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NMSP Week 1 > Demyelinating/Degenerative/Genetic/Toxic diseases- PART 1 (Martin) > Flashcards

Demyelinating/Degenerative/Genetic/Toxic diseases- PART 1 (Martin) Flashcards

1
Q

What is the most common demyelinating disease?

A

Multiple sclerosis

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2
Q

What is MS characterized by?

A

Distinct episodes of neurologic deficits separated in time due to white matter lesions that are separated in space

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3
Q

Why is severe cognitive impairment not a usual feature of MS?

A

Because gray matter is relatively spared

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4
Q

What is frequently the initial symptom seen with MS?

A

Unilateral visual impairment

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5
Q

What is a genetic linkage of MS that increases susceptibility?

A

DR2

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6
Q

What CSF examination finding for MS is indicative of the presence of a small number of activated B cell clones?

A

Oligoclonal IgG bands

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7
Q

What is Neuromyelitis optica?

A

Bilateral optic neuritis and spinal cord demyelination at the same time

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8
Q

Neuromyelitis optica is characterized by antibodies against?

A

Aquaporin-4

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9
Q

Signs and symptoms of acute disseminated encephalomyelitis develop 1-2 weeks after?

A

Previous viral infection

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10
Q

In terms of symptomology, how does acute disseminated encephalomyelitis differ from MS?

A

MS has focal neuro deficits while ADEM has diffuse neuro symptoms

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11
Q

Acute necrotizing hemorrhagic encephalomyelitis almost invariably preceded by a recent episode of?

A

Upper respiratory infection

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12
Q

Central pontine myelinolysis (aka osmotic demyelination disorder) occurs 2-6 days after?

A

Rapid correction of hyponatremia

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13
Q

What cells are damaged during central pontine myelinolysis?

A

Oligodendrocytes

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14
Q

What are the plaques in the neuropil aggregates of in regards to Alzheimer Disease?

The neurofibrillary tangles are aggregates of?

A

1) Aβ (APP protein)

2) tau protein

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15
Q

Between the Aβ aggregates and the tau protein, which needs to come first in order for Alzheimer to occur?

A

Aβ plaques then tau aggregates

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16
Q

In regards to Alzheimer, what is hydrocephalus ex vacuo?

What causes it?

A

1) Compensatory ventricular widening

2) Cortical atrophy with widening of the sulci

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17
Q

What AD plaques contain both Aβ40 and Aβ42?

Which contain predominantly Aβ42?

A

1) Neuritic (senile) plaques

2) Diffuse plaques

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18
Q

In regards to the morphology of the neurofibrillary tangles, they often have what shape in pyramidal cells?

A

Elongated flame shape

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19
Q

Because the neurofibrillary tangles are soluble and resistant to clearance in vivo, how are they described as long after the death of the parent neuron?

A

“ghost” or “tombstone” tangles

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20
Q

Between the tangles and the plaques, which correlate better with the degree of dementia in AD?

A

The number of tangles correlates better with the degree of dementia than does the number of neuritic (senile) plaques

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21
Q

What do frontotemporal lobar degeneration, progressive supranuclear palsy, and corticobasal degeneration all have in common in terms of tau and Aβ plaques?

A

tau deposits appear without Aβ plaque in

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22
Q

With Cerebral amyloid angiopathy, what protein is present?

A

Aβ40 protein

23
Q

How does frontotemporal lobar degenerations differ from AD?

A

Changes in personality, behavior, and language come about before changes in memory

24
Q

What disease is the best known frontotemporal lobar degenerations with tau-containing inclusions?

A

Pick disease

25
Q

What occurs morphologically in Pick disease?

A

Asymmetric atrophy of the frontal and temporal lobes that spares the posterior 2/3 of the superior temporal gyrus

26
Q

How is the gyral atrophy of frontal and temporal lobes described in Pick disease?

A

Knife-like thin gyri

27
Q

What is the pathologic hallmark of progressive supranuclear palsy?

A

Presence of tau-containing inclusions in neurons and glia

28
Q

What are the clinical features of Parkinson disease?

A

1) Diminished facial expression
2) Festinating gait (progressively shortened and accelerated steps)
3) Pill rolling tremor
4) Slow voluntary movement

29
Q

What system is damaged in Parkinson disease?

A

Nigrostriatal Dopaminergic system

30
Q

The diagnosis of PD is made by looking at the characteristic features and the symptomatic response to?

A

L-DOPA (response decreases over time)

31
Q

The diagnostic hallmark of PD is?

What is a major component of this?

There is an autosomal dominant PD when?

A

1) The Lewy body
2) α-synuclein
3) α-synuclein is mutated

32
Q

What occurs in the substantia nigra and locus coeruleus with Parkinson’s?

A

Loss of pigmentation (pallor)

33
Q

Cytoplasmic, eosinophilic, round to elongated, dense core and pale halo describes?

A

Lewy Bodies

34
Q

Multiple system atrophy is characterized by?

A

Cytoplasmic inclusions of α-synuclein in oligodendrocytes

35
Q

What is the main sign seen with loss of ANS function due to Multiple System Atrophy?

A

Orthostatic hypotension

36
Q

How does Huntington Disease clinically present?

A

Jerky, hyperkinetic, movements involving all parts of the body (chorea)

37
Q

What is characteristic of Huntington’s?

A

Increased CAG repeats

38
Q

What is “anticipation” in terms of HD?

A

Repeat expansions during spermatogenesis leads to earlier onset

39
Q

The pathologic hallmark of HD is?

A

Intranuclear inclusions of the mutated protein huntingtin

40
Q

Atrophy of what part of the brain is characteristic of HD?

A

Caudate nucleus

41
Q

There is a severe loss of what neurons especially in the caudate nucleus with HD?

A

Striatal neurons

42
Q

What presents as progressive ataxia, spasticity, weakness, sensory neuropathy, and cardiomyopathy?

A

Friedreich Ataxia

43
Q

What do most patients with Friedreich Ataxia die from?

A

Cardiomyopathy complications such arrhythmias and CHF

44
Q

What trinucleotide repeat is associated with Friedreich Ataxia?

In what protein?

A

1) GAA

2) FRATAXIN

45
Q

What gene is mutated in Chr (11q22-q23) in Ataxia-telangiectasia?

A

ATM gene

46
Q

Development of immunodeficiency due to Ataxia-telangiectasia causes what recurrent infections?

A

Sinopulmonary infections

47
Q

What does Amyotrophic lateral sclerosis (ALS) cause in the spinal cord and brainstem?

In the cerebral cortex?

A

1) Loss of lower motor neurons

2) Loss of upper motor neurons

48
Q

What is most commonly mutated that leads to ALS?

A

SOD1

49
Q

In the morphology of ALS, the loss of LMN fibers, loss of anterior horn neurons and reactive gliosis causes?

A

Anterior roots of spinal cord to be thin

50
Q

In the morphology of ALS, what structure may be atrophic especially in severe cases?

A

Precentral gyrus (primary motor cortex)

51
Q

In the morphology of ALS, what leads to the loss of the corticospinal tracts?

A

Loss of UMNs

52
Q

In the morphology of ALS, what do skeletal muscles show?

A

Neurogenic atrophy

53
Q

What early symptom of ALS is seen?

A

Asymmetric weakness of the hands (dropping objects)

54
Q

In regards to ALS, what motor neuron involvement is associated with primary lateral sclerosis?

With progressive muscular atrophy?

A

1) UMN

2) LMN

NMSP Week 1 (13 decks)

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