraneoplastic syndrome (BOARD FAVORITE!)

RAGGED RED FIBERS

BOARD FAVORITE!

RIMMED VACUOLE

Fukuhara N, Tokiguchi S, Shirakawa K, Tsubaki T. Myoclonus epilepsy associated with ragged-red fibres (mitochondrial abnormalities ): disease entity or a syndrome? Light-and electron-microscopic studies of two cases and review of literature. J Neurol Sci. 1980 Jul;47(1):117-33.

Muscle, Mitochondrial disease, seizures, raggedred fibers, rimmed vacuoles, type 1 fiber atrophy, type 2 fiber atrophy

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Q? •

Neuropathology

This peripheral nerve biopsy is consistent with: A Myasthenia A. M asthenia gra gravis is B. Onion bulb formation C. Wallerian degeneration D. Neurofibroma

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A A. • •

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Neuropathology

The correct answer is B, onion bulb formation. Onion bulb formation occurs after repeated cycles of demyelination and remyelination result in a thick layer of abnormal myelin around the peripheral axons. These changes cause what is referred to as an onion bulb appearance. May be seen in a few disorders: Charcot-Marie-Tooth (CMT) and Refsum’s disease (phytanic acid disorder).

ONION BULBS

BOARD FAVORITE!

ONION BULB (electron microscopy)

Bornemann A, Hansen FJ, Schmalbruch H: Nerve and muscle biopsy in a case of hereditary motor and sensory neuropathy type III with basal lamina onion bulbs. Neuropathol Appl Neurobiol 1996 Feb; 22(1): 77-81.

Peripheral nerve, Onion bulb formation, myelin, Charcot-Marie-Tooth, Refsum’s disease

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Q? •

Neuropathology

Symptoms of a patient with this diagnosis might be alleviated with which hi h off the th following f ll i ddrugs: A. Retroviral drug B. Anticholinesterase inhibitor C. Beta-interferon D TrimethoprimD. Trimethoprim sulfamethoxazole

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A A. • •

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Neuropathology

The correct answer is A, anticholinesterase inhibitor. Mi Microscopy shows h an amyloid l id plaque and neurofibrillary tangles that would be consistent with the diagnosis of Alzheimer’s disease. Donepezil is a synthetic noncovalent reversible inhibitor of acetylcholinesterase (AChE) for the treatment of mild to moderate dementia associated with Alzheimer's disease. Other drugs include galantamine and rivastigmine.

A l id plaque Amyloid l

Neurofibrillary tangle

Kuljis RO: Modular corticocerebral pathology in Alzheimer's disease. In: Mangone CA, Allegri RF, Ariza, eds. Dementia: A Multidisciplinary Approach. 1997: 14355.

Degenerative disease, Dementia, Alzheimer’s disease, neurofibrillary tangles, amyloid plaque, Donepezil, Acetylcholinesterase inhibitor

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Q? •

Neuropathology

This brain specimen is most consistent with: A. Alzheimer’s disease B. Parkinson’s disease C. Pick’s disease D. Wilson’s disease

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Neuropathology

BOARD FAVORITE!

The correct answer is B, Parkinson’s disease. Microscopy shows melanophages and neuronal cell loss in the substantia nigra pars compacta. Lewy bodies are also seen. These are formed from αsynuclein. Pick bodies are seen in Pick’s disease using silver stain. NEURONAL LOSS

LEWY BODY

Hodaie M, Neimat JS, Lozano AM. The dopaminergic nigrostriatal system and Parkinson's disease: molecular events in development, disease, and cell death, and new therapeutic strategies. Neurosurgery. 2007 Jan;60(1):17-28; discussion 28-30.

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Degenerative disease, Movement disorder, Parkinson’s disease, melanophages, lewy bodies, alpha-synuclein

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Neuropathology

This neonatal brain specimen is consistent with which hemorrhage grade? A. Grade I B. Grade II C. Grade III D. Grade IV

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Neuropathology

The correct answer is C, grade III. This gross specimen demonstrates germinal matrix hemorrhage (GMH) and intraventricular hemorrhage (IVH) and hydrocephalus which are the most common and most important neurologic injuries in preterm neonates. Premature infants lack the ability to autoregulate the cerebral blood pressure. Fluctuations in cerebral bl d pressure andd flow blood fl can rupture t th the primitive i iti germinal matrix vessels or lead to infarction of the metabolically active germinal matrix. Neurologic sequelae, include cerebral palsy, mental retardation, and seizures. Grading System Grade 1 - confined GMH Grade 2 - IVH without hydrocephalus. Grade 3 - IVH with associated hydrocephalus Grade 4 - Intraparenchymal hemorrhage

BOARD FAVORITE!

IVH and hydrocephalus GMH

GRADE 3

Levy ML, Masri LS, McComb JG: Outcome for preterm infants with germinal matrix hemorrhage and progressive hydrocephalus. Neurosurgery 1997 Nov; 41(5): 1111-7; discussion 1117-8.

Vasculature, hemorrhage, hemorrhage grading, germinal matrix hemorrhage hemorrhage, intraventricular hemorrhage, Pediatrics

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Q? •

Neuropathology

This pathology is most likely due to: A. Vitamin A toxicity B. Cyanocobalamine deficiency C. Thiamine deficiency D Vitamin B6 deficiency D. E. Alcohol intake

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A A. • •

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Neuropathology

The correct answer is C, thiamine deficiency. Thiamine deficiency is seen in chronic malnourished alcoholics. Note the old infarction that has occurred in the mamillary bodies. Wernicke-Korsakoff’s syndrome y – subacute amnesia seen in damage to the mamillary bodies and may be caused by thiamine deficiency and may be reversible with the administration of thiamine.

BOARD FAVORITE!

Old infarction

©™ Torvik A. Topographic distribution and severity of brain lesions in Wernicke's encephalopathy. Clin Neuropathol. 1987 Jan-Feb;6.

Nutritional deficiency, Thiamine deficiency, Wernicke-Korsakoff syndrome, mamillary bodies

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Neuropathology

This brain specimen is most consistent with which disease? A. Wilson’s (hepatolenticular degeneration) B. Alzheimer’s C. Pick’s D Parkinson D. Parkinson’ss E. Huntington’s

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A A. • •

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Neuropathology

BOARD FAVORITE!

The correct answer is A, Wilson's disease (hepatolenticular degeneration). Wilson’ss disease is caused by accumulation Wilson of copper in the brain with decreased serum copper and ceruloplasmin and increased urine copper. Kayser-Fleischer rings form around the iris. Microscopy demonstrates Alzheimer type 2 astrocytes t t which hi h hhave a llarge clear l vesicular i l nuclei and prominent nucleoli. Proliferation of large protoplasmic astrocytes such as Opalski cells may be seen. Research suggests that these reactive astrocytes may be involved in the process of copper detoxification in WD. Bertrand E, Lewandowska E, Szpak GM, Hoogenraad T, Blaauwgers HG, Czlonkowska A, Dymecki J. Neuropathological analysis of pathological forms of astroglia in Wilson's disease. Folia Neuropathol. 2001;39(2):73-9.

Degenerative disease, Wilson’s disease, Alzheimer type 2 astrocytes, Opalski cells, Copper

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Q? •

Neuropathology

This muscle biopsy specimen is consistent with: A. Congenital myopathy B. Myotonic dystrophy C. Steroid myopathy D. Mitochondrial myopathy 1 Note: Type 1 fibers labeled (1).

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A A. • •

Neuropathology

BOARD FAVORITE!

The correct answer is C, steroid myopathy. Steroid myopathy is characterized by type 2 myofiber atrophy! TYPE 2 ATROPHY

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Type 1 fiber atrophy- think muscle disease! -myotonic dystrophy and congenital g myopathy y p y Type 2 muscle atrophy- think neurogenic or muscle disease! –denervation, disuse, myasthenia gravis, steroids and paraneoplastic syndrome (BOARD FAVORITE)

STEROID MYOPATHY

©™ Griggs RC, Mendell JR, Miller RG: Congenital myopathies. In: Evaluation and Treatment of Myopathies. Philadelphia: FA Davis Co; 1995: 211-46.

Muscle, myopathy, steroid myopathy, type 2 fiber atrophy

Q? •

Neuropathology

This gross brain specimen is most consistent with: A. Adrenoleukodystrophy B. Multiple sclerosis C. Mitochondrial myopathy D. Canavan’s disease

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Neuropathology

BOARD FAVORITE!

Periventricular The correct answer is B, multiple sclerosis. location Multiple sclerosis (MS) is an idiopathic (autoimmune?) inflammatory demyelinating disease of the CNS. Animal model experimental allergic encephalomyelitis demonstrated by autoimmunity to myelin basic proteins. (BOARD FAVORITE) Episodes of de/remyelination result in a chronic h i bburned-out d t plaque l with ith relative l ti preservation of axons and gliosis (recently axon transection has been reported in acute exacerbations). Chronic plaques Incidence is higher in Caucasians. Femaleto-male ratio is 2:1. Classic presentation -optic neuritis, Noseworthy JH, Lucchinetti C, Rodriguez M, Weinshenker BG: Multiple sclerosis. N Engl J transverse myelitis, internuclear Med 2000 Sep 28; 343(13): 938-52. ophthalmoplegia, paresthesias

Demyelinating y g disease, Inflammatory y disease, Multiple Sclerosis, myelin basic protein

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Q? •

Neuropathology

The gross spinal cord specimen (A) is most consistent with: A. Adrenoleukodystrophy B. Multiple sclerosis C. Amyotrophic lateral sclerosis D. Neurofibromatosis

A

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A A. • •

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Neuropathology

The correct answer is C, amyotrophic lateral sclerosis. Amyotrophic lateral sclerosis progressive loss of upper and lower motor neurons. Pathology: progressive loss of anterior horn cells leads to atrophy of ventral nerve fibers and of skeletal muscles; i lloss off primary i motor t neurons progressive in motor cortex leads to degeneration of corticospinal/ corticobulbar tracts. Adrenoleukodystrophy (ALD)leukodystrophy is an x-linked recessive disease due to deficiency in peroxisomal lipid oxidation.

BOARD FAVORITE!

Ventral nerve root atrophy

A

Normal ventral roots

B ©™

Gartner J, Braun A, Holzinger A , et al: Clinical and genetic aspects of X-linked adrenoleukodystrophy. Neuropediatrics 1998 Feb; 29(1): 3-13.

spinal cord, Degenerative disease, Amyotrophic lateral sclerosis, Upper motor neuron, Lower motor neuron

Q? •

Neuropathology

This gross brain specimen shows atrophy that is consistent with: A. Multiple sclerosis B. Pick’s disease C. Alzheimer’s disease D. Acute cerebral infarction

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Neuropathology

The correct answer is Alzheimer’s disease. Al h i ’ disease Alzheimer’s di i th is the mostt common dementing illness in adults, characterized by progressive dementia over several years. There is increased frequency with increasing age and in familial cases, cases usually earlier onset. Note the diffuse brain atrophy of Alzheimer's disease, unlike Pick’s disease which has mostly frontal lobe atrophy. p y Risk is increased in Down’s syndrome (BOARD FAVORITE).

BOARD FAVORITE!

Frontal Atrophy

Temporal Atrophy

Kuljis RO: Modular corticocerebral pathology in Alzheimer's disease. In: Mangone CA, Allegri RF, Ariza, eds. Dementia: A Multidisciplinary Approach. 1997: 143-55.

Degenerative disease, Alzheimer’s disease, dementia, diffuse atrophy

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Q? •

Neuropathology

The pathology seen here is most likely due to: A. Cerebellar astrocytoma B. Alcoholism C. Trauma D. Ruptured aneurysm E. Arteriovenous malformation

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A A. • •

Neuropathology

BOARD FAVORITE!

The correct answer is…. To obtain the answer to this question and to view over 400 more comprehensive pathology questions please purchase the full product here!

©™ Bolla L, Palmer RM: Paraneoplastic cerebellar degeneration. Case report and literature review. Arch Intern Med 1997 Jun 9; 157(11): 1258-62.

Degeneration, Cerebellum, Alcohol related degeneration, superior vermis of cerebellum

Neurobehavioral Section Neurobehavioral Section

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Q? •

Neurobehavioral

Which of the following types of mental retardation is associated with a maternallyinherited deletion of segment 11-13 of chromosome 15? A. Prader-Willi syndrome B. Rett’s syndrome C. Fragile X syndrome D. Angelman’s syndrome

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Neurobehavioral

BOARD FAVORITE!

The correct answer is D, Angelman’s syndrome. Angelman’s syndrome is classically associated with a maternally-inherited deletion of segment 11-13 11 13 on chromosome 15. 15 This ssyndrome ndrome is characterized characteri ed by b mental retardation, retardation abnormal gait, speech impairment, seizures, and inappropriate happy behavior that is often described as “happy puppet syndrome.” Prader-Willi syndrome consists of the same deletion (segment 11-13 of chromosome 15) but is located on the paternal chromosome 15. (Remember: P-P, Paternal-Prader-Willi) Rett’s syndrome is a progressive neurodevelopmental disorder that affects females only. only The cause of Rett’s syndrome is the MeCP2 gene, located on the long arm of the X chromosome. Fragile X syndrome is the most common form of inherited mental retardation. It is caused by a CGG repeat on the X chromosome, and effects include long face, enlarged ears, and macroorchidism. ©™ Fauci A, Braunwald E, et al. Harrison’s Principles of Internal Medicine 17th Edition. New York, NY: McGraw-Hill, Medical Pub. Division 2005. p. 407-14.

