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MHD- Neuro > Neurodegenerative Disease > Flashcards

Neurodegenerative Disease Flashcards

1
Q

What is aphasia?

A

Loss of ability to understand or express speech

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2
Q

What is apraxia?

A

Inability to form words- motor dysfunction of speech

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3
Q

What is agnosia?

A

Inability to interpret sensations and recognize things

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4
Q

Name 6 common treatable causes of dementia

A
  1. Stroke
  2. Infections (CJD, Syphilis, HIV)
  3. Neoplasms
  4. Drugs (barbiturates, alcohol, heavy metals)
  5. Metabolic (hypothyroidism, liver disease)
  6. Vitamin deficiencies (cobalamin, thiamine, niacin)
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5
Q

Alzheimer’s disease primarily affects what part of the brain?

A

Cerebral cortex

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6
Q

Lewy body disease primarily affects what part of the brain?

A

Cerebral cortex

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7
Q

Pick’s disease primarily affects what part of the brain?

A

Cerebral cortex

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8
Q

Idiopathic Parkinson’s primarily affects what part of the brain?

A

Basal ganglia

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9
Q

Huntington’s disease primarily affects what part of the brain?

A

Basal ganglia

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10
Q

Multiple system atrophy affects what part of the CNS?

A

Cerebellum and spinal cord

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11
Q

ALS affects what part of the CNS?

A

Cerebellum and spinal cord

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12
Q

Spinal muscular atrophy affects what part of the CNS?

A

spinal cord

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13
Q

Frederich’s ataxia affects what part of the CNS?

A

Cerebellum and spinal cord

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14
Q

What is the most common neurodegenerative cause of senile dementia?

A

Alzheimer’s disease

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15
Q

What percent of Alzheimer’s cases are early onset (familial)?

A

10%

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16
Q

What is the inheritance pattern of Alzheimer’s?

A

Autosomal Dominant

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17
Q

What is the pathogenesis of familial alzheimers?

A

Deposition of amyloid protein

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18
Q

Spitting of what protein leads to amyloid protein?

A

Amyloid precursor protein (APP) located on chromosome 21

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19
Q

What do Down’s patients develop alzheimer’s earlier?

A

They have an extra copy of 21 and therefore more APP. They tend to get alzheimer’s earlier on (40’s and 50’s)

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20
Q

What is the role of presenilin 1 and 2 in alzheimers?

A

They split the APP to form amyloid

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21
Q

What is the role of ApoE4 and ApoE2 in alzheimers?

A

ApoE4 is correlated with higher incidence of alzheiers

ApoE2 is correlated with lower incidence of alzheimers

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22
Q

APP is normally cleaved by _____ to form Abeta protein?

A

Alpha-secretase

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23
Q

Mutated APP is cleaved by ______ to form A beta peptide, which deposits as amyloid in alzheimers

A

beta-secretase

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24
Q

How does the gross appearance of a brain with alzheimers appear?

A

Significant cortical atrophy
Dilated ventricles (hydrocephalus ex vacuo)
atrophied hippocampus

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25
Q

What is the characteristic microscopic findings of alzheimers disease?

A

Neurofibrillary tangles

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26
Q

Are neurofibrillary tangles intra or extracellular?

A

Intracellular

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27
Q

What are neurofibrillary tangles composed of?

A

hyperphosphorylated Tau proteins (tau- normal intermediate filament protein)

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28
Q

Are amyloid plaques intra or extracellular?

A

Extracellular

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29
Q

What is the stain used to visualize amyloid plaques?

A

Bielschowsky silver

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30
Q

What does amyloid angiopathy have to do with alzheimers disease?

A

Amyloid angiopathy is present in variable degrees in nearly all AD cases.

It is the same amyloid found in senile plaques

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31
Q

What is amyloid angiopathy

A

Amyloid deposition in the walls of arterial vessels in the subarachnoid space and superficial cortex

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32
Q

What is the second most common type of neurodegenerative dementia?

A

Lewy Body Disease

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33
Q

WHat is the clinical progression of Lewy Body Disease?

A

Early dementia with visual hallucinations followed by parkinsonian symptoms

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34
Q

WHat is the composition of lewy bodies?

A

alpha-synuclein

35
Q

Where are lewy bodies found in the CNS?

A

Cortex and brainstem

36
Q

What is the third most common cause of neurodegenerative diseases?

A

The group of diseases known as Frontotemporal lobar degeneration

37
Q

What are clinical symptoms associated with frontotemporal lobar degeneration?

