Cognitive Disorders

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Delirium, Dementia & Amnestic Disorder Cognitive Disorders - All disorders of brain dysfunction which manifest themselves through cognitive deficits - Most, if not all, psychological disorders have neurological influences but the following disorders were grouped together for their impairment of cognitive functions, some examples of these functions are: o Memory, attention, perception & thinking - Develop later in life. So mental retardation would fit because although is it from a dysfunction of the brain, it is present from birth. Delirium - What is delirium? o A clinical syndrome (or set of symptoms) of disturbances in consciousness that is accompanied by symptomatic manifestations of early brain dysfunction, which can not be better explained by dementia o An often temporary condition displayed as confusion & disorientation o Onset is acute & rapid (hours to days) o Severity may fluctuate - Read case vignette – Pg 62 - What are the main symptoms? o Confusion  May lose the capacity for clear & coherent thought • Easily distracted  May have disorganized or incoherent speech • Aphasia – impairment in understanding & producing speech • Dysarthia – impaired articulation  May not be able to concentrate • Can not sustain focus on even very simple tasks  May have loss of awareness of the surroundings, environment & context in which the person exists. o Disorientation  may lose awareness of time (not knowing what time of day, day of week, month, season or year it is)  may lose awareness of place (not knowing where you are)  may lose awareness of person (not knowing who you are) o Cognitive changes, such as:  Memory dysfunction • Both short term & long term  Perceptual dysfunction (e.g. hallucinations) • Alcohol withdrawal - delirium tremens (“the DT’s” or “the shakes”) hallucinations often take the form of masses of small crawling creatures • Otherwise distortions of size or intensity of surrounding environment

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 Constructional apraxia - difficulty drawing  Dysnomia – impaired ability to name objects - What are the different types of diagnostic deliriums o Delirium due to a medical condition  Must have evidence that the cognitive disturbance is directly from a medical condition  Central Nervous System Disorders • Head trauma, Infection of the brain, Stroke  Metabolic disorders • Renal or hepatic disease (kidney & liver issues), Hypoglycemia  Cardiopulmonary Disorders • Congestive heart failure, Shock  Systemic Illness • Pneumonia, Sensory deprivation, Urinary Tract infection o Delirium due to substance intoxication  Must have evidence of substance intoxication  Can be from alcohol, amphetamine, cannabis, cocaine, hallucinogen, inhalant, opioid, PCP, sedative  Also includes side effects from medications • i.e. anti-asthmatic agents, anticonvulsants, corticosteroids, immunosuppressive agents  Most common in elderly • tend to use prescription meds more than any other group • Eliminate drugs from systems less effectively o Delirium due to substance withdrawal  Only when the symptoms are in excess of normal withdrawal syndrome  Most often alcohol – called delirium tremens  Could also be from sedatives, hypnotics or anxiolytics (anti-anxiety meds) o Delirium due to multiple etiologies  Common occurrence  Possible combinations of illnesses & medications are so numerous, determining the cause is difficult o Delirium not otherwise specified  Used for a delirium that is suspected to be from a general medical condition or from substance but there is not enough evidence to substantiate  Also for causes that do not fit other categories (i.e. sensory depravation) - What are other possible effects? o Emotional disturbances (e.g. fear, anxiety, euphoria, depression, anger, apathy) o Tremors o Autonomic hyperactivity (e.g. while seating, raised HR & BP) o Reversal of night-day sleep-wake cycle

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o Extreme shifts in psycho-motor activity  agitation & hyperactivity ------------------------- sluggishness & lethargy Who is at risk? o Elderly (>60)  Estimated to cause 32k hip fractures  16k serious car accidents o Children o Those in substance withdrawal o Those with preexisting brain injuries What is the course? o Begins abruptly  The disturbances have developed over a short period of time (usually hours to days)  tends to fluctuate over the course of the day o Usually resolves within days  Full recovery w/in several weeks  Less likely with elderly • Elderly have 20% - 75% chance of death during hospitalization • Comorbid with pneumonia o Resolution depends on swift identification & correction of etiological factors o Complete recovery is common Differential Diagnosis o To rule out:  Dementia (most common problem) • Could be a combo of both  Substance intoxication  Substance withdrawal  Brief psychotic disorder  Mood disorder with psychotic features  Schizophrenia (duration of at least 6 mo.)  Schizophreniform Disorder – typical cause if subject is young  Acute Stress Disorder  Malingering & Factitious Disorder Are there other causes? o Severe medical or mental illness o Fever o Poisoning o Brain injury o Severe lack of food or water o Sleep deprivation o Excess stress