Classification: Neurobehavioral & Psychiatry, y y, mental retardation,, ggenetics

Q? •

Neurobehavioral

Fragile X syndrome is the most common form of inherited mental retardation. Somatic abnormalities seen commonly in Fragile X patients include a long face, large ears, and macroorchidism Which of the following genetic abnormalities is the most likely cause of Fragile macroorchidism. X syndrome? A. Absence of segment 11-13 on chromosome 5 B. MeCP2 gene on the long arm of the X chromosome C. CGG trinucleotide expansion of an area of the X chromosome D Inheritance D. I h i off an extra copy off chromosome h 21

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A A. • •

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Neurobehavioral

BOARD FAVORITE!

The correct answer is C, CGG trinucleotide expansion of an area of the X chromosome. Fragile X syndrome is the most common form of inherited mental retardation. It is caused b a CGG repeat on the X chromosome by chromosome, and effects incl include de long face face, enlarged ears ears, and macro-orchidism. It affects males more than females. Angelman’s syndrome is classically associated with a maternally-inherited deletion of segment 11-13 on chromosome 15. This syndrome is characterized by mental retardation, abnormal gait, speech impairment, seizures, and inappropriate happy behavior that is often described as “happy happy puppet syndrome. syndrome ” Prader-Willi syndrome consists of the same deletion (segment 11-13 of chromosome 15) but is located on the paternal chromosome 15. Rett’s syndrome is a progressive neurodevelopmental disorder that affects females only. The cause of Rett’s syndrome is the MeCP2 gene, located on the long arm of the X chromosome.

Sadock B, Sadock V. Kaplan & Sadock’s Synopsis of Psychiatry, 10th Edition. Philadelphia, PA: Lippincott Williams & Wilkins 2007. p. 1143.

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Q? •

Neurobehavioral

A 6 year-old boy has been sent for evaluation by a psychiatrist for attention deficit hyperactivity disorder (ADHD) because he has not been completing his homework and is constantly t tl interrupting i t ti his hi teacher t h andd classmates. l t His Hi teacher t h reports t that th t hhe hhas trouble sitting still. Upon questioning his parents, they report that their son’s inattention and hyperactivity started over 6 months ago. With a diagnosis of attention deficit hyperactivity disorder in this child, which of the following would be the first-line treatment? A. Individual psychotherapy B. Selective serotonin reuptake inhibitors & tricyclic antidepressants C. Methylphenidate (Ritalin) D. Monoamine oxidase inhibitor (MAOI) ©™

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A A. • •

Neurobehavioral

BOARD FAVORITE!

The correct answer is C, methylphenidate (Ritalin). Methylphenidate is considered the first line therapy for children diagnosed with attention deficit hyperactivity disorder (ADHD). Significant improvement is seen in roughly 75% of children when taking methylphenidate. Individual psychotherapy with a focus on behavioral modification can be used as adjunctive therapy, but is not considered first-line. Selective serotonin reuptake inhibitor (SSRI) and tricyclic antidepressant (TCA) therapy can be used as adjunctive pharmacologic therapy for ADHD, but is not a first-line treatment. Monoamine oxidase inhibitors (MAOIs) are also antidepressants, but are not used in the treatment of attention deficit hyperactivity disorder. Of note, it is important to recognize that in order for a diagnosis of attention deficit hyperactivity disorder to be made, symptoms must be present for a minimum of 6 months, and the child must have the onset of symptoms before the age of 7. ©™

Sadock B, Sadock V. Kaplan & Sadock’s Synopsis of Psychiatry, 10th Edition. Philadelphia, PA: Lippincott Williams & Wilkins 2007. p. 1211-14.

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Neurobehavioral

The treatment of attention deficit hyperactivity disorder (ADHD) classically involves the use of stimulants, such as methylphenidate (Ritalin). Which of the following are common adverse d effects ff t off methylphenidate? th l h id t ? A. Tachycardia, insomnia, and abdominal pain B. Bradycardia and hyperphagia C. Suicidal thoughts, gestures, or ideation D Increased intracranial pressure (ICP) D.

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A A. • • • •

Neurobehavioral

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The correct answer is A, tachycardia, insomnia, and abdominal pain. Tachycardia, insomnia, and abdominal pain are all reported side effects of methylphenidate. Bradycardia is not seen. Methylphenidate is also reported to cause a decrease in appetite, not hyperphagia. Increased intracranial pressure is not a side effect of methylphenidate use.

Sadock B, Sadock V. Kaplan & Sadock’s Synopsis of Psychiatry, 10th Edition. Philadelphia, PA: Lippincott Williams & Wilkins 2007. p. 1211-12.

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Neurobehavioral

At which age are developmental milestones of group play, ability to ride a tricycle, and the ability to copy a line or circle drawing present? A. 2 years B. 3 years C. 4 years D. 5 years

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A A. • • • •

Neurobehavioral

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The correct answer is B, 3 years. The above milestones are classically present at age 3. 4 year-olds, on the other hand, can participate in cooperative play and construct simple drawings (stick figure drawings, for example). Milestones like abstract reasoning and the formation of personality occur during adolescence (age 11 for girls and age 13 for boys).

Bhushan V, Le T, Chandwani R, Ozturk A. First Aid for the USMLE Step 1 2005: A Student to Student Guide. New York, NY: McGraw-Hill 2005. p. 120.

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Neurobehavioral

A 25 year-old medical student is arrested at school after stealing a laboratory scale. He has a history of multiple childhood arrests, both for abusing his neighbor’s dog and setting tti fires fi to t hhouses nearby. b Hi His school h l records d show h a hi history t off disciplinary di i li action ti for aggressiveness and fighting with classmates. During questioning, he shows no remorse for his actions. Prior to the age of 18, this individual would most probably have been diagnosed with which of the following? A. Antisocial personality disorder B. Schizotypal personality disorder C. Conduct disorder D. Borderline personality disorder E. Histrionic personality disorder ©™

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A A. • •

• •

•

Neurobehavioral

BOARD FAVORITE!

The correct answer is C, conduct disorder. Prior to age 18, individuals with the above behavior are classified as having conduct disorder. Currently, as a 25 year-old medical student, he would be classified as having antisocial personality disorder. Borderline personality disorder is characterized by unstable moods and labile, unstable relationships. Histrionic personality disorder is characterized by excessive attention-seeking behavior and emotionality. These individuals are dramatic, sexually provocative, and unable to form long-lasting relationships. Schizotypal personality disorder is characterized by individuals with eccentric behavior and peculiar thought patterns. They commonly exhibit odd beliefs or magical thinking. thinking ©™

Sadock B, Sadock V. Kaplan & Sadock’s Synopsis of Psychiatry, 10th Edition. Philadelphia, PA: Lippincott Williams & Wilkins 2007. p. 1220-1224.

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Neurobehavioral

Which of the following is a metabolic abnormality associated with anorexia nervosa? A. Hypochloremic hyperkalemic metabolic alkalosis B. Hypochloremic hypokalemic metabolic alkalosis C. Hyperkalemic metabolic acidosis D. Hyponatremia

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A A. • •

•

• •

Neurobehavioral

BOARD FAVORITE!

The correct answer is B, hypochloremic hypokalemic metabolic alkalosis. Anorexia nervosa is an eating disorder in which body weight is 15% below normal. Additionally, there is an intense fear of gaining weight, disturbed body image, and amenorrhea. The most common metabolic complication resulting from anorexia nervosa is hypochloremic hyperkalemic metabolic alkalosis. Lanugo, or fine body hair, is also commonlyy seen in the anorexic ppatient. Hypochloremic hypokalemic metabolic alkalosis is commonly seen in bulimia nervosa (secondary to emesis), not in anorexia. Hyponatremia and hyperkalemic acidosis are usually not seen in anorexia nervosa.

Sadock B, Sadock V. Kaplan & Sadock’s Synopsis of Psychiatry, 10th Edition. Philadelphia, PA: Lippincott Williams & Wilkins 2007. p. 731-2.

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Neurobehavioral

A 23 year-old female is brought in to your psychiatric clinic by her parents for an eating disorder evaluation. Her parents report that they rarely see her eating and are concernedd th thatt she h iis suffering ff i ffrom anorexia i nervosa. Y You perform f a hi history t andd physical examination on the patient without her parents present, and find that she is mildly overweight. You notice that she has calloused knuckles on her right hand. When you ask her questions about her body image, she informs you that she is extremely distressed by her inability to achieve a lower body weight. What is the most lik l di likely diagnosis? i ? A. Anorexia nervosa B. Bulimia nervosa, purging type C. Bulimia nervosa, non-purging type D This patient is not suffering from an eating disorder. D. disorder ©™

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A A. • • •

• •

•

Neurobehavioral

BOARD FAVORITE!

The correct answer is B, bulimia nervosa, purging type. This patient is suffering from bulimia nervosa, purging type. C Common findings fi di iin a bulimic b li i patient ti t off the th purging i ttype include i l d hypochloremic h hl i h hypokalemic k l i metabolic alkalosis, dental erosion, and calloused knuckles (all as a result of self-induced vomiting). Interestingly, while anorexia nervosa patients have body weight 15% below-average, bulimia nervosa patients often have normal body weights or are slightly overweight. In addition,, bulimia is described as ego-dystonic g y ((distressingg to the ppatient). ) These ppatients are aware that they have a problem and are distressed by it, making them more likely to seek help than anorexic patients. Anorexia nervosa, on the other hand, is ego-syntonic (not distressing to the patient) and patients suffering from anorexia are less likely to seek help. The purging type of bulimia nervosa involves vomiting, the use of laxatives, or the use of diuretics as a means to lose weight. The non-purging type of bulimia involves excessive exercise or fasting. Note that while anorexia and bulimia can potentially involve excessive fasting, fasting the anorexic will have a low body weight while the bulimic will have a normal body weight or be overweight.

Sadock B, Sadock V. Kaplan & Sadock’s Synopsis of Psychiatry, 10th Edition. Philadelphia, PA: Lippincott Williams & Wilkins 2007. p. 735-8.

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Neurobehavioral

A 23 year-old female gives birth to a healthy baby girl. However, on further inspection the newborn is noted to be deaf, has congenital cataracts, and has cardiac abnormalities b liti with ith possible ibl mental t l retardation. t d ti The Th mother th reported t d having h i an unremarkable pregnancy except during the first trimester when she reported having a mild illness consisting of a maculopapular rash, arthritis, aching joints, and diffuse swollen lymph nodes lasting 2-3 days. These congenital anomalies could have best been prevented by which of the following? A. Treatment of penicillin prior to pregnancy B. Receiving the measles, mumps, and rubella vaccine prior to pregnancy C. Avoiding uncooked pork and cat litter during the pregnancy D. Maintaining on highly active antiretroviral therapy (HAART) during the pregnancy E Receiving E. R i i th the varicella i ll zoster t vaccine i prior i tto pregnancy

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A A. • • • • • •

Neurobehavioral

The correct answer is B, receiving the measles, mumps and rubella vaccine prior to pregnancy. This patient who was not immunized to rubella, contracted the disease during her pregnancy as noted by her classic symptoms and classical congenital defects. defects Congenital rubella syndrome (CRS) has the highest transference rate during the first trimester. Choice A , refers to syphilis which usually manifests as a late abortion or congenital syphilis with eighth nerve deafness, saber shins, Hutchinson teeth, and a saddle nose Choice C, refers to Toxoplasmosis gondii a common protozoan parasite whose transmission also occurs during the first trimester. However, this parasite often manifests with fevers, seizures, chorioretinitis hepatosplenomegaly chorioretinitis, hepatosplenomegaly, jaundice, jaundice and hydrocephaly or microcephaly. microcephaly Choice D, refers to HIV infected mothers. Transmission is believed to occur late in pregnancy or during delivery. Choice E, refers to the varicella zoster virus , which can also occur during the first trimester, but in this trimester the effects are often a spontaneous abortion and possible teratogenic effects but not the series of effects listed. ©™

Charles R. B. Beckmann et al. Obstetrics and Gynecology Fifth Edition. Philadelphia, PA: Lippincott Williams & Wilkins 2006. p. 181- 184.

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Neurobehavioral

A 19 year old women presents to the psychiatrist with her mother. The patient reports having heard voices for the past month. On further questioning the psychiatrist discovers that for the past 2 months the patient has had grossly disorganized behavior and a delusion that someone is following her. Her mother states that she was completely normal until two months ago. What is the most likely diagnosis at this time? A. Schizophrenia B. Brief psychotic disorder C Schizoaffective C. S hi ff ti di disorder d D. Schizophreniform E. Schizotypical

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A A. • •

• •

Neurobehavioral

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The correct answer is D, schizophreniform. While this patient displays the DSM IV criteria for schizophrenia, her symptoms have only been present for the past 2 months. The DSM IV requires that symptoms be present for the past 6 months in order to diagnose schizophrenia (choice A). If the symptoms last under 1 month then the diagnosis of a brief psychotic disorder is made (choice B). Choice C, schizoaffective disorder is diagnosed when a patient meets both the DSM IV criteria for schizophrenia p and a mood disorder. Choice E, schizotypical is a personality disorder where a patient displays odd or magical beliefs, lack of friends, and the criteria for true psychosis is not met.

