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Neuro Round 2 wk 2+ > Degenerative Dz JB > Flashcards

Degenerative Dz JB Flashcards

1
Q

What are the 5 cognitive domains?

A
  1. Memory
  2. Language
  3. Visual-Perceptual-Spatial
  4. Executive Function
  5. Social Function/behavior
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2
Q

What is the criteria for delirium?

A
  • acute change or fluctuating course of mental status
  • inattention
  • either
    • Altered level of consciousness
    • disorganized thinking
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3
Q

positive antibodies to neuronal cell-surface or intracellular antigens such as VGKC or NMDA would make you think of…

that presents with ssx of…

A

limbic encephalitis

altered sensorium, rapid cognitive decline, altered mood/personality, and hx of autoimmunity likely

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4
Q

What is a special consideration for pts with limbic encephalitis?

A

a/w certain cancers - ovarian teratoma, small cell lung cancer, adenocarcinoma of breast, testicular cancer, lymphoma, etc

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5
Q

an LP of a pt with limbic encephalitis might show…

A
  • increased protein
  • elevated IgG index
  • oligoclonal bands present
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6
Q

What is caused by a ‘deviant protein’ called a ‘proteinaceous infectious particle’?

A

prion disease - Jakob-Creutzfeldt Disease

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7
Q

How does Jakob-Cruetzfeldt Dz present?

A

rapidly progressive dementia with behavioral abnormalities, ataxia and eventually myoclonus, visual/cerebellar dysfunction

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8
Q

What does MRI and EEG show on Jakob-Creutzfeldt Disease, classically?

A

cortical ribboning

triphasic perioidic sharp wave complexes

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9
Q

What are the 5 criteria for dementia?

A
  1. interfere with the ability to function daily
  2. decline from prior levels
  3. not explained otherwise
  4. dx from H&P and objective cognitive assessment
  5. impairment of at least 2 cognitive domains (memory doesn’t have to be one)
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10
Q

pre-dementia/ mild cognitive impairment is different from dementia in that…

A

daily function is preserved

it is impairment in one or more cognitive domains

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11
Q

What is the MC mild cognitive impairment?

A

amnestic MCI, prodromal state of Alzheimer Disease

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12
Q

Not all MCI is early Alzheimer Disease. What else could it be?

A

depression

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13
Q

What is the most preventable cause of dementia?

A

vascular cognitive impairment

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14
Q

What criteria is needed with dx of vascular cognitive impairment?

A
  • imaging evidence
  • temporal relationship between vascular event and onset of cognitive deficits
  • no hx of gradually progressive cognitive deficits before or after stroke to suggest another cognitive disorder
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15
Q

A major form of VCI is from multiple infarcts. These pts have a … course

A

step-wise course

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16
Q

small strokes in the basal ganglia and white matter or single stroke in thalamic region likely to impair cognition would be…

A

lacunar infarcts and single strategic infarcts

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17
Q

In most cases, nonstroke vascular cognitive impairment is caused by subcortical ischemic cerebral small vessels disease called…

A

Binswanger Disease

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18
Q

Clinical exam of Binswanger Disease would show…

A

gait disturbances, executive dysfunction, evidence of small strokes (hyperreflexia), apathy/depression

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19
Q

A major form of VCI is CADASIL (stands for….)

This is a genetic small vessel disease with vessel smooth muscle …. accumulation

ssx are…

A

Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopathy

NOTCH3

migraines, multiple strokes, and dementia

20
Q

unrelated to atherosclerosis, this major form of VCI is caused by vascualy beta-amyloid deposition in the cerebral cortex and leptomeninges, this causes the vessels to…

A

cerebral amyloid angiopathy (CAA)

become fragile and can rupture leading to intracranial bleeding (superficial/lobar)

21
Q

What is the MC neurodegenerative disorder?

When is it usually first symptomatic?

A

Alzheimer Disease

symptomat after age 65

22
Q

What are the three steps of pathophys behind AD?

A
  1. Tau hyperphosphorylation causing interneuronal neurofibrillary tangles
  2. overproduction or impaired clearance of beta-amyloid causing neuritic plaques
  3. mesial temporal begins global atrophy
23
Q

What are some risk factors for AD?

