B7-073 Parkinsons/LB Dementia Flashcards
early feature that strongly suggests dementia with lewy bodies, often seen years before other symptoms
REM sleep disorder
bipolar patients treated with […] can develop toxicity, causing the acute development of confusion, tremor, and personality changes
lithium
what class of medications should be avoided in a patient with LBD?
antipsychotics
a mutation in […] gene indicates familial prion disease
PRNP
cognitive decline begins within 1 year of the onset of parkinsonism
DLB
abnormal DAT uptake on SPECT scan
DLB/Parkinson’s
progressive cognitive impairment
fluctuations of cognition
visual hallucinations
REM sleep disorder
DLB
cognitive domains affected by DLB [3]
attention
executive function
visual-spatial skills
accumulation of what protein is associated with DLB?
alpha synuclein
antipsychotic that increases mortality in the elderly
haloperidol
(i think all antipsychotics increase mortality in elderly)
drug approved for Parkinson related psychosis
nuplazid (pimavanserin)
parkinsonism symptoms precede dementia symptoms by more than one year
Parkinson’s
what class of drugs improves cognition in patients with DLB?
cholinesterase inhibitors
(donepezil)
[…] medications can cause worsening of confusion in patients with dementia
anticholinergics
what class of medications can worsen hallucinations in patients with DLB?
dopamine agonists
reduced dopamine transporter levels on SPECT scan are typical of [2]
DLB
Parkinson’s
atypical antipsychotic that can be used in the treatment of hallucinations and delusions
quetiapine
(however, use of antipsychotics in elderly is associated with increased mortality)
first step in DLB patient with worsening hallucinations is to decrease
dopamine agonists
rapid cognitive decline with psychosis
parkinsonism
myoclonus
CJD
autosomal dominant disorder in which the patient presents with dystonia and myoclonus and cognition is preserved
myoclonus-dystonia
what findings on EEG can aid in the diagnosis of CJD?
periodic epileptiform discharges (1 Hz)
(seen in 2/3 of cases, but can also be seen in other diseases)
what would you expect to see on pathology of a patient with CJD? [3]
nerve cell loss
gliosis
vacuolation (spongiform change)
pathologic diagnosis of prion disease requires identification of […] on immunohistochemistry
PrPSc
genetic or familial forms of CJD are due to autosomal dominant mutations in the […] gene
PRNP
what would you expect to see on MRI in later stages of CJD?
cortical ribboning signal hyper-intensity with increased hyper-intensity in the deep gray matter
cortical ribboning, pulvinar sign, hockey stick
periodic sharp waves complexes are characteristic of what type of CJD?
sporadic CJD
patients with what type of CJD typically have a younger age of onset and less rapid progression
variant
REM sleep behavioral disorder is associated with
DLB
effective for the management of REM behavioral disorder
melatonin
what causes syncope in patients with DLB?
autonomic dysfunction –> orthostatic hypotension
core features of DLB [4]
prominent fluctuations in cognition
parkinsonism
visual hallucinations/delusions
REM sleep disorder
supportive features of DLB [3]
antipsychotic sensitivity
excessive daytime sleepiness
hyposmia
no cognitive issues early in disease course
may never develop dementia
Parkinson’s disease
substantial cognitive issues dominate the clinical presentation are are present within a year of onset of motor symptoms
DLB
fluctuations in attention and level of alertness and disorientation indicate […] sensorium
abnormal
neurologic disorder
presence of hallucinations and delusions with clear sensorium suggests
primary psychiatric disorder
absence of normal REM atonia
recurrent dream enactment behavior that includes movements and mimicking dream content
REM sleep disorder
treatment for REM sleep disorder [2]
melatonin
benzos
DLB causes loss of […] neurons in the substantia nigra
dopaminergic
DLB causes loss of […] neurons in the ventral forebrain
cholinergic
patients with DLB/PD can have abnormalities on DAT SPECT imaging because of degeneration of […] pathways
nigrostriatal dopaminergic
cholinesterase inhibitors that can aid in cognition in DLB [2]
donepizil
rivastigmine
atypical antipsychotics that can aid with psychosis in DLB [2]
quetiapine
clozapine
(avoid typicals)
medications to aid in symptoms of Parkinsonism
carbidopa/levodopa (can cause hallucinations)
(avoid dopamine agonists)
most probable causes of rapidly progressive dementia with normal head CT [4]
medication induced encephalopathy
infection
autoimmune
prion disease
associated with startle myoclonus
CJD
CSF analysis for CJD may show […] pleocytosis and […] protein
no pleocytosis
mildly elevated protein
CSF protein tests for prion disease [2]
14-3-3
RT-QuIC (more specific, but not widely available)
rapidly progressive dementia commonly associated with psychosis, seizures, and hyperkinetic movement disorders
autoimmune encephalitis
clues to diagnosis of autoimmune encephalitis [2]
subtle abnormalities on MRI
mild CSF pleocytosis (6-20 WBCs)
symptoms of Parkinson’s disease [4]
Tremor
Rigidity
Akinesia/bradykinesia
Postural instabilty
TRAP
mutation in the PRNP gene leading to spontaneous abnormal folding of PrP
familial prion disease
(fCJD, familial fatal insomnia)
idiopathic
exact clinical presentation dependent on allelic variations in normal PrNP gene
sporadic prion disease
(sCJD, fatal insomnia)
prion disease caused by known exposure to exogenous abnormally folded PrP
acquired prion disease
(kuru, iatrogenic, vCJD)
reversible causes of dementia [5]
depression (pseudodementia)
hypothyroidism
B12 deficiency
neurosyphilis
normal pressure hydrocephalus