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- How often does delirium occur? o The single most common acute disorder affecting adults in general hospitals  Affects 10-20% hospitalized adults & 30-40% hospitalized elderly - What is the treatment? o Haloperidol used to treat:  Delirium caused by alcohol withdrawal  Acute delirium - Key Diagnostic Point o Distinguishing feature is a disturbance of consciousness accompanied by some changes in cognition - Read discussion of case vignette – Pg 86

Dementia - What is dementia? o A gradual deterioration of brain functioning that affects judgment, memory, language & other advanced cognitive processes  As opposed to delirium’s acute onset - What are the main symptoms o The individual & cause itself may have impact on which initial symptoms show, but eventually all aspects of cognitive function become affected. o No disorientation or confusion like delirium o Memory impairment is typically seen as an inability to register ongoing events  Can function & remember events from a month ago but forgot what just happened in the past hour  For example a person may know how to drive but forget their own street name or how to get home o One or more of the following cognitive disturbances:  Agnosia • The inability to recognize objects despite intact sensory function • One of the most familiar symptoms • Facial Agnosia o Inability to recognize even very familiar faces  Aphasia • A disturbance in language that differs depending on the area of the brain affected. • Broca’s aphasia – loss of the ability to produce spoken (& often written) language without impairment in the comprehension of language • Wernicke’s aphasia – is the loss of the ability to comprehend language coupled with an inability to produce coherent language

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 Apraxia • The inability to carry out motor activities that the individual was previously able to perform  Impairment in executive functioning • i.e. planning, organizing, abstraction o Advanced dementia patients can not feed, bath or dress themselves  They often wander away from home & become lost  Lose social stigma & may engage in public displays of sexual behavior such as masturbation - What are the types of dementia? o Based on the 6 etiologies  Dementia of the Alzheimer’s type • 50% of dementia cases ultimately get labeled as the Alzheimer’s type • Characterized by gradual & steady deficits in memory, orientation, judgment & reasoning • Tend to lose interest in others & become socially isolated • Can become agitated, confused, depressed, anxious & possibly combative o These characteristics tend to show more intensity later in the day – in a phenomenon called “sun-downer syndrome” • Also tend to show one or more of the following cognitive disturbances o Aphasia, Apraxia, Agnosia • Some research suggests Alzheimer’s people with the least amount of formal education are more susceptible than those with more education o Theory of educational attainment  “mental reserve” o Behavioral Theory  better able to cope & can avoid detection longer o Biological Theory  More synapses developed  deterioration takes longer to affect day to day activities  Reserve of synapses serves as a protective factor  Vascular dementia • Vascular dementia is from blocked or damaged blood vessels in the brain • Most often the result of a stroke • 2nd most likely cause of dementia • Profile of degeneration & skill loss differs from person to person depending on where the damaged blood vessels are in the brain • Signature neurological symptoms are abnormalities in walking & weakness in limbs • The onset is usually sudden (prob from stroke) • Ultimately the same diagnosis as Alzheimer’s