Benjamin James Sadock, M.D. and Virginia Alcott Sadock, M.D. Kaplan & Sadock’s Synopsis of Psychiatry. Philadelphia, PA: Lippincott Williams & Wilkins 2003. p. 471-511; 520-529; 806-807.

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Neurobehavioral

A nine year-old boy was adopted when he was one years old. His adopted papers showed that his mother was a recovering alcoholic and admitted to drinking heavily d i her during h pregnancy. Since Si he h started t t d school h l att age 6, 6 he h has h bbeen iin special i l education due to learning disabilities and an IQ test of 63. According to his IQ measurements, the boy would be classified as having: A. Borderline intellectual functioning B. Mild mental retardation C. Moderate mental retardation D. Severe mental retardation

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A A. • • • • •

Neurobehavioral

BOARD FAVORITE!

The correct answer is B, mild mental retardation. In mild mental retardation, IQ ranges from 50/55 to approximately 70. In moderate mental retardation, IQ ranges from 35/40 to 50/55 In severe mental retardation, IQ ranges from 20/25 to 35/40. Borderline intellectual function is a term no longer used.

Shaywitz SE, Cohen DJ, Shaywitz BA. Behavior and learning difficulties in children of normal intelligence born to alcoholic mothers. J  Pediatr. Jun 1980;96(6):978‐82

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Neurobehavioral

A 45 year-old man is brought into the doctor’s office by his wife. She states that her husband has been acting “weird” for the past few months. He does not eat food on the l ft side left id off hi his plate, l t ddoes nott shave h th the lleftft side id off hi his fface, andd will ill deny d that th t hhe even has a left arm. When the doctor asked the patient to draw a clock, he only drew a right sided clock. Shown here. What is the best diagnosis for this patient? A. Gerstmann’s syndrome B. Agnosia g C. Dementia D. Hemineglect E. Homonymous hemianopsia ©™

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A A. • • • • •

•

Neurobehavioral

BOARD FAVORITE!

The correct answer is D, hemineglect. Neglect is lack of attention to one side, usually the left. Patients with severe neglect may fail to describe objects on the left, dress or shave their left side. They may even deny their left arm as theirs and claim that it is the examiner’s arm. Patients with neglect do not have a hemianopia, or a primary motor or sensory deficit for that side; rather, rather they have damage to the non-dominant non dominant (usually right) hemisphere. Neglect is usually caused by lesions in the right frontal or parietal lobe

Guttmann-Steinmetz S, Crowell JA. Attachment and externalizing disorders: a developmental psychopathology perspective. J Am Acad Child Adolesc Psychiatry. Apr 2006;45(4):440-51.

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66

Neurobehavioral

A 26 year-old primigravid female has been admitted to the hospital today due to newonset seizures that reportedly began five days ago. She has a history of rapid-cycling bi l di bipolar disorder. d She Sh currently tl complains l i off ddepressive i symptoms t andd possible ibl suicidal ideation. The first year resident assigned to the case is interested in administering carbamazepine (Tegretol) as a mood stabilizer for her bipolar disorder. Which of the following adverse effects could carbamazepine potentially have on the developing fetus? A. Growth deficiency and microcephaly B. Neonatal withdrawal syndrome C. Spina bifida and agranulocytosis D. Ebstein’s anomaly and other cardiac defects E Floppy E. Fl baby b b syndrome d

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A A. • •

Neurobehavioral

BOARD FAVORITE!

The correct answer is…. To obtain the answer to this question and to view over 250 more comprehensive neurobehavioral questions please purchase the full product here !

©™ Ebert M, Loosen P, Nurcombe B. Current Diagnosis & Treatment in Psychiatry. New York, NY: McGraw-Hill 2000. p. 320-1.

Classification: Neurobehavioral & Psychiatry, pharmacology, carbamazepine, t t teratogenicity i it

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Neuroradiology

A 47 year-old male presents with a complaint of pain “shooting down” his right leg. The MRI is shown here here. Which of the following is the most appropriate management for this patient? A. Do nothing, MRI looks normal B. Laminectomy and discectomy C. C Laminectomy and instrumentation of L5-S1 D. Tethered cord release

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A A. • •

•

•

Neuroradiology

The correct answer is B, laminectomy and discectomy. far lateral Lumbar spine MRI shows a right far-lateral herniated disc (T2 axial view). Note that the disc bulge is not noted on the midline sagittal view. Far-lateral herniated discs commonly impinge on the exiting nerve root (same level) while more central discs affect the ttransiting t l di ff t th iti nerve root (level below). These patients generally do well with simple laminectomy and discectomy. There is no need to perform laminectomy and instrumentation of L5-S1. There is no evidence of a thickened filum or low conus suggestive of a tethered cord.

BOARD FAVORITE!

H. Richard Winn, M.D. Youman’s Neurological Surgery 5th Edition. Philadelphia, PA: Elsevier 2004. p. 4514-7.

Classification: Neuroradiology, Herniated Disc, Laminectomy and Discectomy

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Neuroradiology

This MRI of the brain is most suggestive of: A. Iron deposition B. Macrocrania C. Hypoxemia D. Generalized atrophy E. Butterfly glioma

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A A. • •

• • • •

•

Neuroradiology

The correct answer is A, iron deposition. Hallervorden-Spatz syndrome - involves the d deposition iti off iron i especially i ll iin th the globus l b pallidus and the retina. Dystonia, rigidity and neurobehavioral changes MRI: Globus pallidus on T2-weighted images "Eye-of-the-tiger" sign Central region of hyperintensity – Primary tissue insult – Produces edema Surrounding hypointensity – Region high in iron – May be 2° process H. Richard Winn, M.D. Youman’s Neurological Surgery 5th Edition. Philadelphia, PA: Elsevier 2004. p. 2720.

Classification: Neuroradiology, Hallervorden Spatz Syndrome, Syndrome Iron Deposition

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Neuroradiology

Based on the AP and lateral cerebral angiogram of the right external carotid artery shown here, here the most appropriate statement to tell this patient is that: A. The risk of bleeding is high due to the cortical venous drainage. B. The risk of bleeding is low due to the cortical venous drainage. drainage C. If the flow has rapid drainage into a sinus the bleeding risk is increased. D. This is a normal cerebral angiogram.

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A A. • •

• •

Neuroradiology

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The correct answer is A. The risk of bleeding is high due to the cortical venous drainage. This AP and lateral angiogram shows a dural arteriovenous fistula with main feeders from the occipital and middle meningeal arteries. Multiple venous lakes and varices are identified including two large lesions (these suggest high pressure). Drainage of the dural AVF is via cortical veins primarily into the sagittal and transverse sinuses. The risk of bleeding is high not low with cortical venous drainage. If the flow has rapid drainage into a sinus the bleeding risk decreases. ©™

Borden JA, Wu JK, Shucart WA. A proposed classification for spinal and cranial dural arteriovenous fistulous malformations and implications for treatment. J Neurosurg. 1995 Feb;82(2):166-79.

Classification: Neuroradiology, dural arteriovenous fistula, fistula cortical venous drainage

Occipital artery

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Neuroradiology

Locate the central sulcus: A. A B. B C. C D. D E. E F. F

A B D C E

F ©™

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A A. • • •

• •

• • •

Neuroradiology

The correct answer is C. Sigmoid “Hook” or “Ducky Sign” Hook like configuration (B) of the posterior Hook-like surface of the precentral gyrus. The “hook” or “ducky’s breast” corresponds to the motor hand area. The “hook” is seen well on CT (89%) and MRI (98%). Pars bracket sign The paired pars marginalis (F) form a “bracket” to each side of the interhemispheric fissure at or behind the central sulcus (96%). A = precentral sulcus D = postcentral gyrus E = postcentral sulcus

A B D C E

BOARD FAVORITE!

F ©™

Colen CB, Handbook of Neurosurgery and Neurology in Pediatrics, 2006

Classification: Neuroradiology, Neuroanatomy, Location of Central Sulcus

Q?

Neuroradiology

• A 2 day old asymptomatic neonate is transferred to your institution with the f ll i image. following i Which Whi h off the th following f ll i is i CORRECT regarding this pathology? A. It rarely presents with congestive heart failure. B. It never ppresents in older children. C. The embryonic correlate to this malformation is the median prosencephalic vein. D. Treatment for this neonate should include immediate embolization. embolization ©™

11

A A. • •

•

•

Neuroradiology

The correct answer is C, the embryonic correlate to this malformation is the median prosencephalic vein. Vein of Galen aneurysmal malformations (VGAM) probably represent an arteriovenous fistula (AVF) in the wall of a persistent embryonic vascular channel called the median prosencephalic vein. By week 10 of normal fetal development, the median prosencephalic vein regresses as the definitive internal cerebral veins Galen. i appear. A caudal d l remnantt remains i as the th vein i off G l Neonatal presentation with congestive heart failure is frequent with these malformations.

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Vein of Galen aneurysmal malformation

•Initial treatment of VGAM is conservative. Embolisation of a neonate is a high risk procedure and the child should be  treated medically (for cardiac failure) until aged 5 or 6 months with regular outpatient assessment. Elective embolisation  can be scheduled for this time with the aim of closing the AVS with cyanoacrylate glue. If the infant deteriorates  ( i (seizures, failure to thrive, worsening cardiac failure, etc) treatment is performed earlier. f il t th i i di f il t )t t ti f d li Lasjuanias P. Vascular Diseases in Neonates, Infants and Children. New York: Springer Verlag 1997. Horowitz MB, Jungreis CA, Quisling RG, Pollock I. vein of Galen aneurysms: a review and current perspective. AJNR 1994; 15:1486-1496. H. Richard Winn, M.D. Youman’s Neurological Surgery 5th Edition. Philadelphia, PA: Elsevier 2004. p. 3434.

Classification: Neuroradiology, Vascular Pathology Vein of Galen Aneurysmal Pathology, Malformations

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Neuroradiology

A five-month-old male infant presents with an abnormal head shape. What is the most likely di diagnosis? i ? A. Sagittal synostosis B. Bicoronal synostosis C. Left unicoronal synostosis D Right unicoronal synostosis D. E. Metopic synostosis

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A A. • •

•

Neuroradiology

The correct answer is C, left unicoronal synostosis. Coronal synostosis can occur either on the right or left side (unicoronal) or both (bicoronal (unicoronal), (bicoronal.)) It frequently occurs prenatally, prenatally and appears to occur more commonly in males. Restriction of normal cranial growth at one suture between the frontal and parietal bones produces a characteristic flattening of the forehead on the fused side, and a bulging of the forehead on the non-fused side. Thi bbulging, This l i or bbossing, i iis a resultlt off compensatory t growth th off the contralateral (opposite side) coronal suture. Secondary bulging of the temporal region on the ipsilateral (same) side can also occur. The placement of the eyes within the orbit is also typically affected. The superior margin of the orbit on the synostosed side is raised, a feature known as Harlequin eye d f it deformity.

Contralateral •Frontal bossing •Displaced fontanelle p

Ipsilateral •Flattened forehead •Raised brow (Harlequin Eye) •Temporal bulging

©™ H. Richard Winn, M.D. Youman’s Neurological Surgery 5th Edition. Philadelphia, PA: Elsevier 2004. p. 3300-01.

Classification: Neuroradiology, Infantile Pathology, Unicoronal Synostosis

Q? •

Neuroradiology

This x-ray demonstrates which of the following? A. McRae’s line B. McGregor’s line C. Rule of Spence D. Chamberlain’s line A

B

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Used with permission from Handbook of Neurosurgery and Neurology in Pediatrics; By Chaim B. Colen, MD, PhD.

A A. • • • • •

Neuroradiology

BOARD FAVORITE!

The correct answer is C, rule of Spence. McRae’s line - foramen magnum diameter: 35 mm+/- 4 McGregor’s line - line from the hard palate to the caudal most part of the opisthion (odontoid should be < 4 mm above this line > 4 mm suggests basilar impression). Rule of Spence - if the sum of A + B ≥7mm, consider disruption of the transverse ligament (requires rigid immobilization). Chamberlain’ss line - diagonal line from the hard palate to the posterior foramen Chamberlain magnum (odontoid should not extend 1/3 of its height above this line).

Colen CB, Handbook of Neurosurgery and Neurology in Pediatrics, 2006.

Classification: Neuroradiology, Rule of Spence, Clinical Definition

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Neuroradiology

What is the most likely diagnosis depicted in this MRI and MRS? A. Low grade glioma B. Pleiomorphic xanthoastrocytoma C. High grade glioma D None D. N off the h above b

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A A. • •

•

•

Neuroradiology

BOARD FAVORITE!

The correct answer is C, high grade glioma. N-acetylaspartate (NAA) is predominantly located in neurons and is thus decreased in all neoplasms l th thatt cause th the neurons tto bbe di displaced l d or replaced l d with ith malignant li t cells. ll Fi Findings di of numerous studies have demonstrated decreased NAA values in glial neoplasms. Choline (Cho) peak contains contributions from glycerophosphocholine, phosphocholine, and phosphatidylcholine components that are thought to reflect cellular membrane density and turnover. As in any process that leads to hypercellularity and increased membrane proliferation the Cho value is consistently elevated in gliomas. proliferation, gliomas Lactate (Lac) indicates that cellular respiration has shifted from the oxidative metabolism of carbohydrates to nonoxidative metabolism. Increased reliance on anaerobic glycolysis is found in highly malignant tumors.

