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BMB Test 4 > B7-073 Parkinsons/LB Dementia > Flashcards

B7-073 Parkinsons/LB Dementia Flashcards

1
Q

early feature that strongly suggests dementia with lewy bodies, often seen years before other symptoms

A

REM sleep disorder

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2
Q

bipolar patients treated with […] can develop toxicity, causing the acute development of confusion, tremor, and personality changes

A

lithium

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3
Q

what class of medications should be avoided in a patient with LBD?

A

antipsychotics

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4
Q

a mutation in […] gene indicates familial prion disease

A

PRNP

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5
Q

cognitive decline begins within 1 year of the onset of parkinsonism

A

DLB

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6
Q

abnormal DAT uptake on SPECT scan

A

DLB/Parkinson’s

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7
Q

progressive cognitive impairment
fluctuations of cognition
visual hallucinations
REM sleep disorder

A

DLB

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8
Q

cognitive domains affected by DLB [3]

A

attention
executive function
visual-spatial skills

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9
Q

accumulation of what protein is associated with DLB?

A

alpha synuclein

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10
Q

antipsychotic that increases mortality in the elderly

A

haloperidol

(i think all antipsychotics increase mortality in elderly)

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11
Q

drug approved for Parkinson related psychosis

A

nuplazid (pimavanserin)

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12
Q

parkinsonism symptoms precede dementia symptoms by more than one year

A

Parkinson’s

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13
Q

what class of drugs improves cognition in patients with DLB?

A

cholinesterase inhibitors

(donepezil)

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14
Q

[…] medications can cause worsening of confusion in patients with dementia

A

anticholinergics

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15
Q

what class of medications can worsen hallucinations in patients with DLB?

A

dopamine agonists

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16
Q

reduced dopamine transporter levels on SPECT scan are typical of [2]

A

DLB
Parkinson’s

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17
Q

atypical antipsychotic that can be used in the treatment of hallucinations and delusions

A

quetiapine

(however, use of antipsychotics in elderly is associated with increased mortality)

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18
Q

first step in DLB patient with worsening hallucinations is to decrease

A

dopamine agonists

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19
Q

rapid cognitive decline with psychosis
parkinsonism
myoclonus

A

CJD

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20
Q

autosomal dominant disorder in which the patient presents with dystonia and myoclonus and cognition is preserved

A

myoclonus-dystonia

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21
Q

what findings on EEG can aid in the diagnosis of CJD?

A

periodic epileptiform discharges (1 Hz)

(seen in 2/3 of cases, but can also be seen in other diseases)

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22
Q

what would you expect to see on pathology of a patient with CJD? [3]

A

nerve cell loss
gliosis
vacuolation (spongiform change)

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23
Q

pathologic diagnosis of prion disease requires identification of […] on immunohistochemistry

A

PrPSc

24
Q

genetic or familial forms of CJD are due to autosomal dominant mutations in the […] gene

A

PRNP

25
Q

what would you expect to see on MRI in later stages of CJD?

A

cortical ribboning signal hyper-intensity with increased hyper-intensity in the deep gray matter

cortical ribboning, pulvinar sign, hockey stick

26
Q

periodic sharp waves complexes are characteristic of what type of CJD?

A

sporadic CJD

27
Q

patients with what type of CJD typically have a younger age of onset and less rapid progression

A

variant

28
Q

REM sleep behavioral disorder is associated with

A

DLB

29
Q

effective for the management of REM behavioral disorder

A

melatonin

30
Q

what causes syncope in patients with DLB?

A

autonomic dysfunction –> orthostatic hypotension

31
Q

core features of DLB [4]

A

prominent fluctuations in cognition
parkinsonism
visual hallucinations/delusions
REM sleep disorder

32
Q

supportive features of DLB [3]

A

antipsychotic sensitivity
excessive daytime sleepiness
hyposmia

33
Q

no cognitive issues early in disease course

may never develop dementia

A

Parkinson’s disease

34
Q

substantial cognitive issues dominate the clinical presentation are are present within a year of onset of motor symptoms

A

DLB

35
Q

fluctuations in attention and level of alertness and disorientation indicate […] sensorium

A

abnormal

neurologic disorder

36
Q

presence of hallucinations and delusions with clear sensorium suggests

A

primary psychiatric disorder

37
Q

absence of normal REM atonia

recurrent dream enactment behavior that includes movements and mimicking dream content

A

REM sleep disorder

38
Q

treatment for REM sleep disorder [2]

A

melatonin
benzos

39
Q

DLB causes loss of […] neurons in the substantia nigra

A

dopaminergic

40
Q

DLB causes loss of […] neurons in the ventral forebrain

A

cholinergic

41
Q

patients with DLB/PD can have abnormalities on DAT SPECT imaging because of degeneration of […] pathways

A

nigrostriatal dopaminergic

42
Q

cholinesterase inhibitors that can aid in cognition in DLB [2]

A

donepizil
rivastigmine

43
Q

atypical antipsychotics that can aid with psychosis in DLB [2]

A

quetiapine
clozapine

(avoid typicals)

44
Q

medications to aid in symptoms of Parkinsonism

A

carbidopa/levodopa (can cause hallucinations)

(avoid dopamine agonists)

45
Q

most probable causes of rapidly progressive dementia with normal head CT [4]

A

medication induced encephalopathy
infection
autoimmune
prion disease

46
Q

associated with startle myoclonus

A

CJD

47
Q

CSF analysis for CJD may show […] pleocytosis and […] protein

A

no pleocytosis
mildly elevated protein

48
Q

CSF protein tests for prion disease [2]

A

14-3-3
RT-QuIC (more specific, but not widely available)

49
Q

rapidly progressive dementia commonly associated with psychosis, seizures, and hyperkinetic movement disorders

A

autoimmune encephalitis

50
Q

clues to diagnosis of autoimmune encephalitis [2]

A

subtle abnormalities on MRI

mild CSF pleocytosis (6-20 WBCs)

51
Q

symptoms of Parkinson’s disease [4]

A

Tremor
Rigidity
Akinesia/bradykinesia
Postural instabilty

TRAP

52
Q

mutation in the PRNP gene leading to spontaneous abnormal folding of PrP

A

familial prion disease

(fCJD, familial fatal insomnia)

53
Q

idiopathic
exact clinical presentation dependent on allelic variations in normal PrNP gene

A

sporadic prion disease

(sCJD, fatal insomnia)

54
Q

prion disease caused by known exposure to exogenous abnormally folded PrP

A

acquired prion disease

(kuru, iatrogenic, vCJD)

55
Q

reversible causes of dementia [5]

A

depression (pseudodementia)
hypothyroidism
B12 deficiency
neurosyphilis
normal pressure hydrocephalus

BMB Test 4 (14 decks)

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