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o Require formal nursing care until death from an infectious disease  Dementia due to other general medical conditions • Diseases o HIV (human immunodeficiency virus)  Dementia caused by HIV infection itself  Symptoms are cognitive slowness, impaired attention, forgetfulness, clumsiness  Tend to become apathetic & socially withdrawn  The later the stage of the disease  the more susceptible the patient is to impairments  Referred to as a sub cortical dementia • Primarily affects the deeper areas of the brain below the cortex • Difference btw cortical & sub cortical is where it affects in the brain & ultimately how the dementia is expressed Characteristic Cortical (Alzheimer’s) Subcortical Dementias Language Aphasia No aphasia Memory Recall & recognition Impaired recall; normal impaired or less impaired recognition Visuospatial Skills Impaired Impaired Mood Less severe depression More severe depression & anxiety & anxiety Motor speed Normal Slowed Coordination Normal until late in Impaired progression • More likely to have severe depression & anxiety w/ subcortical o Head trauma  Memory loss most common symptom o Parkinson’s Disease  Degenerative brain disorder  Janet Reno & Michael J Fox  Symptoms include slow body movements, tremors, jerkiness in walking • From degeneration of dopamine pathways in the brain that control complex movement  Also affects speech – changes to soft monotone voice  Subcortical type  Not guaranteed to develop dementia from disease, rates are 2x more likely than general population though

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o Huntington’s Disease  Genetic disorder  Characteristic symptom is involuntary limb movements  Between 20% - 80% develop dementia  Subcortical type o Pick’s Disease  Very rare neurological condition  Produces cortical dementia  Typically occurs during patient’s 40’s or 50’s o Creutzfeldt-Jakob Disease  Extremely rare (1 in 1 million)  New cases are linked to Mad Cow Disease • Other medical conditions o Normal pressure hydrocephalus (excessive water in the cranium, due to brain shrinkage) o hypothyroidism (under active thyroid) o brain tumor o vitamin B12 deficiency  Substance-induced persisting dementia • Cortical type • Prolonged drug use (especially in combination with poor diet) can lead to long lasting brain damage  which can sometimes lead to dementia • 7% of people dependant on alcohol meet the criteria for dementia • Drugs that can lead to dementia include o Alcohol, inhalants (glue, gasoline), sedatives, hypnotic & anxiolytic drugs  Dementia due to multiple etiologies  Dementia not otherwise specified • Mainly for when the etiology cannot be determined - What are other possible side effects o Emotional changes (possibly bc patients become aware that they are deteriorating mentally)  Irrational beliefs  Depression  Agitation  Aggression  Apathy o Hard to say if these are from brain deterioration or from frustration & discouragement at the inevitable loss of mental functioning as well as losing their loved ones. - Who is at risk? o Highest incidence in older adults

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 Doubles w/ every 5 years of age  Sex, social class & education do not seem to have a bearing • Though more women are said to have dementia, it is most likely due to women living longer than men - What is the course? o Cognitive functioning continues to deteriorate until the patient requires almost total support to carry on day-to-day o Death occurs from inactivity combined with an illness such as pneumonia - Differential Diagnosis o Delirium  Clinical course is best teller o Schizophrenia (multiple cog. Impairments & decline in functioning)  Earlier age onset  Characteristic symptom pattern  Less severe cognitive impairments o MDD (Major Depressive Disorder)  MDD – normal premorbid state & abrupt cognitive decline  Cognitive impariements due to MDD are known as “pseudodementia” o Age Related Cognitive Decline  Much more serious than normal decline of cognitive functioning with age - What are the causes of dementia o Brain trauma  Stroke  Severe head injury o Infectious diseases  Syphilis & HIV o Toxic or poisonous substances o Diseases  Parkinson’s  Huntington’s  Alzheimer’s (most common cause of dementia) • Alzheimer’s gets the majority of attention of researchers bc it is so widespread & we are still in the dark as to its cause • Definitive diagnosis of Alzheimer’s can not be made until autopsy o An affected brain contain two distinctive types of degeneration  neurofibrillary tangles - Large #’s of tangled, standlike filaments