Law M, Hamburger M, Johnson G, Inglese M, Londono A, Golfinos J, Zagzag D, Knopp EA. Differentiating surgical from non-surgical lesions using perfusion MR imaging and proton MR spectroscopic imaging. Technol Cancer Res Treat. 2004 Dec;3(6):557-65. Review.

Classification: Neuroradiology, Magnetic Resonance Spectroscopy Spectroscopy, High Grade Glioma

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Neuroradiology

A 64 year-old black male presented with left sided headache and slurring of speech. Head CT is shown. Which of the following would likely be found on physical examination? i ti ? A. Contralateral hemiparesis B. Ipsilateral hemiparesis C. Restricted left (lateral) gaze D Restricted right (medial) gaze D. E. Locked-in-syndrome F. Both A and C

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49

A A. • • • • •

Neuroradiology

The correct answers are A, (C and F). This patient has Millard Gubler syndrome! REMEMBER - 7th nerve (7 letters in Millard) and - 6th nerve (6 letters in Gubler) PLUS the corticospinal tract. Millard-Gubler syndrome is associated with abducens (CN6) and facial nerve (CN7) paralysis, as well as contralateral hemiplegia of the extremities. It involves unilateral damage to the inferior pons, pons commonly caused by pontine infarction or hemorrhage, and leads to damage of the above structures. The muscles of the ipsilateral side of the face are paralyzed, diplopia, internal strabismus, and loss of extroversion are also typically present.

Onbas O, Kantarci M, Alper F, Karaca L, Okur A. Millard-Gubler syndrome: MR findings. Neuroradiology. 2005 Jan;47(1):35-7.

Classification: Neuroradiology, Millard Gubler Syndrome, Physical Exam

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50

Neuroradiology

A 57 year-old male underwent transnasal/ transfacial ethmoidectomy for metastatic squamous cellll carcinoma. i P Post-operatively t ti l hhe developed clear rhinorrhea. Lumbar pucture was performed daily for 3 days draining 20 cc each time. On the third day he became confused and lethargic. On examination he was non-verbal and l li d tto painful localized i f l stimulus. ti l H Headd CT iis shown. h What is his most likely diagnosis? A. Recurrent tumor B. Meningitis C. Tension pneumocephalus D. Normal postoperative scan

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A A. • •

•

Neuroradiology

The correct answer is C, tension pneumocephalus. This head CT shows the Mt Fuji sign - twin peaks with bifrontal pneumocephalus. The Mount Fuji sign is a finding that can be observed on computed tomographic (CT) scans of the brain (1), in which bilateral subdural hypoattenuating collections cause compression and separation of the frontal lobes. The collapsed frontal lobes and the widening of the interhemispheric space between the tips of the frontal lobes have the appearance of the silhouette of Mount Fuji hence, the Mount Fuji sign. Given this patient’s decline in mental status he is likely developing tension pneumocephalus. pneumocephalus

H. Richard Winn, M.D. Youman’s Neurological Surgery 5th Edition. Philadelphia, PA: Elsevier 2004. p. 616. Ommaya AK: Cerebrospinal fluid fistula and pneumocephalus. In: Wilkins RH, Rengachary SS, eds. Neurosurgery. 2nd ed. New York: McGraw-Hill; 1996: 2773-82

Classification: Neuroradiology, Tension Pneumocephalus, CT scan

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Q? •

Neuroradiology

A 4 year-old boy is brought to the emergency room after a fall. Lateral skull k ll x-ray is i shown h tto th the right. i ht Which of the following is the most likely diagnosis? A. Epidermoid p ggranuloma B. Eosinophilic C. Hemangioblastoma D. Normal skull x-ray

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66

A A. • •

•

• •

Neuroradiology

BOARD FAVORITE!

The correct answer is B, eosinophilic granuloma. AP view of the skull reveals an approximate 3 cm. circular lytic lesion overlying the left f t frontoparietal i t l region i with ith wellll ddelineated li t d non-sclerotic l ti margins, i suggestive ti off hi histiocytosis ti t i X (eosinophilic granuloma). Differential Diagnosis: Solitary lytic lesion in the skull includes metastatic lesion, myeloma, fibrous dysplasia, tuberculosis, trauma, histiocytosis X, osteomyelitis, leptomeningeal cyst, and epidermoid/dermoid. E i Eosinophilic hili granuloma l - lytic l ti llesion i with ith wellll ddelineated li t d non-sclerotic l ti margins i andd beveled edges. Epidermoids - lytic lesion with sclerotic margins.

©™ Yang JT, Chang CN, Lui TN, Ho YS. Eosinophilic granuloma of the skull--report of four cases. Changgeng Yi Xue Za Zhi. Dec 1993;16(4):257-62.

Classification: Neuroradiology, Pediatric Pathology, Eosinophilic Granuloma

Q? •

Neuroradiology

Treatment for this lesion in a patient with neurofibromatosis type -1 should consist of: A. Removing the optic nerve and attached globe. B. Removing the optic nerve and both globes. C. Radiotherapy with 25 Gray D. Radiotherapy with 80 Gray

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A A. • •

•

•

Neuroradiology

The correct answer is A, removing the optic nerve and attached globe. MRI demonstrates a high intensity lesion of the distal right optic nerve, suggestive of optic glioma, especially in a patient with neurofibromatosis type 1. p gglioma distal to the Treatment of optic chiasm involves removing the optic nerve and attached globe. Treatment of optic glioma involving the chiasm involves removing the optic nerve, globe and radiotherapy (less than 8 Gray). Gray) Board Favorite! Hollander MD, FitzPatrick M, O'Connor SG, et al: Optic gliomas. Radiol Clin North Am 1999 Jan; 37(1): 59-71.

Classification: Neuroradiology, NF-1 MRI, Treatment Modalities

BOARD FAVORITE!

©™

Q? •

Neuroradiology

This tumor most likely has elevated serum levels of which of following: A. Alpha-fetoprotein B. Carcinoembryonic antigen (CEA) C. Cancer antigen-125 (CA-125) D. β-human chorionic gonadotropin (βHCG) E. Placental alkaline phosphatase (PLAP)

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69

A A. • •

• • • • • • •

Neuroradiology

The correct answer is D, β-HCG. T1W non-contrast MRI sagittal image shows a pineal recess mass compressing the tectal plate and causing triventricular hydrocephalus. INTRACRANIAL GERM CELL TUMORS Germinoma is the most common. Most common tumor of pineal recess 40% Males Respond well to chemo and radiotherapy Cancer antigen-125 (CA-125)- ovarian cancer Placental alkaline phosphatase (PLAP) - ovarian cancer

BOARD FAVORITE!

Teilum Concept

©™ Colen CB, Handbook of Neurosurgery and Neurology in Pediatrics, 2006 p. 41

Classification: Neuroradiology, Intracranial Germ Tumors, Serum levels

Q? •

70

Neuroradiology

A 20 year-old female presents with 3 weeks of headaches, nausea, andd vomiting. iti MRI iis shown here. Which of the following is the most appropriate next step in management? A. Operative resection B. No intervention needed C. Shunt placement or third ventriculostomy D. Radiotherapy E A andd C E.

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A A. • •

Neuroradiology

BOARD FAVORITE!

The correct answer is…. To obtain the answer to this question and to view over 250 more comprehensive neuroradiology questions please purchase the full product here !

©™ Barkovich AJ, Krischer J, Kun LE, et al: Brain stem gliomas: a classification system based on magnetic resonance imaging. Pediatr Neurosurg 1990-91 ;16(2): 73-83

Classification: Neuroradiology, Tectal Glioma, Treatment Modalities

Q? •

Neurology

This patient most likely sustained damage to which of the following structures? t t ? A. Bilateral hypoglossal nuclei B. Left hypoglossal nerve C. Left hypoglossal nucleus D. D Left upper motor neuron to the hypoglossal nucleus E. Right vagal nucleus

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2

A A. • •

Neurology

BOARD FAVORITE!

The correct answer is D, left upper motor neuron to the hypoglossal nucleus. Damage to the left hypoglossal nucleus (or nerve) would cause tongue deviation to the left (ipsilateral). However, as depicted in the photo, damage to the upper motor neurons (which cross), would cause tongue deviation to the right (contralateral).

Midstokke S, Hess SJ, Saini T, Edwards PC. Unilateral tongue atrophy. Gen Dent. 2006 Nov-Dec;54(6):425-7.

Classification: Neurology, upper motor neuron lesion, hypoglossal nucleus

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Q? •

Neurology

The function of the superior olive is: A. Sound localization B. Proprioception C. Vestibular localization and function D. Visual gaze control

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6

A A. • •

•

•

Neurology

The correct answer A, sound localization. The superior olivary nucleus (or superior olive) li ) is i a smallll mass off gray substance b t situated on the dorsal surface of the lateral part of the trapezoid body. The superior olivary nucleus’ primary input is bilateral and from the bushy cells of the anterior ventral cochlear nuclei (AVCN) (AVCN). This input occurs primarily via the ventral acoustic stria. Its output is to the lateral lemnisci. The superior olivary nucleus is the first point where binaural input is combined.

BOARD FAVORITE!

Superior and Inferior colliculi

Superior Olivary nucleus Cochlear nucleus Nucleus gracilis Nucleus cuneatus

©™ Kandel ER, Schwartz JH, Jessell TM. Principles of Neural Science, 4th ed. McGraw-Hill, New York. 2000. p. 606.

Classification: Neurology, Superior Olive Nucleus, Function

Q? •

Neurology

The function of the superior olive is demonstrated by which waveform: A. 1 B. 2 C. 3 D. 4 1 E 5 E. F. 6 G. 7

45 23

6

7

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7

A A. • • • • • • • • • •

Neurology

The correct answer C, 3. Brainstem auditory evoked responses test the i t it off the integrity th auditory dit pathway. th Wave 1 –auditory nerve Wave 2 –Cochlear nuclei (pons) Wave 3 –Superior olivary complex Wave 4 –Lateral lemniscus Wave 5 –Inferior colliculus Wave 6 –Medial geniculate nucleus Wave 7 –Auditory radiations (cortex) Damage to one of the structures will result in an increased latency! (e (e.g. g acoustic neuroma will show increased latency of 1-3).

BOARD FAVORITE!

45 1

23

Kandel ER, Schwartz JH, Jessell TM. Principles of Neural Science, 4th ed. McGraw-Hill, New York. 2000. p. 606.

Classification: Neurology, Evoked Potential, Superior Olive

6

7

KNOW THESE WAVEFORMS WELL! BOARD FAVORITE!

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Q? •

Neurology

Match the following statements with the correct answer: 1. Lateral vestibulospinal tract 2. Rubrospinal tract A. B. C. D.

Extensor tone Flexor tone Both Neither

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9

A A.

Neurology

BOARD FAVORITE!

1. The correct answer is A, extensor tone. The lateral vestibulospinal tract regulates extensor tone through the lateral vestibular nucleus. • The vestibulospinal tract arises from the lateral vestibular nucleus (i.e. Deiter’s nucleus) and descends bilaterally in the anterior part of the lateral funiculus. 2. The correct answer is B, flexor tone. Flexor activity is regulated by the rubrospinal tract from the red nucleus. • The rubrospinal tract arises from magnocellular neurons in the red nucleus and crosses at the ventral tegmental decussation. Stimulation of the red nucleus leads to excitation of contralateral flexor alpha motor neurons and inhibition of extensor alpha motor neurons.

©™ Kandel ER, Schwartz JH, Jessell TM. Principles of Neural Science, 4th ed. McGraw-Hill, New York. 2000. p. 668.

Classification: Neurology, Motor Tracts, lateral vestibulospinal and rubrospinal tract

Q? •

Neurology

Which brainstem nuclei release serotonin? A. Raphé nuclei B. Vestibular nuclei C. Hypoglossal nuclei D. Nucleus cuneatus E. Nucleus gracilis

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11

A A. •

Neurology

The correct answer is A, raphé nuclei. The raphé nuclei are located in the brainstem. Neurons in the dorsal raphé nucleus produce serotonin, they have long projections (green arrow) that carry the neurotransmitter to the orbital prefrontal cortex.

CROSS SECTION

Dorsal raphé nuclei

©™ Dorsal raphé nuclei

Classification: Neurology, Brainstem Nuclei, Serotonin Releasing Cells

Q? •

Neurology

Lack of sensory loss, presence of a “pinch sign”, and hand weakness is characteristic of: A. Carpal tunnel syndrome B. Anterior interosseous syndrome C. Ulnar nerve entrapment D. Posterior interosseous syndrome

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32

A A. •

Neurology

BOARD FAVORITE!

The correct answer is B, anterior interosseous syndrome. The “pinch sign” often occurs when there is a complete anterior interosseous nerve (AIN) lesion. Attempts to pinch the tips of the terminal phalanges of the index finger and thumb results in an extension of the distal phalanges. phalanges Thus Thus, the pulps rather than the tips of these two digits approximate.

NORMAL

PINCH SIGN

Note the distinctive findings g of a AIN syndrome y on pperformingg the ppinch test. There is loss of function in the AINinnervated flexor pollicis longus (FPL) and flexor digitorum profundus (FDP) muscles, leading to weakness in the distal phalanges of the thumb and forefinger. The left photograph shows the normal function when attempting this hand posture. H. Richard Winn, M.D. Youman’s Neurological Surgery 5th Edition. Philadelphia, PA: Elsevier 2004. p. 3925..

