FA Cognitive Disorders

Question 1

What are the clinical manifestations of Alzheimers?

Answer 1

Progrssive decline in cognitive functions, esp memory and language. Personality changes, mood swings, paranoia. Motor and sensory sx absent until late in the course. Gradual course 10 yars from dx to death

Question 2

What are the Alzheimer genes

Answer 2

Presenelin I, presenelin II, amyloid precursor protein (APP)

Question 3

What is the major susceptibility gene for Alzheimers?

Answer 3

APOe4. apolipoprotein e4. homozygous (2% of population): 50-90% chance of developing Alzheimers by age 85. Heterogygous (15% of population): 45% chance of developing demntia by age 85. 20% chance in general population

Question 4

How much are genes involved in Alzheimers.

Answer 4

Rare accounting for only 5% of cases, usually early onset. Most cases, especially after 65 are sporadic.

Question 5

Neuritc plaques or neurofibrially tangles correlated with severity of dementia in alzheimers

Answer 5

Neuritic plaques.

Question 6

What is the dominant explanation for Alzheimers?

Answer 6

Amyloid cascade hypothesis. Excess of Abeta peptides due to overproduction or decreased clearance. Alzheimers genes patients are chronic overproducers.

Question 7

What’s the deal with Ach in Alzheimers?

Answer 7

Decreased Ach due to loss of noradrenergic neurons in the basal ceruleus and decreased choline acetyltransferase (required for ACH synthesis)

Question 8

What drugs are used to treat Alzheimers?

Answer 8

Mild to moderate: Cholinesterase inhibitors - Tacrine (Cognex), donezipil (Aricept), rivastigmine (exelon), galantamine (razadyne). Slows cognitive decline by 6-12 months. For moderate to severe disease use NMDA antagonists such as memantine (namenda)

Question 9

What is the pattern of cognition in vascular dementia?

Answer 9

No specific pattern of cogition defines vascular dementia

Question 10

What symptoms are common in vascular dementia?

Answer 10

Laterizing signs: hemiparesis, ataxia, psudobalbar palsy (extreme emotional lability, abnormal speech cadence, dysphagia). Depression, anger and paranoia are common.

Question 11

How is vascular dementia treated?

Answer 11

Cholinesterase inhibitors used successfully. Otherwise physical/emotional support, etc. Antihypertension meds may prevent

Question 12

What are Lewy neurites?

Answer 12

pathological aggregates of alpha synuclein. They are more closely linked with clinical sx than lewy bodies.

Question 13

What are the core features of Lewy Body dementia

Answer 13

Waxing and waning of cogition, parkinsonism, sensitivity to neuroleptics and visual hallucinations

Question 14

How do you treat lewy body dementia?

Answer 14

Cholinesterase inhibitors help improve visual hallucinations, atypicals have been slightly effective in stopping delusions and agitation, psychostimulants/levodopa/caribodpa/dopamine agonists may imrpove cogition, apathy, clonazepam for REM sleep behavior disorder

Question 15

Mutation in what gene is associated with Pick Disease/FTD?

Answer 15

20-30% familiar. May be a/w with progranulin or MAPT gene

Question 16

What parts of cognition are well preserved in FTD?

Answer 16

Memory, language and spatial functions are well preserved

Question 17

What is the pathology of FTD/Picks?

Answer 17

Marked atrophy of the frontal and temporal lobes. Neuronal loss, microvacuolization, and astrocytic gliosis in cortical layer 2. only moderate correlation between clinicl and pathologic findings

Question 18

What is usually preseved in HIV dementia?

Answer 18

Language

Question 19

What is the pathology of Huntington disease?

Answer 19

Expanded trinucleotide CAG repeats. Longer the CAG repeat, the earlier the age of onset

Question 20

How do you diagnosie Hungtintons?

Answer 20

MRI - caudate atrophy and sometimes cortical; genetic testing

Question 21

How does dementia progress in Huntingtons?

Answer 21

1 year before or after the chorea.Patients are often aware of deteriorating mentation.

Question 22

What is the age of onset for Hungtingtons?

Answer 22

35-50

Question 23

Dementia due to parkinsons is exacerbated by which medications?

Answer 23

Antipsychotics

Question 24

What is the pathology of PD?

Answer 24

Loss of neurons in the substantia niagra which supplies dopamine to the basal ganglia => decreased dopamine and loss of dopaminergic tracts. Similar to Alzheimers - senile plaques and neurofibrillary tangles, loss of neurons and decreased choline acetyltransferase

Question 25

PD sx?

Answer 25

Dementia similar to Alzheimers. Parkinsonism (bradykinesia, cogwheel ridigidity, resting tremor, masklike facial expression, shuffling gait, dysarthria)

Question 26

Wht are the 3Ws of normal pressure hydrocephalus

Answer 26

Wobbly (apraxia - often appears first), Wet and wacky

Question 27

dementia in normal pressure hydrocephalus is reversible or irreversible

Answer 27

Usually reversible

Question 28

Normal pressure hydrocephalus patients have what in brain?

Answer 28

Englarged ventricles with incresed CSF pressure. Etiology is idiopathic or secondary to obstruction of CSF reabsorption due to trauma, infection or hemorrhage.

Question 29

Causes of Delirium

Answer 29

AEIOU TIPS - Alcohol/drugs toxcity or withdrawal, E - Eletctrolyte abnormality, I - iatrogenic (anticolinergics, benzos, antiepileptics, blood prssure meds, insulin, hypoglycemics, narcotics, steroids, h2 receptor blokers, NSAIDS, antibiotics, antiparkinsonians), O-oxygen hypoxia (bleeding, central venous, pulmonary), U- uremia, hepatic encephalopathy, T - trauma, I - infections, P - poisons, S - seizures

Question 30

What are the most common causes of delirium

Answer 30

Infections, medications, drug toxicity/withdrawal, and electrolyte imbalances

Question 31

what meds can be used for symptomatic treatment of the delirious patient?

Answer 31

esp for agitation, neuroleptics: haldol is most studient and can be given po/Im/iv. Benzos EXACERBATE delirium.

Question 32

What are the most common types of dementia

Answer 32

Alzheimers and then vascular dementia

Question 33

What is delirium

Answer 33

Waxing and waning consciousness with reduced ability to focus, sustain or shift attention. A CHANGE in cognition or development of perceptual disturbance. Disturbance lasts over a short period of time

Question 34

What is dementia

Answer 34

Change in cognition without alteration in consciousness

Question 35

What is the workup for reversible causes of dementia

Answer 35

CBC, electrolytes, TFTs, VDRL/RPR, B12 and folate levels, Brain CT or MRI

Question 36

What is the diagnosis criteria for dementia?

Answer 36

Memory impairment and at least one of the following APRAXIA, APHASIA, AGNOSIA, DISTURBANCE IN EXECUTIVE FUNCTIONING (planning, organizing, abstracting, sequencing). These disturbances cause significant social or occupational functioning and these deficits do not occur in a period of delirium.

Question 37

what medical condition can cause reversible dementia?

Answer 37

Hypothyroidism. Usually accompanies by depressed mood and lethargy.

Question 38

What is the MMSE?

Answer 38

1) Orientation time - date, month, year (5 points), place - city, state, hospital (5 points)
2) Registration - name three objects and repeat them (3 points)
3) Attention and calculation. Serial 7s or spell world backwards (5 points)
4) Recall - name 3 objects 5 minutes later (3 points)
5) Language. name a pen and clock (2), say no ifs and or buts (1), three step command (3)
6) read and obey following. close your eyes, write a sentence, copy design (1, 1, 1). total 30 points. dysfunction is less than 25 points