B7-073 Parkinsons/LB Dementia

Question 1

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

Answer 1

REM sleep disorder

Question 2

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

Answer 2

lithium

Question 3

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

Answer 3

antipsychotics

Question 4

a mutation in […] gene indicates familial prion disease

Answer 4

PRNP

Question 5

cognitive decline begins within 1 year of the onset of parkinsonism

Answer 5

DLB

Question 6

abnormal DAT uptake on SPECT scan

Answer 6

DLB/Parkinson’s

Question 7

progressive cognitive impairment
fluctuations of cognition
visual hallucinations
REM sleep disorder

Answer 7

DLB

Question 8

cognitive domains affected by DLB [3]

Answer 8

attention
executive function
visual-spatial skills

Question 9

accumulation of what protein is associated with DLB?

Answer 9

alpha synuclein

Question 10

antipsychotic that increases mortality in the elderly

Answer 10

haloperidol

(i think all antipsychotics increase mortality in elderly)

Question 11

drug approved for Parkinson related psychosis

Answer 11

nuplazid (pimavanserin)

Question 12

parkinsonism symptoms precede dementia symptoms by more than one year

Answer 12

Parkinson’s

Question 13

what class of drugs improves cognition in patients with DLB?

Answer 13

cholinesterase inhibitors

(donepezil)

Question 14

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

Answer 14

anticholinergics

Question 15

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

Answer 15

dopamine agonists

Question 16

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

Answer 16

DLB
Parkinson’s

Question 17

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

Answer 17

quetiapine

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

Question 18

first step in DLB patient with worsening hallucinations is to decrease

Answer 18

dopamine agonists

Question 19

rapid cognitive decline with psychosis
parkinsonism
myoclonus

Answer 19

CJD

Question 20

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

Answer 20

myoclonus-dystonia

Question 21

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

Answer 21

periodic epileptiform discharges (1 Hz)

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

Question 22

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

Answer 22

nerve cell loss
gliosis
vacuolation (spongiform change)

Question 23

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

Answer 23

PrPSc

Question 24

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

Answer 24

PRNP

Question 25

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

Answer 25

cortical ribboning signal hyper-intensity with increased hyper-intensity in the deep gray matter | cortical ribboning, pulvinar sign, hockey stick

Question 26

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

Answer 26

sporadic CJD

Question 27

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

Answer 27

variant

Question 28

REM sleep behavioral disorder is associated with

Answer 28

DLB

Question 29

effective for the management of REM behavioral disorder

Answer 29

melatonin

Question 30

what causes syncope in patients with DLB?

Answer 30

autonomic dysfunction --> orthostatic hypotension

Question 31

core features of DLB [4]

Answer 31

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

Question 32

supportive features of DLB [3]

Answer 32

antipsychotic sensitivity excessive daytime sleepiness hyposmia

Question 33

no cognitive issues early in disease course may never develop dementia

Answer 33

Parkinson's disease

Question 34

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

Answer 34

DLB

Question 35

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

Answer 35

abnormal | neurologic disorder

Question 36

presence of hallucinations and delusions with clear sensorium suggests

Answer 36

primary psychiatric disorder

Question 37

absence of normal REM atonia recurrent dream enactment behavior that includes movements and mimicking dream content

Answer 37

REM sleep disorder

Question 38

treatment for REM sleep disorder [2]

Answer 38

melatonin benzos

Question 39

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

Answer 39

dopaminergic

Question 40

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

Answer 40

cholinergic

Question 41

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

Answer 41

nigrostriatal dopaminergic

Question 42

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

Answer 42

donepizil rivastigmine

Question 43

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

Answer 43

quetiapine clozapine (avoid typicals)

Question 44

medications to aid in symptoms of Parkinsonism

Answer 44

carbidopa/levodopa (can cause hallucinations) (avoid dopamine agonists)

Question 45

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

Answer 45

medication induced encephalopathy infection autoimmune prion disease

Question 46

associated with startle myoclonus

Answer 46

CJD

Question 47

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

Answer 47

no pleocytosis mildly elevated protein

Question 48

CSF protein tests for prion disease [2]

Answer 48

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

Question 49

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

Answer 49

autoimmune encephalitis

Question 50

clues to diagnosis of autoimmune encephalitis [2]

Answer 50

subtle abnormalities on MRI mild CSF pleocytosis (6-20 WBCs)

Question 51

symptoms of Parkinson's disease [4]

Answer 51

Tremor Rigidity Akinesia/bradykinesia Postural instabilty TRAP

Question 52

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

Answer 52

familial prion disease (fCJD, familial fatal insomnia)

Question 53

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

Answer 53

sporadic prion disease (sCJD, fatal insomnia)

Question 54

prion disease caused by known exposure to exogenous abnormally folded PrP

Answer 54

acquired prion disease (kuru, iatrogenic, vCJD)

Question 55

reversible causes of dementia [5]

Answer 55

depression (pseudodementia) hypothyroidism B12 deficiency neurosyphilis normal pressure hydrocephalus