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 amyloid plaques – gummy protein deposits (aka senile or neuritic plaques) • found in healthy older adults but in far fewer #’s o Brain shrinkage also seen often  Leads to enlarged ventricles  Can be from multiple etiologies so it is not directly linked to Alzheimer’s o Multiple genes involved  Deterministic genes – presence of one nearly guarantees development of the disease • Only affect a small % of patients  Susceptibility genes – like the name • Only slightly increase your chances but affect much more of the population  There are three different forms of a protein that transports cholesterol that are associated with late onset Alz. (apoE-2, apoE-3, apoE-4) • One interesting study found that dementia pugilistica (which is caused by repeated blows to the head – named after boxers with the disease) was much more likely to occur with patients who carry the apoE 4 gene - What is the treatment? o Unlike most disorders where treatment prospects are fairly good – this is unfortunately not the case with dementia o There is no way to replace neurons & plasticity can only go so far o Goals of treatment shift from cure to:  Try to prevent controllable conditions that bring on dementia • Such as substance abuse  Try to stop brain damage from spreading & getting worse  Attempt to help patients cope with advancing deterioration  Caregivers use more psychotropic drugs, have a much greater chance of depression & report stress symptoms 3x the average • Efforts are being made to help caregivers- the unintended other victims of dementia o Biological Treatments  Two approaches in the research phase that look to be our best bet so far • A substance called Glial Cell Derived Neurotrophic Factor o A small protein that potently promotes the survival of many types of neurons. The most prominent feature of GDNF is its ability to support the survival of dopaminergic & motor neurons. o may one day be used to reduce or reverse the progress of cognitive degeneration

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• Transplanting fetal brain tissue (taken from aborted fetuses) into the brains of afflicted patients  Cognex, Aricept, Exelon & Reminyl are major drugs on the market now that have an impact on the decline of cognitive ability of Alzheimer patients. • Prevent the breakdown of the neurotransmitter acetylcholine (which is deficient) • Shows short term regression back to where patients were 6 months earlier • Gain is not permanent, most patients (75%) stop taking meds bc of side effects (of liver damage & nausea) & meds are expensive ($250/month)  Nonsteroidal anti-inflammatory drugs have been shown to slow the onset of the disease  Selective Seretonine Reuptake Inhibitors are usually prescribed to alleviate depression & anxiety common with cognitive decline o Psychosocial Treatments  Adaptations for early stages include “memory wallets” • Index cards that state the person’s name, who their spouse is & where they live often help them to better socialize with others  Putting colored arrow & grids on the floors to designate safe & dangerous areas • Help patients be more autonomous & help relieve caregivers of constant monitoring  Assertiveness training to caregivers as patients often can become agitated & verbally or physically aggressive. • The training helps caregivers deal with the patients more effectively so that they do not take offense from the behavior as much & possibly respond by withholding food or meditation or possibly physical abuse - Prevention o Estrogen replacement therapy decreases the risk of dementia of the Alzheimer’s type in women  But increases chance of breast cancer o Proper treatment of systolic hypertension MAY cut the risk of dementia o Non-steroidal anti-inflammatory drugs MAY decrease the risk of developing dementia - Key Diagnostic Point o Distinguishing feature is a memory impairment accompanied by at least one major cognitive disturbance (i.e. aphasia, apraxia, agnosia) - Play audio & video Amnestic Disorder - What is amnestic disorder o The loss of memory without the loss of other high-level cognitive functions o More specifically the inability to transfer information into long-term memory, which can cover minutes, hours or years

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o Must be the direct physiological effect of a medical condition or due to persisting effects of a substance (i.e. drug abuse, medication or toxin exposure) - What are the main symptoms o Inability to learn new information or to recall previously learned information - called anterograde amnesia  After the onset of the disorder, the sufferer will not be able to remember anything that occurs after his attention is shifted away from one subject for more than a few seconds.  Retrograde amnesia is the inability to recall events that occurred before the onset of amnesia o Often can get along with daily chores but have issues with social & occupational functioning due to the importance of memory in those activities  In order to qualify as amnestic disorder it must be a marked decline from a previous level of functioning - What is the course? o Variable  *will detail the recovery with the diff types* o Acute onset  Brain injury, stroke & certain types neurotoxic exposure o Subtle onset  Prolonged substance abuse, chronic neurotoxic exposure or sustained nutritional deficiency o Due to head trauma is largely variable  Greatest deficit right after injury with improvement until about 2 years - What are the types of amnestic disorder o Based on etiology o Amnestic disorder due to a general medical condition (i.e. physical trauma or vitamin deficiency)  Must have evidence that the memory disturbance is the direct physiological consequence of a medical condition  Transient type if symptoms last < 1 month  Chronic type if occur > 1 month o Substance-induced persisting amnestic disorder (includes medication side effects)  Must have evidence that memory disturbance is directly related to the persisting effects of substance abuse (drug abuse, medication or toxin exposure) • Persisting bc the affects on memory are occurring long after the experience of substance intoxication or withdrawal  Alcohol-induced persisting amnestic disorder from prolonged, heavy ingestion of alcohol & the resulting thiamine deficiency is known as Korsakoff’s Syndrome

