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STEP 1 > Neurodegenerative disorders > Flashcards

Neurodegenerative disorders Flashcards

1
Q

hypothyroidism, vitamin B12 deficiency, neurosyphilis, normal pressure hydrocephalus
are all what?

A

Reversible causes of Dementia

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2
Q

Loss of dopaminergic neurons (depigmentation)

of substantia nigra pars compacta

A

Parkinson

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3
Q

Lewy bodies are found in what disease?

A

Parkinson

Lewy body dementia

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4
Q

MPTP, a contaminant in illegal drugs, is metabolized to MPP+, which is toxic and can cause

A

Parkinson

damage to Substania Nigra

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5
Q

Atrophy of caudate and putamen with ex vacuo ventriculomegaly.

A

Huntington’s

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6
Q

Tremor (pill-rolling tremor at rest)

Rigidity (cogwheel)

Akinesia (or bradykinesia)

Postural instability

Shuffling gait

Small handwriting (micrographia)

A

Parkinson

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7
Q

Autosomal dominant trinucleotide (CAG)n repeat expansion

A

Huntington’s

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8
Q

gene on chromosome 4

Neurodegenerative disease

A

HTT gene

Huntington’s

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9
Q

chorea, athetosis, aggression, depression, dementia (sometimes initially mistaken for substance abuse).

A

Huntington’s

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10
Q

Anticipation results from Tri nucleotide expansion in what codon and gene

seen in what disease?

A

Huntington’s

CAG –> HTT gene

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11
Q

Neuronal death via NMDA-R binding and glutamate excitotoxicity.

A

Huntington’s

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12
Q

High dopamine
Low GABA & ACh
in brain

A

Huntington’s

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13
Q

Most common cause of dementia in elderly

A

Alzheimer’s

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14
Q

Down syndrome patients haveincreased risk because APP is located on chromosome 21

A

Alzheimer’s

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15
Q

Associated with the following altered proteins:

ApoE-2:______ risk of sporadic form

ApoE-4:______ risk of sporadic form

A

Alzheimer’s

2: decreased
4: increased

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16
Q

APP, presenilin-1, presenilin 2: familial forms (10%) with risk of ________

A

earlier onset Alzheimer’s

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17
Q

Wide spread cortical atrophy, especially hippocampus

Narrowing of gyri and widening of sulci

A

Alzheimer’s

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18
Q

_______ are composed of extra cellular β amyloid core; found in gray matter

Amyloid β (Aβ) synthesized by cleaving amyloid precursor protein (APP)

In what disease?

A

Senile (Alzheimer) plaques
(looks like black dot aggregates)

Alzheimer’s

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19
Q

Intracellular, hyper phosphorylated tau protein are insoluble cytoskeletal elements called?

Number of these correlates with degree of which dementia?

A

Neurofibrillary Tangles in Alzheimer’s
(Poppy’s Troll hair)

*Tangles consist Tau protein, which forms part of the microtubule.

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20
Q

intracellular eosinophilic proteinaceous rods in hippocampus are called? In what’s disease?

A

Hirano bodies

Alzheimer’s

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21
Q

Early changes in personality and behavior
(gambling, shopping, hypersexual, aggressive, etc.),

or aphasia (primary progressive aphasia).

May have associated movement disorders

A

Fronto-temporal lobe dementia (Pick’s disease)

due to Fronto-temporal lobe degeneration

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22
Q

Round inclusions of hyper phosphorylated tau are called what and seen in what dementia?

A 
Pick bodies  (Brownish/black spots on histo)
seen in Fronto-temporal lobe dementia (Pick's disease)
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23
Q

Aggregates of ubiquitinated TDP-43

a DNA/mRNA binding protein

A

Fronto-temporal lobe dementia (Pick’s disease)

look brown

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24
Q

Visual hallucinations

A

Lewy Body Dementia

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25
Q

dementia with fluctuating cognition/alertness

A

Lewy Body Dementia

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26
Q

REM sleep behavior disorder, and parkinsonism

A

Lewy Body Dementia

27
Q

Called _______ dementia if cognitive and motor symptom on set <1 year apart

A

Lewy body dementia

*otherwise considered dementia 2° to Parkinson disease

28
Q

Intracellular Lewy bodies located primarily in the ____

A

cortex

29
Q

Result of multiple arterial infarcts and/or chronic ischemia.

A

Vascular Dementia

30
Q

Step-wise decline in cognitive ability with late-onset memory impairment

A

Vascular Dementia

31
Q

2nd most common cause of dementia in elderly

A

Vascular Dementia

32
Q

MRI or CT shows multiple cortical and/or subcortical infarcts.

A

Vascular Dementia

33
Q

Rapidly progressive (weeks to months) dementia with myoclonus (“startle myoclonus”) seizures and/or ataxia

A

Creutzfeldt -Jakob disease

34
Q

Commonly see periodic sharp waves on EEG

A

Creutzfeldt -Jakob disease

35
Q

Increased 14-3-3 protein in CSF

A

Creutzfeldt -Jakob disease

36
Q

Spongiform cortex (vacuolization without inflammation).

A

Creutzfeldt -Jakob disease

37
Q

Prions PrPcalpha sheets are converted to

PrPsc β pleated sheet resistant to ______.

