Resident Tutorial: Ataxia

Resident Tutorial Long case Board Examination Surat Tanprawate, MD, FRCPT Northern Neuroscience Center Chiangmai University

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www.neurologycoffeecup.com By Dr. Surat

Present “Fear factors in Long Case Neuro Examination” and “How to approach to ataxia”

Fear Factors

• Hard to get neurological history • Hard to do the physical neurological exam

• Hard to conclude the results • Hard to make neurological diagnosis

Fear Factors •

Hard to get neurological history

•

Hard to do the physical neurological exam

•

Hard to conclude the results

Practice to list the problem

•

Hard to make neurological diagnosis

To know the common diseases

Symptomatology Practice the physical exam

Key Concept Symptoms approach

“Algorithm”

Key Concept Symptoms &Signs “Specific examination”

Key Concept To diagnose “Problem list and Conclusion”

Symptomatology Syndrome

Symptomatology • Disorder of consciousness • Level of consciousness • Content of consciousness

• Mental disorder • • • • •

Memory Intelligence Personality Behavioral Dementia

• Visual disorder • Visual loss

• Ocular motility disorder • Diplopia • Abnormal ocular ossilation

Symptomatology Lower cranial nerve disorder

• • • • •

Deafness/tinnitus Vertigo Balance/staggering Swallowing Voice change

Multiple Cranial Nerve Disorders

Symptomatology • Sensory disorder • Pain disorder • •

Headache and facial pain Others pain disorder

• Numbness/tingling

• Motor disorder • • • •

Weakness Movement disorder Gait abnormality Ataxic disorder

• Sphincter disorder • Episodic disorder – – – – –

Seizure/epilepsy Syncope TIA Abnormal movement Migraine

Syndrome • • • •

•

Amnistic and Dementia syndrome Neuro-opthalmology syndrome Syndrome of Multiple cranial nerve disorder Stroke syndrome

• • •

Cortical stroke syndrome Lacunar stroke syndrome Brain stem stroke syndrome

Spinal cord syndrome

Ataxia

Algorithm Gait abnormality Ataxia Specific examination Specific examination -confirm ataxic disorder -for categorized ataxia Conclusion and Diagnosis

Greek
word A=nega.ve Taxi=order

Ataxia
Algorithm Ataxic symptoms? -Nystagmus -Dysarthria -Trunkcal ataxia -Limb and gait -ataxia Ataxic symptoms mimicker? Mild
weakness • Apraxia • • Abnormal
movement • Gait
abnormality

True Ataxia

Ataxia: disease other than cerebellum

Cerebellar’s disease -Where’s the lesion (cerebellum, cerebellar peduncle, cerebellar tract) -What’s the lesion

Neuroanatomy Neurophysiology

Function of cerebellum

• Coordinating skill voluntary movement

• Muscle activity • Control equilibrium • Muscle tone

Function of cerebellum

• Lesion

• Incoordination (ataxia) of volitional movement

• Tremor (ataxic or intention tremor)

• Disorder of equilibrium and gait • Diminish muscle tone

Cerebellar pathway To
a7ributed
 sensorimotor
 network:


• cerebral
cortex • basal
motor
nuclei • thalamus • re:cular
forma:on

Func.onal
Zone

Part of Cerebellum Functional divisions Vermis (face, proximal body)

Deep Nuclei Fastigial

Intermediate zone Interposed (spinal cord)

Lateral zone (cortex, pons)

Dentate

Flocculonodular Vestibular nuclei, lobe visual system

Connections

Functions

Vestibular nucleus Reticular formation Medial descending system

Axial and proximal muscle control Progressive movement

Red nucleus Motor cortex Lateral descending system

Distal muscle control Progressive movement

Red nucleus Thalamus Motor, premotor cortex

Motor planning Initiation Timing

Vestibular nuclei

Axial equilibrium Eye movements Vestibular reflexes

Basic
Anatomy
–
Cerebellar
peduncles Cerebellar Tracts connect to Major pathways peduncles brain stem

Connections

Superior

Brachium conjunctivuum

Afferent Efferent

Rubral, thalamic, Dentate, spinal cord

Middle

Brachium pontis

Afferent only

Pontine nuclei

Inferior

Restiform body

Afferent Efferent

Vestibular, olive, Spinal cord Receives from Flocculonodular lobe

Blood Vessel

Ataxia
Algorithm Ataxic symptoms? -Nystagmus -Dysarthria -Trunkcal ataxia -Limb and gait -ataxia Ataxic symptoms mimicker? Mild
weakness • Apraxia • • Abnormal
movement • Gait
abnormality

True Ataxia

Ataxia: disease other than cerebellum

Cerebellar’s disease -Where’s the lesion (cerebellum, cerebellar peduncle, cerebellar tract) -What’s the lesion

What is the cerebellar syndrome? What is the associated signs?