A
  • age
  • one or more APOE4 alleles
  • family hx
  • low edu/occupational attainment
  • TBIs
  • poor CR fitness in mid and late life
  • midlife adiposity
  • Afib
  • stroke
  • impaired insulin signaling
24
Q

What is the tx of AD?

A

no FDA approved rx

lack of evidence for AChE inhibitors

aerobic exercise may be effective at reducing the rate of conversion

donepezil and rivastigmine have shown modest effects in trials

memantine is FAD approved for moderate tos evere AD

25
Q

What am I looking at?

A

cortical lewy bodies and brainstem lewy bodies

rounded neuronal cytoplasmic inclusion containing alpga synuclein

26
Q

The second MC dementia after AD is…

it occurs in people…

A

Dementia with Lewy Bodies

older than 65 yo

27
Q

Ascending spread of Lewy Body pathology will include…

A

brainstem

limbic

cortical

28
Q

How may Lewy Body Dementia begin?

A

may enter via the gut and spread by the dorsal motor nucleus of the vagus

29
Q

What are the four clinical features of DLB?

A
  • ealry dementia
  • visual hallucinations
  • fluctuating attention and arousal
  • motor parkinsonism
30
Q

What is the 1 year rule of DLB?

A
  • if dementia precedes or occurs within 1 year after onset of motor parkinsonism, then the pt has DLB
  • if motor parkinsonism precedes dementia by at least one year, pt has PDD
31
Q

Early dementia in DLB will have deficits of attention, executive function and secifically…

A

visual-spatial function

32
Q

Neuropsych ssx of DLB includes…

A

visual misperceptions early, then visual hallucinations (Common); delusions later, and depression/anxiety

33
Q

Lewy body brainstem deposition presents as…

A

staring or daytime fatigue

symmetric rigidity and less commonly, tremor

34
Q

Motor parkinsonism a/w DLB develops..

A

with or after cognitive, neuropsych, and fluctuating arousal/attn; motor signs are often symmetric, unlike PD

pts have limited response to carbidopa levadopa, despite dopamine dysfunction

35
Q

Multiple system atrophy is considered…

What are the two main types?

A

alpha synucleinopathy

MCA - Parkinsonism

MSC - cerebellar

36
Q

What are key features of alpha synucleinopathies?

A

autonomic failure

symmetric onset with rigid bradykinesia

atypical tremor

hypokinetic dysarthria

anterocollis with early postural instability

poor response to levadopa

jaw opening dystonia

37
Q

What is this sign called?

A

Pisa Sign

38
Q

What is this sign called?

A

Hot cross bun sign

39
Q

Frontotemporal dementias ultimately cause…

A

disinhibition

40
Q

People with frontotemporal dementia present with…

What is the gene linked to this and what does it cause?

What is the pathology behind it?

A
  • behavioral/personality changes
  • C9ORF72 expanded repeat leading to early psychotic features
  • tauopathy
41
Q

What is the behavior variant of Frontotemporal Dementia?

Where is the brain atrophying?

A

Pick Disease

right frontal/temporal lobe atrophy

42
Q

“Parkinson plus” disorder is a characterization of what disease?

A

Progressive Supranuclear Palsy with Frontotemporal Dementia

43
Q

Pt in their 60s, starting to have a lot of falls, vertical supranuclear palsy (down gaze most sensitive)

dx?

What is their facial expression?

What can help differentiate this from PD?

A

Progressive supranuclear palsy

worried/astonished look

poor response to L dopa

44
Q

What are we looking at? What disease?

A

humming bird sign

PSP

45
Q

In what disorder/disease state will I see asymmetric rigidity with dystonia and ideomotor apraxia?

What is the term for these ssx?

A

corticobasilar degeneration

alien limb phenomenon

46
Q

Where will I see atrophy on an MRI of corticobasilar degeneration?

A

asymmetric frontoparietal atrophy on MRI brain

Neuro Round 2 wk 2+ (16 decks)

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