Classification: Neurology, Diagnostic Tests, anterior interosseous syndrome

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Q? •

Neurology

30 year-old female presents with a history of shunted pseudotumor cerebri and small ventricles, now complains of worsening headache. Fundoscopic exam is shown below. Whi h off the Which th following f ll i statements t t t iis TRUE? A. It is best treated with Diamox. B. She likely has shunt failure and stiff ventricles. C. She has venous thrombosis. D There is no cause for concern about this headache. D. headache

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63

A A. •

•

•

Neurology

The correct answer is B, she likely has shunt failure and stiff ventricles. A low index of suspicion should be present when evaluating a patient with shunted pseudotumor cerebri (PTC) Approximately 40% of shunts placed in children will fail in the 1st year and almost all (PTC). children will require shunt revision at some point. In PTC, shunt malfunction may result in blindness. PTC is encountered most frequently in young, overweight women between the ages of 20 and 45. Headache, occurring in more than 90 percent of cases, is the most common presenting complaint Dizziness complaint. Dizziness, nausea nausea, and vomiting may also be encountered, encountered but there are typically no alterations of consciousness or higher cognitive function. It is defined clinically by four criteria: (1) elevated intracranial pressure, as demonstrated by lumbar puncture; (2) normal cerebral anatomy, as demonstrated by neuroradiographic evaluation; (3) normal cerebrospinal fluid composition; and (4) signs and symptoms of p increased intracranial ppressure, includingg ppapilledema. Martin TJ, Corbett JJ: Pseudotumor cerebri, in Youmans JR(ed): Neurological Surgery, ed 4. Philadelphia: WB Saunders,1996, Vol 4, pp 2980–2997.

Classification: Neurology, pseudotumor cerebri, shunt failure

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Q? •

Neurology

Based on the radiological features seen in this MRI, choose the most appropriate statement regarding this condition: diti A. They have no intervening brain tissue between the vascular spaces on histopathology. B. They do have intervening brain tissue between the vascular spaces p on histopathology. p gy C. There is no genetic association. D. The HOX gene is highly associated.

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79

A A. • •

• • • •

Neurology

BOARD FAVORITE!

The correct answer is A, they have no intervening brain tissue between the vascular spaces on histopathology. Cavernous malformations (i.e. (i e angioma angioma, cavernoma cavernoma, or cavernous hemangioma) may be inherited or sporadic and consist of variable sized sinusoids or cavernous spaces between capillaries. Unlike arteriovascular malformations and capillary telangiectasias., they have no intervening brain tissue between the vascular spaces and have thus been described as “blood sponges”. MRI shows welldefined, usually rounded lesions with little or no mass effect and are without vasogenic edema (unless hemorrhage is present). There may be small areas of new or old hemorrhages shown as a rim of hemosiderosis around the cavernous angioma in the surrounding brain tissue. tissue Genetics: more common in Hispanics. CCM1 (for cerebral cavernous malformation 1) -chromosome 7 at band 7q11.2-q21. It is also known as KRIT1, for the protein created by the gene. 40% of familial cavernous angiomas. CCM2 -band 7p15-p13, protein named malcavernin. 20% of familial cavernous angiomas. CCM3 identified as linked to familial cavernous angioma is on chromosome 3 at band 3q. 3q

Craig HD, Gunel M, Cepeda O, et al: Multilocus linkage identifies two new loci for a mendelian form of stroke, cerebral cavernous malformation, at 7p15-13 and 3q25.2-27. Hum Mol Genet 1998 Nov; 7(12): 1851-8.

Classification: Neurology, Cavernous Malformations, Histopathology

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Q Q. •

Neurology

This fundus is MOST likely seen in which of the following patients? A. 25 year-old female B. 14 year-old diabetic male C. 75 year-old diabetic male D. 25 year-old obese male E 25 year-old E. ld obese b ffemale l

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80

A A. • •

Neurology

The correct answer is E, 25 year-old obese female. This ocular fundoscopic image demonstrates classic grade 2 papilledema most likely seen in a 25 yearold obese female with pseudotumor cerebri (idiopathic intracranial hypertension).

Grade II papilledema. The halo of edema now surrounds the optic disc.

Brazis PW, Lee AG: Elevated intracranial pressure and pseudotumor cerebri. Curr Opin Ophthalmol 1998 Dec; 9(6): 27-32.

Classification: Neurology, Pseudotumor Cerebri, Papilledema

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Q? •

Neurology

In myopathic disorders the motor unit potentials on electromyography would most likely show which of the following? A. Increased amplitude B. Decreased duration C. Monophasic D. Few in numbers with decreased recruitment E A andd D E.

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86

A A. • •

Neurology

BOARD FAVORITE!

The correct answer is…. To obtain the answer to this question and to view over 300 more comprehensive neurology questions please purchase the full app here !

Polyphasic “giant wave” of reinnervation

Low amplitude wave of myopathy

Daube JR: The description of motor unit potentials in electromyography. Neurology 1978 Jul; 28(7): 623-5.

Classification: Neurology, Electromyography, Myopathic Disorders

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Q Q. •

Neurocritical Care

What is the hallmark indicator for disseminated intravascular coagulation (DIC)? A. Decreased fibrin-split products B. Increased factor X C. Low platelets D. Decreased d-dimer

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5

A A. •

Neurocritical Care

The correct answer is C, low platelets. Coagulation factors are decreased. Fibrin-split products and d-dimers increase. Inciting factor: Ex. crush injury, endotoxin

Systemic activation of coagulation factors

Widespread intravascular fibrin deposition

Consumption of platelets and clotting factors

Thrombosis & organ failure

Thrombosis & organ failure ©™

DIC

Classification: Neurocritical Care, Coagulation g Disorders,, DIC

Q? •

Neurocritical Care Neurocritical Care

A 36 year old male presents to the emergency room as a trauma code. During resuscitation, clear fluid is noted dripping out of the left nare. An appropriate statement regarding di the th bedside b d id glucose l test t t is i that th t it has: h A. high sensitivity, low specificity B. high sensitivity, high specificity C. low sensitivity, low specificity D high sensitivity, D. sensitivity low specificity

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22

A A. •

•

•

Neurocritical Care Neurocritical Care

The correct answer is C, low sensitivity, low specificity. The bedside glucose test has low sensitivity and low specificity. Therefore, it should be used only for trying to rule out a CSF leak rather than ruling it in. in In the emergent setting, when evaluating a patient with rhinorrhea, if the rhinorrhoea contains glucose, the specificity of the test for CSF can be improved by excluding other factors that increase the glucose concentration of nasal discharge. If the nasal discharge is not blood stained, the blood glucose (measured at the same time as CSF glucose) is <6 mmol L–11, and there are no other symptoms of upper respiratory tract infection mmol.L infection, such as sneezing, nasal blockage, cough, sore throat, sputum, or purulent nasal discharge, then this increases the likelihood that the discharge contains CSF. If rhinorrhoea does not contain glucose, then either it does not contain CSF or CSF glucose concentrations are below the limit of detection by the sticks (false negative measurement). g y sensitive and specific p but would require q a few days y to Beta-2 transferrin test is both highly process the Western blot at most institutions. Chan DT, Poon WS, IP CP, Chiu PW, goh KY. How useful is glucose detection in diagnosing cerebrospinal fluid leak? The rational use of CT and Beta-2 transferrin assay in detection of cerebrospinal fluid fistula. Asian J Surg. 2004 Jan;27(1):39-42.

Classification: Neurocritical Care, CSF Leak,, Bedside Glucose Test

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Q? •

Neurocritical Care Neurocritical Care

Name three functions of platelets: A. Attach, agglutinate, agranulate B. Adhere, activate, avascularize C. Adhere, aggregate, agranulate D. Apoptosis, aggregate, agranulate E. Acidification, activate, agranulate

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34

A A. •

Neurocritical Care Neurocritical Care

The correct answer is C, adhere, aggregate, and agranulate (degranulate) (Hint AAA). Vascular Vascular phase phase

Adherence Adherence

Vessel V j Vessell IInjury Injury

Platelet Platelet phase phase

Platelet GPIb-IX-V complex GPIb receptor

vWf

Vasospasm Vasospasm 2

Endothelial cell

Collagen

Agranulation g Agranulation

•Platelets can be activated by binding to collagen, which is mediated directly through glycoprotein VI (GPVI) or indirectly via von Willebrand factor (VWF) binding to GPIb-V–IX. Signal transduction from any of these receptors leads to phospholipase C recruitment, which mediates calcium mobilization, platelet shape change, degranulation, and activation of GPIIb/IIIa to allow binding of fibrinogen and plateletplatelet interactions.

Release of plateletactivation factors

+

GpIIb/IIIa +

Phospholipase C activation

GPIb-IX-V complex

1

Adherence Adh Adherence

fibrinogen

3

Yee DL, Bergeron AL, Sun CW, Dong JF, Bray PF. Platelet hyperreactivity generalizes to multiple forms of stimulation. J Thromb Haemost. 2006 Sep;4(9):2043-50

Classification: Neurocritical Care, Coagulation g Cascade,, Platelet Function

Aggregation Aggregation

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Q?

Neurocritical Care Neurocritical Care

1. Choose the most common inherited cause of post-operative bleeding? A. Bernard-Soulier disease B. Von Willebrand disease C. Hemophilia A D. Hemophilia B E. Leiden factor disease 2. Inheritance of this disease is mostly: A. Autosomal dominant B. Autosomal recessive ©™

36

A A.

Neurocritical Care Neurocritical Care

1. The correct answer is B, von Willebrand disease. 2. The correct answer is A, autosomal dominant. • Von Willebrand disease is the most common coagulation disorder. Inheritance is autosomal dominant transmission in 90% of cases. Children born with the disease have either low levels (quantitative defect) of von Willebrand factor (vWF), or defective form (qualitative defect) of the same protein. • Bernard-Soulier: defective or decreased expression of the glycoprotein Ib/IX/V complex on th surface the f off th the platelets. l t l t Thi This complex l iis th the receptor t ffor von Will Willebrand b d ffactor t ((vWF), WF) andd the result of decreased expression is deficient binding of vWF to the platelet membrane at sites of vascular injury, resulting in defective platelet adhesion. • Factor V Leiden is the most common hereditary hypercoagulability disorder. In this disorder the Leiden variant of factor V, cannot be inactivated by activated protein C. Franchini M. Advances in the diagnosis and management of von Willebrand disease. Hematology. 2006 Aug;11(4):219-25.

Classification: Neurocritical Care, Coagulation Disorders, Von Willebrand Disease Di

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Q? •

Neurocritical Care

You are consulted on a 22 year-old female who is one day postpartum and has been admitted to the intensive care unitit with ith widespread id d petechiae, t hi purpura, andd mucocutaneous bleeding. Head CT is shown on the right. Which of the following is INCORRECT regarding this condition? A. Blood clotting mechanisms are activated throughout the body. B. Thrombocytopenia may occur. C. Thrombocytosis is common D. Fibrin-split products are increased. ©™

38

A A. • • •

• •

Neurocritical Care

The correct answer is C, thrombocytosis is common Thrombocytopenia occurs, not thrombocytosis. The immediate postpartum period is a highly thrombogenic state. There are many risk factors (amniotic fluid) that can cause a dysregulated hypercoagulable condition resulting in disseminated intravascular coagulation (DIC). DIC occurs when blood clotting mechanisms are activated throughout the body. Small blood clots form throughout the body, exhausting the supply of blood clotting factors which become rare at sites of real tissue injury. Simultaneously, clot dissolving mechanisms are also increased. Hence,, this disorder can result in both excessive bleedingg and clotting. DIC may be caused by infections (especially gram-negative bacteria) , severe trauma, cancer, blood transfusions, and obstetrical complications. Pathophysiology: two main mechanisms- generation of thrombin (microvascular thrombosis and organ ischemia) and generation of plasmin (characterized mainly by hemorrhagic symptoms). Fibrin-split products are increased in DIC.

Levi M, de Jonge E, van der Poll T, ten Cate H. Advances in the understanding of the pathogenetic pathways of disseminated intravascular coagulation result in more insight in the clinical picture and better management strategies. Semin Thromb Hemost. 2001 Dec;27(6):569-75. Review.

Classification: Neurocritical Care, Coagulation g Disorders,, Post Partum

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Q? •

Neurocritical Care Neurocritical Care

This ocular fundus is MOST likely seen in which of the following patients? A. 25 year-old obese female punched in the stomach with a GCS of 15 B. 14 year-old male status post temporal lobectomy C. 31 year year-old old male thrown from a 3rd story window with a GCS of 5 D. 56 year-old with a 1 cm cerebellar hematoma with a GCS of 15

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46

A A. • •

•

Neurocritical Care Neurocritical Care

The correct answer is C, 31 year-old male thrown from a 3rd story window with a GCS of 5. Thi type This t off papilledema ill d is i seen in i the th setting tti off hi highh intracranial pressure (ICP), such as the above patient who has suffered severe traumatic brain injury. Cerebellar hematoma acutely would cause high ICPs in the infratentorial compartment that potentially could result in acute obstructive hydrocephalus with papilledema, papilledema but this is less likely in a patient that has a 1 cm cerebellar hematoma and a GCS of 15. Grade IV papilledema. There is severe swelling in addition  to a circumferential halo. The edema covers major blood  vessels as they leave the optic disk (grade III) and vessels on  the disk (grade IV). A subretinal hemorrhage is present at 7 o A subretinal hemorrhage is present at 7 o'clock clock.  ©™

Bhatt UK. Bilateral optic disc swelling; is a CT scan necessary? Emerg Med J. 2005 Nov;22(11):827-30.