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There is an acute stage of Wernicke’s encephalopathy – neurological condition that shows symptoms of confusion & eye movement abnormalities • The acute stage passes but major impairment of memory remains & is most often indefinite with little chance of remittance • Effects are severe & often require constant care until death  Sedative-, Hypnotic-, or Anxiolytic-Induced Disorder • Course is variable & full recovery is possible  Impairment may remain stable or could worsen even without present of the substance o Amnestic Disorder Not Otherwise specified  Clinician is unable to determine a specific etiology - Differential Diagnosis o The ability to immediately repeat a sequential string of information (i.e. digital span) is typically not impaired – a good test  Failure shows problems with attention – delirium o Memory impairment with any cognitive impairment is indicative of dementia o Dissociative amnesia & dissociative identity disorder are ruled out by means that they are not from the direct effect of a general medical condition or substance abuse  Dissociative disorders typically do not affect learning, just selective amnesia for traumatic or stressful situations - Key Diagnostic Point o Distinguishing feature is memory impairment without other cognitive deficits - Amnestic disorder in the media o Oliver Sacks characterizes two individuals with anterograde amnesia in his book The Man Who Mistook His Wife for a Hat.  Both suffer from Korsakoff's Syndrome, which causes irreversible anterograde amnesia.  The story of one patient is called "The Lost Mariner": he began forgetting everything from the end of WWII and felt certain that the date was constantly 1945. He does not even know that he suffers from amnesia and believes himself to be decades younger than he is.  The other story is called "A Matter of Identity". Rather than having a consistent false belief about his situation, this patient dealt with his amnesia by constantly re-evaluating and re-explaining his situation. He would greet whoever was in the room over and over again, each time with a different name. Much like "The Lost Mariner", he was unaware of his condition. o Most movies misrepresent amnestic patients and show them as starting over with a clean slate  The two examples here are praised for their accurate portrayals of the disease o Dory from Finding Nemo •

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The fish shows actual problems like learning, retaining information, recalling names and not knowing where she is going or why  The movie shows the frustration of the other fish around her with constant repetition  Also accurately reflects the feelings of people who live with amnesiac patients • Such as when she finds herself lost, alone and confused o Memento  Guy Pearce’s character has severe anterograde amnesia • Little retrograde memory loss and retains identity • Shows many of the everyday issues with memory loss Making A Diagnosis - The cognitive disorders group is one of only three diagnostic groups in the DSM that requires the identification of both specific behavioral symptoms & a causative physiological agent for a diagnosis. - Process is two tiered o 1st – the initial recognition that the patients symptoms are consistent with one of the recognized syndromes of cognitive impairment o 2nd – the search for an underlying physiological cause for the behavior - To delineate which cognitive disorder o Does the patient have disturbances of consciousness?  No – delirium ruled out  Impaired consciousness may be very mild & could easily be dismissed as the patient is upset about his/her illness o Does the patient have an impairment of cognition?  Can appear in many areas • Memory, orientation, perception, language ability, motor activity, object recognition, abstract reasoning  If patient’s only impairment is in memory disturbances  amnestic disorder  If patient has disturbances in consciousness & impairment in cognition that tends to fluctuate over the course of the day  delirium  If patient has memory disturbance & a cognitive impairment w/o disturbance in consciousness dementia o What is the temporal basis of the patient’s disturbance  Delirium & dementia can sometimes be differentiated by the time course of the appearance of symptoms & their change over time  Cognitive impairments develop slowly (over weeks & months) & remain stable w/ little fluctuation over the course of the day  dementia  Cognitive impairments develop rapidly (over hours & days), worsen rapidly & fluctuate  delirium 