Associated signs

Pure cerebellum

With Brainstem signs

With mild hemiparesis

Classified Cerebellar syndrome

Classified Brainstem Syndrome?

Involve frontoPontoCerebellar Pathway “Ataxic hemiparesis”

Classified
cerebellar
syndrome

Unilateral Cerebellar hemispheric intermediate, syndrome lateral zones

Rostral vermis syndrome

Ant, sup vermis

Caudal vermis syndrome

Flucculo nodular, post vermis

All Pan cerebellar regions syndrome

Cerebellar syndrome and its disorders Cerebellar
 syndromes

Regions
 involved

Distribu.ons
of
 Common
 deficits causes

Cerebellar
 hemisphere
 syndrome

Cerebellar hemisphere

Ipsilateral
head
&
 Infarct,
neoplasm,
 body abscess,
 demyelina:on

Rostral
vermis
 syndrome

Ant,
sup
vermis Gait,
trunk

Alcoholism,
 thiamine
def

Caudal
vermis
 syndrome

Flucculonodular,
 Axial
 diisequilibrium post
vermis

Midline
neoplasm

Pancerebellar
 syndrome

All
regions

Bilateral
 symmetrical
signs
 of
cerebellar
 dysfunc:on

Toxic,
metabolic,
 infec:ous,
 paraneoplas:c,
 degenera:ve

Posterior inferior cerebellar artery (PICA) Anterior inferior cerebellar artery (AICA) Superior cerebellar artery (SCA) Posterior cerebral artery (PCA)

PICA = lateral medulla & inferior cerebellum AICA = lateral caudal pons & part of cerebellum SCA = superior cerebellum & rostral laterodorsal pons PCA = midbrain, thalamus, medial surface of occipital lobe, inferior and medial surfaces of temporal lobe

Brainstem Vascular Territories

SYNDROME STRUCTURES DAMAGED CLINICAL SYMPTOM Lateral medullary (PICA, Corticospinal tract (pyramid) Wallenberg’s)syndrome Spinothalamic tract Damage Level: Lateral medullar

Vascular supply PICA

Contralateral hemiplegia Contralateral hemisensory loss

Trigerminal spinal nucleus

Ipsilateral facial hemisensory loss

Nucleus ambiguous

Ipsilateral palatal, pharyngeal, vocal cord paralysis Dysarthria, dysphagia

Sympathetic fiber

Ipsilateral Horner’s syndrome

Vestibular nuclei

Vertigo, N/V

Cerebellum

Ipsilateral cerebellar sign

SYNDROME STRUCTURES DAMAGED CLINICAL SYMPTOM Vestibular nucleus Lateral inferior pontine syndrome (AICA stroke syndrome) Cochlear nucleus Damage Level: Lateral inferior pons

Nuclear of CN7 Cerebellum CN 5 Spinothalamic tract

Ipsilateral vertigo, N/V, nystagmus Ipsilateral deafness Ipsilateral facial palsy Ipsilateral ataxia Ipsilateral hemisensory loss of face Contralateral hemisensory loss

Neurological examination in ataxia

Patient Video

Nystagmus

Patient Video

Intention tremor

Patient Video

Finger to Nose Test

Patient Video

Ataxic gait

Patient Video

Ataxic speech

What’s lesion? Time, Caused, Onset Medical History Neurological Signs

Time Caused Onset • Sudden •

Vascular

• Acute • •

Intoxication Viral, Post infectious

•

Subacute

•

Intoxication

• •

Hereditary

• •

Hypothyroid

• Chronic Paraneoplastic syndrome Intoxication

Symmetrical ataxia plus syndrome

• Acquired • Wernicke’s encephalopathy • Miller Fisher syndrome • Hereditary • SCA • FA

Pt.
with
progressive
ataxia Imaging • to exclude identifiable structural lesion • Atrophy of cerebellum or spinal cord

AD SCA1,2 MJD SCA6,
7 SCA10,12 DRPLA SCA17

Evaluate •Accurate family history • Phenotype

AR

Singleton patient

Acquired causes -Alcohol/medication -Hypothyroid -Vit.B12 -Anti-HIV -Paraneoplastic study -GAD Ab

FA AT AVED Abetalipoproteinemia AOA Mitochondrial
disorder

Next Episode: Neuro-opthalmology approach Diplopia, Visual loss, Ptosis

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