Cognitive Disorders

Question 1

What are the most common causes of delirium?

Answer 1

infection, medications, substance intoxication or withdrawal, and electrolyte imbalances

Question 2

What are the core features of delirium?

Answer 2

• acute and fluctuating course
• inattention
• disorder thinking or altered level of consciousness

Question 3

What is the treatment for delirium?

Answer 3

• treatment of the underlying cause
• ensuring patient safety in the form of one-on-one observation and keeping shades open/not napping
• haloperidol for severe agitation (benzodiazepines will likely worsen delirium)

Question 4

What is the most likely diagnosis when the following are present: dementia with a stepwise increase in severity and focal neurological signs.

Answer 4

multi-infarct, vascular dementia

Question 5

What is the most likely diagnosis when the following are present: dementia, cogwheel rigidity, and resting tremor.

Answer 5

Lewy body dementia or Parkinson disease

Question 6

What is the most likely diagnosis when the following are present: dementia, gait disturbance, urinary incontinence, and dilated cerebral ventricles.

Answer 6

normal pressure hydrocephalus

Question 7

What is the most likely diagnosis when the following are present: dementia, obesity, coarse hair, constipation, and cold intolerance.

Answer 7

hypothyroidism

Question 8

What is the most likely diagnosis when the following are present: dementia, diminished position and vibration sensation, and megaloblastic anemia.

Answer 8

B12 deficiency

Question 9

What is the most likely diagnosis when the following are present: dementia, tremor, abnormal LFTs, and Kayser-Fleischer rings.

Answer 9

Wilson disease

Question 10

What is the most likely diagnosis when the following are present: dementia, diminished position and vibration sensation, and Argyll Robertson Pupils.

Answer 10

neurosyphilis

Question 11

What are the neurotransmitter and pathological changes are associated with Alzheimer disease?

Answer 11

• a decrease in acetylcholine due to a loss of noradrenergic neurons in the basal cerulean and a decrease in choline acetyltransferase
• gross examination of the brain reveals central atrophy, narrowing of the gyri, widening of the sulci, and dilation of the ventricles
• histology reveals neuritic plaques with an AB amyloid and entangled neuritic processes in addition to the presence of intracellular, hyperphosphorylated tau protein deposits
• dementia correlates with the number of neurofibrillary tangles

Question 12

How is Alzheimer disease differentiated from many of the other types of dementia?

Answer 12

motor and sensory symptoms are affected very late if at all in the course

Question 13

What are the genetic risk factors for Alzheimer’s?

Answer 13

• ApoE4 increases one’s susceptibility

- presenilin 1 and 2 mutations as well as trisomy 21 are associated with early onset Alzheimer disease

Question 14

How can Lewy body dementia and Parkinson disease be differentiated?

Answer 14

• dementia is a late feature of Parkinson disease whereas Lewy body has parkinsonian features with early onset dementia and hallucinations
• often dementia with Lewy bodies is a diagnosis made if the dementia has an onset within 12 months of the parkinsonism symptoms

Question 15

How does Lewy body dementia present?

Answer 15

• a dementia that is more waxing and waning
• parkinsonism
• visual hallucinations
• REM sleep behavior disorder

Question 16

What is the treatment for Lewy body dementia?

Answer 16

• cholinesterase inhibitors improve hallucinations
• atypical neuroleptics are added to stop delusions and agitation
• psychostimulants, levodopa/carbidopa, and dopamine agonists are used to improve cognition, apathy, and psychomotor slowing
• clonazepam is used to treat REM sleep behavior disorder

Question 17

What are the core features of pick disease?

Answer 17

it is a frontotemporal dementia characterized by changes in personality and social conduct, disinhibition, cognitive deficits, and poor insight into their condition

Question 18

What are the pathological features of Lewy body dementia?

Answer 18

lewy neurites, which are pathologic aggregations of alpha-synuclein, primarily in the basal ganglia

Question 19

What are the pathological features of pick disease?

Answer 19

• marked frontal and temporal atrophy

- neuronal loss, micro vacuolization, and astrocytic gloss in cortical layer II

Question 20

What is the treatment for pick disease?

Answer 20

anticholinergics and antidepressants

Question 21

What are the risk factors, clinical manifestation, and treatment for HIV-associated dementia?

Answer 21

• risk increases with duration of illness, low CD4 count, and high viral loads
• presents with a rapid decline in cognition, poor memory, psychomotor retardation, social withdrawal, and depression
• treat with HAART and psychostimulants for fatigue/psychomotor retardation

Question 22

What are the core features of huntington disease?

Answer 22

• progressive dementia
• choreiform movements and muscular hypertonicity
• depression, psychosis, and alcoholism; they have a high rate of suicide

Question 23

What is the pathologic change associated with Huntington disease?

Answer 23

atrophy of the caudate

Question 24

What is the cause of Huntington disease?

Answer 24

a CAG trinucleotide repeat

Question 25

What are the core features of Parkinson disease?

Answer 25

• bradykinesia
• cogwheel rigidity
• resting tremor
• manlike facies
• shuffling gait
• dysarthria
• depression is extremely common
• REM sleep movement disorder is a common preceding sign

Question 26

What are the pathologic features associated with Parkinson disease?

Answer 26

a loss of dopaminergic neurons in the substantial nigra, which typically project to the basal ganglia

Question 27

Periodic sharp waves on an EEG are indicative of what disease process?

Answer 27

Creutzfeldt-Jakob disease

Question 28

What are the core features of NPH?

Answer 28

“wet, wobbly, and wacky” = incontinence, ataxia, and dementia