Classification: Neurocritical Care, Hematoma,, Ocular Changes g

Q? •

Neurocritical Care Neurocritical Care

Which of the following is the preferred ventilation pattern in non-paralyzed patients with central nervous system dysfunction? A. Continuous mandatory ventilation B. Assist control ventilation C. Intermittent mandatory ventilation D. Synchronous intermittent mandatory ventilation E Pressure E. P supportt ventilation til ti

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47

A A. • • •

Neurocritical Care Neurocritical Care

The correct answer is D, synchronous intermittent mandatory ventilation. Patients with CNS dysfunction tend to exhibit variations in tidal volume and respiratory drive. Synchronized intermittent mandatory ventilation (SIMV) delivers volume-cycled breaths that coincide with spontaneous lung inflations. The patient can breathe unassisted and spontaneously between mechanical breaths. Also, a preselected p y rate can be used if the ppatient`s respiratory p y drive is insufficient to pprevent respiratory hypercarbia.

Stock MC, Perel A: Handbook of mechanical ventilatory support. Baltimore: Williams & Wilkins; 1997.

Classification: Neurocritical Care, CNS dysfunction, y , breathingg ppatterns

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Q? •

Neurocritical Care Neurocritical Care

Which of the following characteristics are seen in patients with acute respiratory distress syndrome (ARDS)? A. Bilateral lung infiltrates B. Pulmonary capillary wedge pressure less than or equal to 18 mm Hg C. PAO2/FIO2 ratio of < 200 mm Hg D. All of the above

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53

A A. • •

Neurocritical Care Neurocritical Care

BOARD FAVORITE!

The correct answer is D, all of the above. Acute respiratory distress syndrome (ARDS) is characterized by acute onset, bilateral infiltrates in chest x-ray, pulmonary capillary wedge pressure (PCWP) less than or equal to 18 mm Hg and a PAO2/FIO2 ratio < 200 mm Hg.

Kollef MH, Schuster DP: The acute respiratory distress syndrome. N Engl J Med 1995 Jan 5; 332(1): 27-37.

Classification: Neurocritical Care, Respiratory Distress Syndrome, Lab Vl Values

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Q? •

Neurocritical Care Neurocritical Care

Which of the following findings will distinguish cardiogenic pulmonary edema from acute respiratory distress syndrome (ARDS)? A. Bilateral lung infiltrates on chest x-ray B. Hypoxemia C. Pulmonary capillary wedge pressure (PCWP) > 18 mm Hg D. All of the above

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55

A A. • •

Neurocritical Care Neurocritical Care

BOARD FAVORITE!

The correct answer is C, Pulmonary capillary wedge pressure (PCWP) > 18 mm Hg. Occasionally it is very difficult to distinguish between ARDS and cardiogenic pulmonary edema. Both can present with hypoxia and bilateral lung infiltrate, but PCWP is > 18 mm Hg in cardiogenic pulmonary edema.

Ware LB, Matthay MA: The acute respiratory distress syndrome. N Engl J Med 2000 May 4; 342(18): 1334-49.

Classification: Neurocritical Care, Acute Respiratory Disress Syndrome, Pulmonary Changes Ch

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Q? •

Neurocritical Care Neurocritical Care

What is most common finding on ECG in patients with pulmonary emboli? A. Large Q wave in lead III B. Inverted T wave in lead III C. Right axis deviation D. Wide S complex in lead I E. Right bundle branch block F. Sinus tachycardia

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63

A A. • •

Neurocritical Care Neurocritical Care

BOARD FAVORITE!

The correct answer is F, sinus tachycardia. All of the answers can be seen in case of pulmonary emboli. The classic findings of “S1,Q3,T3” are not very sensitive in diagnosis of acute pulmonary embolism.

Feied CF: Pulmonary embolism. In: Rosen and Barkin, eds. Emergency Medicine Principles and Practice. Vol 3. 4th ed. 1998:chap 111.

Classification: Neurocritical Care, Pulmonaryy Emboli,, ECG Changes g

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Q? •

Neurocritical Care Neurocritical Care

A 21 year-old patient with history of recurrent sinusitis developed an allergic reaction after transfusion of a cross matched blood. What is the most likely cause? A. Selective IgA deficiency B. Lab error C. Rh group incompatibility D. Rapid transfusion of blood products

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66

A A. • •

•

Neurocritical Care Neurocritical Care

BOARD FAVORITE!

The correct answer is A, selective IgA deficiency. Selective IgA deficiency syndrome is the most common primary immunodeficiency. Usually the patient is asymptomatic, but a history of recurrent infections may be present; most commonly ear infections, sinusitis, and pneumonia. Allergies are another possible symptom including allergic reaction to blood products. If only fever develops within 1-6 hours of receiving cross matched blood, this is p blood that react to donor leukocytes. y Leukocyte y ppoor caused byy antibodies in recipient red cells can be used to prevent this reaction.

Ballow M. Primary immunodeficiency disorders: antibody deficiency. J Allergy Clin Immunol. Apr 2002;109(4):581-91. Zhao SM. Clinical assessment of preventing febrile nonhemolytic transfusion reaction by leukocyte-depleted blood transfusion. 2002 Dec;10(6):568-70.

Classification: Neurocritical Care, Congenital Immune Disorders, IgA Def. S d Syndrome

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Q? •

Neurocritical Care Neurocritical Care

Which of the following is the appropriate immediate treatment in case of an intraoperative venous air embolism? A. Irrigation of the surgical field B. Lower the patient’s head with lateral left decubitus position C. Aspiration of air from a multiforce central venous pressure catheter D. Manual occlusion of the jugular veins E All off the E. th above b

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70

A A. • •

•

Neurocritical Care Neurocritical Care

BOARD FAVORITE!

The correct answer is E, all of the above. Intraoperative venous air embolism (VAE) has a high incidence during procedures performed in the sitting position. VAE is characterized by the development of bronchoconstriction, hypoxia, hypercarbia, hypotension, shock, cardiac arrhythmias, increased airway pressure, and decreased end tidal CO2. The most sensitive diagnostic modality for VAE is transesophageal echocardiography. g p y

Ballki M.Venous air embolism during awake craniotomy in a supine patient.Can J Anaesth. 2003 Oct;50(8):835-8.

Classification: Neurocritical Care, Intraoperative Venous Embolism, T t Treatment t

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Q? •

Neurocritical Care Neurocritical Care

Match the following arterial blood gas values with the appropriate acid/base disorder. A. Metabolic acidosis B. Metabolic alkalosis C. Respiratory acidosis D. Respiratory alkalosis E. Combined respiratory acidosis and metabolic acidosis 1. pH 7.26, PaCO2 53 mmHg, HCO3- 24 mEq/L 2. pH 7.49, PaCO2 46 mmHg, HCO3- 33 mEq/L 3. pH 7.30, PaCO2 33 mmHg, HCO3- 18 mEq/L 4. pH 7.27, PaCO2 41 mmHg, HCO3- 18 mEq/L ©™

75

A A. • •

Neurocritical Care Neurocritical Care

BOARD FAVORITE!

The correct answers are…. To obtain the answer to this question and to view over 250 more comprehensive neurocritical care questions please purchase the full product here !

Narins RG, Emmett M: Simple and mixed acid-base disorders: a practical approach. Medicine (Baltimore) 1980; 59(3): 161-87.

Classification: Neurocritical Care, Acid Base Disorders,, Lab Values

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Neuropharmacology Section

©™

Q? •

•

Neuropharmacology

A young man is brought to your clinic who has a history of seizures. He stopped taking his medication for a while, but then decided to “double up” after having a seizure recurrence a ffew months th ago. U Upon physical h i l exam, you notice ti ataxia, t i slurred l d speech, h and gingival proliferation. Which of the following seizure drugs is associated with the side effects mentioned above? A. Carbamazepine p B. Phenytoin C. Valproate D. Zolpidem E. Phenobarbital ©™

1

A A. •

• •

Neuropharmacology

The correct answer is B, Phenytoin. • Phenytoin acts by stabilizing the inactive state of calcium channels. • Although the other agents mentioned cause various cognitive shifts, only phenytoin causes gingival proliferation. • Carbamazepine toxicity may cause hyponatremia, ataxia, nystagmus, slurring of speech, dystonia, and varying degrees of CNS depression. • Valproate toxicity may cause coma, coma confusion confusion, somnolence somnolence, and cerebral edema. Zolpidem toxicity may cause psychosis, distortion in visual perception, and lethargy. Phenobarbital toxicity can cause fatal respiratory depression, nystagmus, and ataxia.

Amstrong L, Goldman M, Lacy C, Lance L. Drug Information Handbook. 13th Edition. 2005. pp 1195-1199

Classification: Neuropharmacology, antiseizure medication,, pphenytoin y toxicityy

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Q? •

•

Neuropharmacology

A 61 year-old woman presents to your office complaining of shooting pains on the left side of her face. The attacks of pain are so severe that she avoids brushing her teeth on the th left l ft side, id andd describes d ib multiple lti l attacks tt k throughout th h t the th day. d Which of the following drugs would be considered first line treatment for her condition? A. Oxycodone B. Phenytoin C Ethosuximide C. D. Carbamazepine E. Phenobarbital

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2

Classification: Neuropharmacology, antiseizure medication,, pphenytoin y toxicityy

A A. • • • • • • • •

Neuropharmacology

The correct answer is D, Carbamazepine. The patient is suffering from trigeminal neuralgia, which is best treated with carbamazepine. Carbamazepine is related to the structure of tricyclic antidepressants. The method of action of this drug is related to the inhibition of voltage sensitive calcium channels and the stabilization of sodium channels. Trigeminal neuralgia is a debilitating disease characterized by neuropathy of the fifth cranial nerve (trigeminal nerve). Intense pain is experienced in the jaw, scalp, forehead, eyes, and nose. Most patients who develop trigeminal neuralgia are over 40 years-old. None of the other drugs g listed are considered first line therapy py for this condition. ©™

A t Amstrong L, L G Goldman ld M M, LLacy C C, LLance LL. D Drug IInformation f ti H Handbook. db k 13th Edition. Editi 2005. 2005 pp 255 255-257 257

Classification: Neuropharmacology, trigeminal g neuralgia, g , carbamazepine p

Q? •

•

Neuropharmacology

An eight year old boy is brought to your clinic. His mother mentions that her son often drifts off in space, and has memory lapses during this period. EEG reveals a 3/second spike ik andd wave pattern. tt You prescribe ethosuximide for this patient. Which of the following is the most likely mechanism of action for this drug? A. Reduces current in T-type calcium channels on primary afferent neurons B. Activates a hyperpolarizing potassium current and reduces voltage gated sodium currents C. Inhibits neuronal and glial uptake of GABA D. Enhances the inhibitory action of the GABA receptor. ©™

3

A A. • • •

• • •

Neuropharmacology

The correct answer is A, reduces current in T-type calcium channels on primary afferent neurons. This patient is presenting with classic absence seizures. Ethosuximide is a first line treatment for absence seizures. It reduces current in Ttype calcium channels on primary afferent neurons. Adverse effects include lethargy and GI problems. Topiramate activates a hyperpolarizing potassium current current, reduces voltage gated sodium current, and increases postsynaptic GABA-A receptor currents. Tiagabine inhibits neuronal and glial uptake of GABA. It is used for treatment of partial seizures. Phenobarbital increases the inhibition of the GABA channel by increasing the length off titime th the chloride hl id channel h l remains i open. Amstrong L, Goldman M, Lacy C, Lance L. Drug Information Handbook.

13th

Edition. 2005. pp 1488-1489

Classification: Neuropharmacology, antiseizure medication,, ethosuximide MOA

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•

4

Neuropharmacology

A young mother brings her one year old baby to the emergency room. The baby has an elevated temperature of 102°F, has been vomiting, and just had a seizure. The b b has baby h received i d no immunizations. i i ti A spinal i l tap t is i performed. f d The Th CSF is i cloudy l d and the following lab values are obtained: CSF glucose: 30 CSF protein: 80 Opening pressure: 220 Microbiology reports gram negative coccobacilli that are beta lactamase positive. Which is the most appropriate antibiotic regimen to begin in this patient? A. Vancomycin B. Moxifloxacin C Ceftriaxone C. D. Penicillin G

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A A. •

• • • •

Neuropharmacology

The correct answer is C, Ceftriaxone. • This child has Haemophilus B (HiB) meningitis. • Although infection with HiB is declining due to the widespread use of the vaccine, children who have not received the vaccine are still susceptible. Ceftriaxone is a third generation cephalosporin with coverage for HiB. Vancomycin is used against gram positive organisms. Haemophilus is a gram negative ti organism. i Quinolones could be used to treat HiB, but they are not recommended for use in children due inhibition of growth at the epiphyseal plate in some studies. Penicillin G would not be used as lab reports indicated the bacteria, beta lactamase resistant,, was ppresent. In addition,, ppenicillin G does not have HiB coverage. g

Amstrong L, Goldman M, Lacy C, Lance L. Drug Information Handbook. 13th Edition. 2005. pp288-289

Classification: Neuropharmacology, antimicrobials, third generation cephalosporins h l i

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Q? •

Neuropharmacology

An eight year-old boy is taken by his father to the emergency room. An aseptic meningitis is diagnosed by the ER physician. Physical exam shows sores in i th the mouth th located l t d on the th tongue t andd gums andd a skin ki rashh presentt on the th palm of the patient’s hands and the soles of his feet. What treatment is most appropriate? A. Amantadine B. Acyclovir y C. Tenofovir D. No treatment needed

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A A. • • • • • • •

Neuropharmacology

The correct answer is D, no treatment needed. This patient has hand-foot-mouth disease, often caused by coxsackie virus A16, which is a subgroup of the enteroviruses. Currently there is no specific treatment available for any of the enteroviruses. The clinical course of coxsackie A16 is relatively mild. Amantadine is indicated for treatment of type A influenza. It is also used in Parkinson’ss disease. Parkinson disease Acyclovir is the agent of choice in herpes simplex and herpes zoster infections. Tenofovir is a nucleotide analogue reverse inhibitor used in HIV.