A

Creutzfeldt -Jakob disease
proteases

*(look like purple specks with some white halos)

38
Q

α synuclein (eosinophilic inclusion) +

A

(Lewy bodies)
Lewy body demetia
and
parkinson’s

39
Q

Parkinsonism degeneration of midbrain and frontal subcortical white matter

A

Progressive supranuclear palsy

40
Q

Gait disturbances, vertical gaze palsy, abnormally phosphorylated Tau protein, loss of executive functions

A

Progressive supranuclear palsy

41
Q

composed of α synuclein

intracellular eosinophilic inclusions

A 
Lewy Bodies
(pink smooth dots, may have brown smudges near them)
42
Q

may cause amyloid angiopathy and/or intracranial hemorrhage

in what disease?

A 
Senile Plaques (Aß core)
Alzheimer's
43
Q

Giant Cells
Infected Oligodendrocytes (viral)
Cerebral atrophy
Worsening memory

A

HIV assoc. Dementia

44
Q

AD is caused by a decrease in the acetylcholine level due to a deficiency of ________.

The decline in acetylcholine is most notable in the nucleus basalis of Meynert, which participates in memory and cognition.

A

choline acetyltransferase

45
Q

As PD progresses, the therapeutic window for levodopa narrows, possibly due to natural or levodopa-induced ____ degeneration.

A

naigrostriatal

46
Q

Ataxia early in disease
Urinary incontinence
Dilated ventricles w/o sulcal widening on neuroimaging
cognitive dysfunction

A

Normal Pressure Hydrocephalus

Wet
Wobbly
Wacky

Reversible with LP drainage of CSF

47
Q

It is thought to be caused by a virus with a mutated or absent matrix protein that prevents mature (enveloped) virion particles from forming. However, the virus continues to replicate intracellularly, leading to a persistent, nonproductive infection
Accumulation of viral nucleocapsids within neurons and oligodendrocytes results in the formation of intranuclear inclusions and eventually leads to inflammation, demyelination, and gliosis.

Patients have high titers of viral antibodies in the serum and cerebrospinal fluid (detectable as oligoclonal bands). The disease causes various neurologic deficits and dementia, and is relentlessly progressive and fatal.

A

Subacute sclerosing panencephalitis (SSPE)

Measles

48
Q

In Huntington’s, prominent atrophy is seen bilaterally in the caudate nucleus and putamen, leading to the enlargement of the ____ ventricles.

A

frontal horns of the lateral ventricles

49
Q

___ agonist medications used to treat Parkinson disease have been associated with the abrupt onset of impulse control disorders,

A

Dopamine

50
Q

Nigrostriatal degeneration in Parkinson disease reduces activity of the ___ and its projections to the cortex, resulting in bradykinesia and rigidity.

A

thalamus

51
Q

Refractory symptoms of Parkinson disease may benefit from high-frequency deep brain stimulation of the globus pallidus INTERNUS or ____ nucleus as it promotes thalamo-cortical disinhibition with improved mobility.

A

subthalamic

52
Q

Multiple ___ infarcts would be expected in vascular dementia.

A

lacunar

53
Q

Negri bodies are round eosinophilic inclusions seen in the cytoplasm of pyramidal HIPPOCAMPAL neurons and ____ cells in patients infected with the rabies virus.

A

cerebellar Purkinje

54
Q

MOA: decreases glutamate release

A

Riluzole

ALS

55
Q

Thin anterior roots
Mild atrophy of the precentral gyrus
Reccurrent INFECTIONS
Loss of neurons in the anterior horn (LMN lesion)
Degeneration and atrophy of the lateral corticospinal tracts (UMN lesion)
Loss of neurons in the motor nuclei of CN V, IX, X, XII
Muscle: denervation atrophy (small angular fibers)

A

ALS

Amyotrophic Lateral Sclerosis

56
Q

Patients with ALS most often die of

A

respiratory failure

due to inspiratory muscle weakness (causing decreased vital capacity due to weak diaphragm),
expiratory muscle weakness (causing a weak cough),
bulbar dysfunction (causing dysphagia and chronic aspiration).

57
Q

____ may cause syncope due to enhanced parasympathetic tone that leads to bradycardia and atrioventricular block with reduced cardiac output.

A 
Acetylcholinesterase inhibitors (donepezil, rivastigmine) 
for AD
58
Q

a peripheral nervous system autoimmune demyelinating polyneuropathy that typically causes lower motor neuron manifestations (eg, areflexia, weakness) in a symmetric ascending pattern.

A

Guillain-Barré syndrome

59
Q

Vitamin B12 and __ deficiency can cause ataxia and loss of propioception (Neurological Symptoms).

A

E

60
Q

___ is seen in children more often than adults. It manifests with irritability, loss of developmental milestones (in infants), or learning problems (in older children). Gastrointestinal symptoms (eg, constipation, nausea, abdominal pain) also occur.

A

Lead intoxication

61
Q

damages the ventral white commissure, leading to bilateral loss of pain and temperature sensation that is limited to the affected levels (typically the arms and hands)

A

Syringomyelia

atrophy of intrinsic hand muscles also a feature

62
Q

Refractory symptoms of Parkinson disease may benefit from high-frequency deep brain stimulation of the ____ or subthalamic nucleus as it promotes thalamo-cortical disinhibition with improved mobility.

A

globus pallidus INTERNUS

63
Q

Refractory symptoms of Parkinson disease may benefit from high-frequency deep brain stimulation of the globus pallidus INTERNUS or subthalamic nucleus as it promotes _____ with improved mobility.

A

thalamo-cortical disinhibition

64
Q

Dopamine agonist medications used to treat Parkinson disease have been associated with the abrupt onset of ____ disorders

A

impulse control

*Pramipexole, Ropinorole, Bromocriptine

STEP 1 (88 decks)

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