©™ Amstrong L, Goldman M, Lacy C, Lance L. Drug Information Handbook.

13th

Edition. 2005. pp 43, 79-80, 1435

Classification: Neuropharmacology, Antiviral medications,, Coxsackie A16

Q? •

Neuropharmacology

A 35 year-old man has suffered from severe asthma attacks for most of his life. Which of the following agents could cause a life threatening bronchospasm for this patient? A. Phenoxybenzamine B. Terazosin C. Acebutolol D. Propanolol

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6

A A. •

• • •

Neuropharmacology

The correct answer is D, Propanolol. • Propanolol is a non-selective beta blocker. • Alpha mediated bronchoconstriction (α1) could thus take precedence over β2 relaxation. Phenoxybenzamine is a non selective α-blocker primarily used for treatment of pheochromocytoma. It causes no adverse effects in asthmatics. Terazosin is an α-1 α 1 adrenergic receptor antagonist for treatment of hypertension and BPH. It is not associated with any adverse effects in asthmatics. Acebutolol is a β -1 antagonist with sympathomimetic activity. It is especially indicated for patients who have asthma or diabetes.

Amstrong L, Goldman M, Lacy C, Lance L. Drug Information Handbook. 13th Edition. 2005. pp1267-1289

Classification: Neuropharmacology, autonomic agents, g , ppropanolol p

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Q? •

Neuropharmacology

Which of the following drugs is given by intravenous infusion, has a very short half life, and is used primarily during hypertensive crises? A. Esmolol B. Prazosin C. Carvedilol D. Yohimbine

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7

A A. •

• • •

Neuropharmacology

The correct answer is A, Esmolol. • Esmolol is a β-1 receptor antagonist for use in hypertensive crises. • It can also be used during episodes of acute supraventricular tachycardia. Prazosin is used for hypertension and benign prostatic hyperplasia. It is not indicated for control of acute episodes of hypertension, however. Carvedilol is an alpha and beta antagonist. At this time it is used to treat heart failure. It is not given to control a hypertensive crisis. crisis Yohimbine is an a-2 agonist. At this time its clinical use is limited.

©™ Amstrong L, Goldman M, Lacy C, Lance L. Drug Information Handbook. 13th Edition. 2005. pp 546-548

Classification: Neuropharmacology, autonomic agents, g , Esmolol

Q? •

Neuropharmacology

A 32 year-old soccer player injures his knee playing in the European World Cup. After surgery to repair a torn ligament, he develops osteoarthritis two years later. He has a history to Which hi t off allergy ll t sulfa lf drugs. d Whi h off the th following f ll i NSAIDS would ld nott be b recommended in this patient? A. Aspirin B. Ibuprofen C. Sulindac D. Piroxicam

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8

A A. • • •

Neuropharmacology

The correct answer is C, Sulindac. Sulindac is an NSAID that contains a sulfa moiety, and thus produces an allergic reaction in those who are allergic to sulfa drugs. None of the other choices contain a sulfa moiety.

©™ Amstrong L, Goldman M, Lacy C, Lance L. Drug Information Handbook.

13th

Edition. 2005. pp 1413-1414

Classification: Neuropharmacology, Analgesics, g , Sulindac

Q? •

Neuropharmacology

Which of the following drugs is used for treatment of myasthenia gravis? A. Edrophonium B. Pyridostigmine C. Echothiophate D. Parathion E. Donezipil

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10

A A. •

• • • •

Neuropharmacology

The correct answer is B, Pyridostigmine. • Pyridostigmine is a carbamate which acts as a pseudo-reversible inhibitor of acetylcholinesterase. • It is rapidly hydrolyzed after carbamoylating the active site of the acetylcholinesterase enzyme. • It is used extensively in the treatment of myasthenia gravis. Edrophonium is a reversible inhibitor of AchE. AchE Used for the diagnosis of myasthenia gravis, but not for treatment due to an extremely short half life. Echothiophate is an irreversible inhibitor of AchE. Primarily utilized for glaucoma. Parathion is an irreversible inhibitor of AChE. Primarily utilized for insect control. Donezipil p is a reversible inhibitor of AChE. Primarilyy utilized for treatment of Alzheimer’s disease. ©™

Amstrong L, Goldman M, Lacy C, Lance L. Drug Information Handbook. 13th Edition. 2005. pp 1281-1282

Classification: Neuropharmacology, anticholinesterase drugs, g , py pyridostigmine g

Q? •

Neuropharmacology

Which variables alter the mechanism of intrathecal drug distribution? A. Age B. Weight C. Vicosity D. Patient position E. All of the above.

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20

A A.

Neuropharmacology

• •

The correct answer is E, all of the above. Factors that influence the distribution of intrathecally administered medication include: 1. Characteristics of the injected solution: baricity, volume/dose/concentration, temperature of injectate, viscosity, additives 2. Clinical technique: patient position, level of injection, needle type/alignment, intrathecal catheters, catheters fluid currents currents, epidural injection 3. Patient characteristics: age, height, weight, sex, intra-abdominal pressure, spinal anatomy, lumbosacral CSF volume, pregnancy

Greene NM. Distribution of local anesthetic solutions within the subarachnoid space. Anesth Analg 1985; 64: 715–30.

Classification: Neurosurgery, Intrathecal Drugs, Variables Affecting Mechanism of Distribution

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Q? •

Neuropharmacology

A 76 year-old male presents to your clinic in consultation with results of a recent angiogram that suggest carotid stenosis 45% by NASCET criteria. He denies current visual i l changes, h hheadaches d h or weakness, k bbutt says th thatt 2 weeks k ago hhe did have h “loss “l of sight” in the left eye for one day . You would recommend: A. Medical therapy (ie, antiplatelet agents and cardiovascular risk factor control/prevention). B. Recommend carotid endarterectomyy after consider ppatient's risk factor pprofile and comorbidities. C. Recommend endarterectomy without considering patient's risk factor profile and comorbidities. D. Carotid endarterectomy plus antiplatelet therapy and cardiovascular risk factor control/prevention control/prevention. ©™

21

A A. • • • • • • • •

Neuropharmacology

BOARD FAVORITE!

The correct answer is A, medical therapy. NASCET criteria for carotid stenosis: Symptomatic (TIA or minor stroke) Lower-grade carotid stenosis (<50% by NASCET criteria) -medical therapy (ie, antiplatelet agents and cardiovascular risk factor control/prevention) Moderate-grade symptomatic carotid stenosis (50% to 69% by NASCET criteria) walk a finer line. Consider patient's risk factor profile and comorbidities. High-grade symptomatic carotid stenosis (70% to 99% by NASCET criteria) -CEA plus antiplatelet therapy and cardiovascular risk factor control/prevention is the treatment of choice. Asymptomatic >60% carotid stenosis (NASCET criteria) walk a fine line. If surgical complications exceed 3%, CEA would be harmful to the ppatient. Ferguson GG, Eliasziw M, Barr HWK, Clagett GP, et al. The North American Symptomatic Carotid Endarterectomy Trial. Stroke. 1999;30:1751-8.

Classification: Neurosurgery, Neurosurgery NASCET, NASCET Carotid Stenosis

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Q? •

Neuropharmacology

A 75-year old man who had just suffered an acute myocardial infarction one week previously now presents to clinic with tachycardia. His EKG is shown here. An appropriate treatment regimen would include which of the following drugs? A. Magnesium ag es u B. Procainamide C. Amiodarone D. Verapamil E. Lidocaine ©™

31

A A. • •

Neuropharmacology

The correct answer is…. To obtain the answer to this question and to view over 250 more comprehensive neuropharmacology questions please purchase the full product here !

Stoelting R, Hillier s. Pharmacology and Physiology in Anesthetic Practice 4th Edition. Philadelphia, PA: Lipincott 2006. p. 620

Category: Pharmacology, torsades de pointe, magnesium 

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Q? •

Neurobiology

Match the following statements with the correct answer: 1. Neuromuscular junction 2. Botox 3. Renshaw cell 4. Blocks Renshaw cell neurotransmitter 5. Curare

A. A A Acetylcholine t l h li release l B. Acetylcholine receptor C. 5 subunits: 2α subunits, a β, a δ and either a γ or an ε. D Strychnine D. E. Glycine

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4

A A. • •

•

•

Neurobiology

BOARD FAVORITE!

The corrects answers are the following: 1 - C, 2 - A, 3 - E, 4 - D, 5 - B. Neuromuscular junction- the neuromuscular junction contains the nicotinic receptor (an acetylcholine receptor subtype (nAChR)) There are 2 types of nAChRs; autonomic and neuromuscular. The autonomic nicotinic receptor has 5 subunits: 3α and 2β. The neuromuscular nAChR also consists of 5 subunits:2α subunits, a β, a δ and either a γ or an ε subunit. Botox –botulism toxin decreases Ach release ((at neuromuscular jjunction and autonomic nerve terminals: both sympathetic & parasympathetic) by binding to synaptotagmin, while curare (δ-tubocurarine) blocks the Ach receptor. Botox has actually become useful for cosmetic purposes and dystonias. The Renshaw cell neurotransmitter is glycine; an aminoacid and whose channel is blocked by strychnine. strychnine Dressler D, AdibSaberi F. New formulation of Botox: complete antibody-induced treatment failure in cervical dystonia. J NeurolNeurosurg Psychiatry. 2007 Jan;78(1):108-9 Adams, M.E. and Olivera, B.M. Neurotoxins: overview of an emerging research technology. Trends Neurosci. 17:151-155. (1994).

Classification: Neurobiology, Neuromuscular junction, Receptor actions

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Q? •

Neurobiology

Which of the following is the principle visceral nucleus of the brainstem? A. Nucleus ambiguus B. Raphe nucleus C. Nucleus solitarius D. Locus ceruleus E. Trigeminal nucleus

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5

A A. • •

•

Neurobiology

BOARD FAVORITE!

The correct answer is C, nucleus solitarius. Nucleus solitarius receives afferent visceral information from the heart, lungs, GI tract, as well as for taste. The solitary nucleus and tract are neural structures in the brainstem that carry and receive visceral sensation and taste from the facial (VII), glossopharyngeal (IX), vagus (X) cranial nerves, as well as the cranial part of the accessory nerve (XI). Nucleus ambiguous g ggives rise to the efferent motor fibers of the vagus g nerve ((CN X)) terminating in the laryngeal and pharyngeal muscles, efferent motor fibers of the glossopharyngeal nerve (CN IX) terminating in the stylopharyngeus and contains the preganglionic parasympathetic neurons for cardiac regulation.

Andresen MC, Doyle MW, Bailey TW, Jin YH. Differentiation of autonomic reflex control begins with cellular mechanisms at the first synapse within the nucleus tractus solitarius. Braz J Med Biol Res. 2004 Apr;37(4):549-58.

Classification: Neurobiology, Brainstem, Nucleus Solitarius

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Q? •

Neurobiology

Choose the MOST accurate statement regarding the physiology of an action potential: A. Voltage-gated potassium channels (also called delayed rectifier potassium channels) have a delayed response, such that potassium continues to flow out of the cell and initiate depolarization. B. Hyperpolarization is caused by K+ influx into a cell. C. Closing of voltage-gated potassium channels is both voltage- and timedependent. D. Depolarization is caused by Na+ efflux from a cell.

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35

A A. •

BOARD FAVORITE!

The correct answer is C, closing of voltage-gated potassium channels is both voltage- and timedependent. As potassium exits the cell, the resulting membrane repolarization initiates the closing of voltage-gated potassium channels. These channels do not close immediately in response to a change in membrane potential. Peak 40 Rather, voltage-gated potassium channels (also called delayed rectifier potassium channels) have a delayed response, such that potassium continues to flow out of the 0 cell even after the membrane has fully repolarized. Thus the membrane potential K efflux dips below the normal resting membrane potential of the cell for a brief moment; this Threshold -55 dip of hyperpolarization is known as the Resting potential undershoot. -70 Hyperpolarization is caused by K+ efflux Stimulus Na influx Hyperpolarization from a cell. 0 1 2 3 4 5 Depolarization is caused by Na Na+ influx into a Time (ms) cell. Membran ne voltage (mV)

•

Neurobiology

•

+

+

•

Kandel ER, Schwartz JH, Jessell TM. Principles of Neural Science, 4th ed. McGraw-Hill, New York. 2000. p. 128-33. Robbins and Cotran. Pathologic Basis of Disease, 6th Edition. W.B. Saunders Company. 1999. p.55-56.

Classification: Neurobiology, Neurophysiology, Action Potential

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Q? •

Neurobiology

Neurotransmitter that is found in a postganglionic sympathetic nerve terminal: A. GABA B. Serotonin C. Acetylcholine D. Norepinephrine E. C + D

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36

A A. • • • • • • • •

Neurobiology

BOARD FAVORITE!

The correct answer is E, acetylcholine (Ach) and norepinephrine (NE). Both Ach and NE are found in postganglionic sympathetic fibers. Ach- sweat, piloerectors, and vasculature, mediated by muscarinic receptors (inhibited by pertussis toxin). REMEMBER: sweating of the palms of the hands is parasympathetic NOT sympathetic! (BOARD FAVORITE!) NE all others NEPeripheral sympathetic pathway –TRAVELS WITH SKELETAL NERVES (sweat, piloerectors, and vasculature) (BOARD FAVORITE!) Head and Neck sympathetic pathway -TRAVELS WITH BLOOD VESSELS (BOARD FAVORITE!) All preganglionic fibers (both sympathetic and parasympathetic) use Ach. Kandel ER, Schwartz JH, Jessell TM. Principles of Neural Science, 4th ed. McGraw-Hill, New York. 2000. p. 1051-52.

Classification: Cl ifi ti N Neurobiology, bi l Neurophysiology, Sympathetic Nerve Terminal

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Q? •

Neurobiology

Which of the following statements is true regarding the intracellular second messenger cyclic AMP. A. It is involved with photoreception B. It is hydrolyzed by phospholipase C. It synergistically activates protein kinase C D. It is increased with D1 receptor stimulation

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37

A A. • • •

• • •

Neurobiology

BOARD FAVORITE!

The correct answer is D, it is increased with D1 receptor stimulation. Hormones that utilize this system include: epinephrine and norepinephrine, glucagon and TSH. TSH In a cAMP dependent pathway, the activated Gs alpha subunit binds to and activates an enzyme called adenylyl cyclase which in turn catalyzes the conversion of ATP into cyclic adenosine monophosphate (cAMP). The intracellular second messenger cAMP activates protein kinase A (PKA). cAMP is decomposed to AMP by the action of the enzyme phosphodiesterase. This cAMP phosphodiesterase - has an activity opposite to kinase, kinase therefore it dephosphorylates cAMP into AMP, reducing the cAMP levels. Deregulation of cAMP pathways (hyperactive) and an aberrant activation of cAMPcontrolled genes is linked to the growth of some cancers Activation of conventional protein kinase C - requires DAG, Ca2+, and phospholipid. Kandel ER, Schwartz JH, Jessell TM. Principles of Neural Science, 4th ed. McGraw-Hill, New York. 2000. p. 230-36.

Classification: Cl ifi ti N Neurobiology, bi l Neurophysiology, Second Messenger Systems

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Q? •

Neurobiology

Match the receptors with their action: 1. Mu1 2. Mu2 3. Kappa 4. Delta 5. Sigma

A. B. C. D. E.

Hallucinations Opiate analgesic euphoria Miosis Reinforcing behavior Respiratory depression

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38

A A. •

Neurobiology

BOARD FAVORITE!

The correct answers are: 1. B, Mu1- Opiate analgesic euphoria 2. E, Mu2- Respiratory depression 3. C, Kappa- Miosis 4. D, Delta- Reinforcing behavior 5. A, Sigma- Hallucinations

Kandel ER, Schwartz JH, Jessell TM. Principles of Neural Science, 4th ed. McGraw-Hill, New York. 2000. p. 483-6. Robbins and Cotran. Pathologic Basis of Disease, 6th Edition. W.B. Saunders Company. 1999. p.55-56.

Classification: Neurobiology, Pharmacology, Receptor Actions

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Q? •

Neurobiology

Hexamethonium blocks which receptor? A. Muscarinic B. Nicotinic C. Glycine D. Glutamate

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39

A A. • • •

Neurobiology

BOARD FAVORITE!

The correct answer is B, nicotinic receptor. Hexamethonium is a nicotinic Ach receptor antagonist, acts by preventing acetylcholine from binding to the cholinergic receptor. receptor It has no effect on the muscarinic acetylcholine receptors, or mAChRs.

Kandel ER, Schwartz JH, Jessell TM. Principles of Neural Science, 4th ed. McGraw-Hill, New York. 2000. p. 971.

Classification: Neurobiology, Pharmacology, Receptor Actions

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Q? •

Neurobiology

A 37 year-old male presented with generalized seizure and expressive dysphasia. MRI is shown. Reoccurrence of this tumor as a high-grade is LEAST likely due to which hi h genetic ti abnormality? b lit ? A. P53 tumor suppressor B. Retinoblastoma gene C. Amplification of platelet derived growth factor D. Amplification of epidermal growth factor receptor

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41

A A. • •

•

Neurobiology

BOARD FAVORITE!

The correct answer is D, amplification of epidermal growth factor receptor. This MRI demonstrates a low-grade glioma. Recurrent progressive low-grade glioma is associated i t d with ith mutations t ti in i the th ttumor suppressor gene p53 53 with ith amplification lifi ti off CDK4 or loss of Rb and amplification or overexpression of PDGF. There are 2 subtypes of glioblastoma – Older patients with de novo tumors • Deletions in cell cycle related INK4a-ARF genes p16INK4A and p19/p14ARF • EGFR amplifications – Younger patients with progressive low grade glioma • Mutations in the tumor suppressor gene p53 with amplification of CDK4 or loss of Rb • Amplification or overexpression of PDGF

Berger MS, Pardos M,Textbook of Neuro-Oncology. Saunders, Philadelphia. 2005. p. 608-9.

Classification: Neurobiology, Genetics, Tumor Growth Factors

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Q? •

Neurobiology

Match the following sensory receptors with their description. 1. Meissner capsule A. Rapidly adaptive, vibration, receptors 2. Merkel receptors B. Slowly adaptive, touch and pressure, receptor 3. Free nerve endings C. Slowly adaptive, heavy pressure, receptor 4. Ruffini corpuscles D. Rapidly adaptive, touch, receptors 5. Pacinian corpuscles E. Pain receptors

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60

A A. • •

Neurobiology

BOARD FAVORITE!

The correct answers are…. To obtain the answer to this question and to view over 250 more comprehensive neurobiology questions please purchase the full product here !

R

M

Ruffini and Merkel drink TONIC SLOWLY! (slowly adapting tonic receptors) Guyton, Arthur C., John E. Hall. Textbook of medical physiology. W.B. Saunders Company; 10th edition, 2000. p. 521, 530-2

Classification: Neurobiology, Neurophysiology, Touch Receptors

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Q? •

Neuroanatomy

Which of the following statements is INCORRECT regarding the anatomy of the cavernous carotid artery? A. The inferior hypophyseal artery is most commonly a branch of the meningohypophyseal trunk. B. The inferior hypophyseal artery passes medially to the posterior pituitary capsule. C. Persistent trigeminal arteries can also originate from the posterior vertical segment of the cavernous internal carotid artery and pass posteriorly through the posterior wall of the cavernous sinus to join the basilar artery between the origin of the superior and anterior inferior cerebellar arteries. D. The tentorial artery, or the artery of Bernasconi and Cassinari, is the most inconstant branch of the meningohypophyseal trunk. ©™

1

A A. •

•

Neuroanatomy

The correct answer is D. The Inferior hypophyseal artery Meningohypophyseal trunk  meningohypophyseal trunk is the most inconsistent branch of the meningohypophyseal trunk. Cerebral Peduncle The meningohypophyseal trunk is the most Pituitary constant artery (labeled below). Tentorial artery

Carotid

CN3

Dorsal meningeal artery

•

•

The meningohypophyseal trunk usually arises from the posterior aspect of the central third of the posterior bend of the artery at the level of the dorsum sella and frequently gives rise to three branches: the tentorial artery (Bernasconi and Cassinari), th ddorsall meningeal the i l artery, t andd the th inferior i f i hypophyseal artery. All other answers are true statements.

CN4

CN5

V1

V1

V2 Middle Fossa

Isolan G, de Oliveira E, Mattos JP. Microsurgical anatomy of the arterial compartment of the cavernous sinus: analysis of 24 cavernous sinus. ArqNeuropsiquiatr. 2005 Jun;63(2A):259-64

Comments:  Cerebral Arteries,  meningohypophyseal, Bernasconi and  Cassinari

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Q? •

Neuroanatomy

α-motor neurons are most commonly found in which Rexed lamina? A. III B. V C. VI D. IX E. X

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2

A A. • •

• • • • • •

Neuroanatomy

The correct answer is D, IX. Rexed lamina IX, is located in the ventral motor areas off th the gray matter. tt D Described ib d iin 1950 1950s bby Bror Rexed as a way to name the different parts of the spinal cord, the Rexed laminae consist of ten layers of grey matter (I-X). It must be noted that Rexed areas are not designated in terms of location but rather by their cellular structure. location, structure

BOARD FAVORITE!

1

2 4

5

6

10

Layer 1 – marginal zone, fast pain (Aδ) and temperature fibers Layer 2 – substantia gelatinosa,slow pain (C fibers) Layer 4 – nucleus propius, interneurons to thalamus Layer 7 – contains the zona intermedia L Layer 9 – α and d γ motorneurons t Layers are all BOARD FAVORITE topics !

3

8 9

9

7 9

Anamizu Y, Seichi A, Tsuzuki N, Nakamura K. Age-related changes in histogram pattern of anterior horn cells in human cervical spinal cord.Neuropathology. 2006 Dec;26(6):533-9.

Comments:  Spine, Rexed lamina

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Q? •

Neuroanatomy

Match the following cortical neuroanatomy: 1. Sylvian fissure 2. Rolandic fissure 3. Pars triangularis 4. Pars opercularis 55. P Pars orbitalis bit li 6. Supramarginal gyrus 7. Angular gyrus

F E B C A

G H

D

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36

A A.

Neuroanatomy

• Cortical neuroanatomy: 1. D, Sylvian fissure 2. E, Rolandic fissure 3. B, Pars triangularis 4. C, Pars opercularis 5. A, Pars orbitalis 6. G, Supramarginal gyrus 7. H, Angular gyrus • Note: F is the precentral sulcus.

Motor cortex

BOARD FAVORITE!

Sensory cortex

F E

B C A

G H

D

©™ H. Richard Winn, M.D. Youman’s Neurological Surgery 5th Edition. Philadelphia, PA: Elsevier 2004. p. 2533-7.

Comments:  Cortical anatomy, fissures, gyri

Q? •

Neuroanatomy

Match the following boundaries of Kawase’s triangle to their anatomic structure. A. Anteromedial boundary B. Anterolateral boundary C. Posterior boundary 1. Greater superficial petrosal nerve (GSPN) 2. Internal auditory canal 3. Lateral edge of the trigeminal nerve

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37

A A. • •

•

•

Neuroanatomy

The correct answers are A - 3, B - 1, C - 2. The anteromedial boundary is formed by the lateral edge of the trigeminal nerve. The anterolateral boundary is formed by the greater superficial petrosal nerve (GSPN). The posterior boundary is formed by the internal auditory canal or a line connecting the hiatus fallopii to the dural ostium of Meckel’s cave.

1

2

3

4

O. C

5 6

1- Anteromedial triangle

4- Lateral triangle (Parkinson's)

2- Medial triangle

5- Posterolateral triangle (Glasscock's)

3 Superior triangle 3-

6 Posteromedial triangle (Kawase 6(Kawase's) s)

H. Richard Winn, M.D. Youman’s Neurological Surgery 5th Edition. Philadelphia, PA: Elsevier 2004. p. 921-5.

Comments:  Kawase’s triangle, boundaries,  CN V, Greater superficial petrosal nerve

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Q? •

Neuroanatomy

The vertebral arteries enter within the transverse foramina of which vertebrae: A. C4 to C1 B. C6 to C1 C. C6 to C2 D. C7 to C2 E. C8 to C2

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52

A A. • •

•

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Neuroanatomy

The correct answer is B, C6 to C1. The vertebral artery passes through the transverse foramina of C6 through C1 before exiting the transverse foramina of the atlas (C1). At this location the vertebral artery curves posterior and superior to lie in a groove on the upper surface of the atlas . At C7 the vertebral artery swings anteriorly and laterally because it normally runs outside the rudimentary transverse foramina of C7. When a foramina is present, transverse foramina of the seventh cervical vertebra almost always contains vascular and nerve branches as well as fibrous and adipose tissues.

BOARD FAVORITE!

Anterior arch of atlas Superior articular  Superior articular fovea

Vertebral arteryy

Cranial view of the course of the vertebral artery over C1

Katsuta T, Rhoton AL Jr, Matsushima T.The jugular foramen: microsurgical anatomy and operative approaches. Neurosurgery. 1997 Jul;41(1):149-201. MS Jovanovic. A comparative study of the foramen transversarium of the sixth and seventh cervical vertebrae. Volume 12, Number 3 / September, 1990

Comments:  Extracranial Vasculature,  Vertebral artery, Cervical spine, transverse  foramina 

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Neuroanatomy

Match the structures associated with this portion of the lateral ventricle: 1 Right 1. Ri ht foramen f off Monro M 2. Left foramen of Monro 3. Choroid plexus 4. Thalamostriate vein 5. Anterior cerebral artery 6. Anterior septal vein 7. Fornix 8. Internal cerebral vein

A B C

E F G

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Neuroanatomy

The correct answers are…. To obtain the answer to this question and to view over 200 more comprehensive neuroanatomy questions please purchase the full product here !

BOARD FAVORITE! Head of caudate

A

E F

B C

D

G

Thalamus ©™

Comments:  Lateral ventricle anatomy,  cerebral vascular anatomy