A

Change in personal and social conduct. –> often associated with disinhibition and language changes

38
Q

The majority of frontotemporal lobar diseases are linked to mutations in what protein? Located on what chromosome?

A

Tau proteins - located on chromosome 17

39
Q

Other than Tau protein, name two other proteins mutated in frontotemporal lobar diseases

A

Ubiquitin

TDP-43 protein

40
Q

What is the presenting feature of Pick’s disease?

A

Aphasia (loss of ability to express or understand speech)

41
Q

What is the classic findings on histologic sections of someone with PIck’s disease?

A

PIck bodies

42
Q

What do Pick bodies look like?

A

Round and basophilic

43
Q

What are pick bodies composed of?

A

Tau

44
Q

Where in teh CNS are pick bodies found?

A

Hippocampus and cortex

45
Q

List the three features that clinically define Parkinson’s DIsease

A

Fluctuating cognition
Visual hallucinations
Features of parkinsonism: rigidity, bradykinesia , stooped posture, masked facies, shuffling gait, resting tremor

46
Q

Name the histological feature seen in Parkinson’s

A

Lewy Bodies- composed of alpha- synuclein

47
Q

What is the appearance of the substantia nigra in someone with Parkinson’s Disease?

A

Pale- there is atrophy of the substantia nigra

48
Q

What is the inheritance pattern of Huntington’s Disease?

A

Autosomal Dominant with anticipation

49
Q

What is the genetic problem seen in Huntington’s Disease? Which chromosome?

A

Abnormal expansion in CAG triplet repeat on chromosome 4

50
Q

What are 2 clinical features of Huntington’s Disease?

A

Choreiform movements

Psychiatric symptoms progressing to dementia and cachexia

51
Q

Decreased levels of which two neurotransmitters are seen in Huntington’s?

A

GABA and ACh

52
Q

What causes neuronal death in Huntington’s?

A

NMDR-R binding and glutamate toxicity

53
Q

Which two parts of the basal ganglia are damaged/atrophy in Huntington’s?

A

Caudate and putamen- (the dorsal striatum)

54
Q

What are the main symptoms of Multiple System Atrophy?

A

Symptoms consist of variable combination of parkinsonism, cerebellar ataxia, and autonomic dysfunction – depend on the neuronal system involved.

55
Q

What is the pathology of Multiple System Atrophy (what types of inclusions are seen)?

A

Glial cytoplasmic Inclusions- composed of alpha-synuclein

56
Q

Coronal brain sections of someone with multiple system atrophy of which part of the basal ganglia?

A

Putamen

57
Q

Outside of the cerebrum, what 4 other parts of the brain can show atrophy in someone with multiple system atrophy?

A

Cerebellum, substantia nigra, pons and olivary nucleus

58
Q

What is the gene associated with familial ALS?

A

SOD-1 on chromosome 21

5-10% of cases are sporadic

59
Q

Describe the appearance of the ventral and dorsal roots in someone with ALS

A

Ventral roots: severely atrophied due to destruction of descending efferent motor neurons (both UMNs and LMNs are affected)

Dorsal roots: normal appearing. Sensory is not impaired in ALS

60
Q

What is the inheritance pattern of Werdnig-Hoffmann Disease?

A

Autosomal recessive

61
Q

Which cells are affected by Werdnig-Hoffmann disease (SMA I)?

A

anterior horn cells –> LMN problems –> neurogenic atrophy of distal muscles

62
Q

Differentiate Spinal Muscle Atrophy types I, II and III

A

SMA I- Werdnig-Hoffmann Disease “Floppy baby syndrome”

SMA II- Occurs at 3 months or later- slow progressive course with normal life span

SMA III- onset anywhere between infancy and adolescence. Very rare

63
Q

All spinal motor atrophy disease (SMA’s I-III) are caused by mutations in what?

A

Survival of Motor Neuron genes (SMN1, SMN2) on chromosome 5

64
Q

What is the most common form of hereditary ataxia?

A

Friedrich’s Ataxia

65
Q

What is the inheritance pattern of Friedreich’s ataxia?

A

Autosomal Recessive

66
Q

What is the gene mutated in Friedreich’s ataxia?

A

Trinucleotide repeat (GAA) - glutamic acid - in the FRATAXIN gene on chromosome 9

67
Q

What is the role of frataxin?

A

Regulation of mitochondrial iron

68
Q

What causes the ataxia in friedreich’s ataxia?

A

Degeneration of the cerebellum

69
Q

What is the most common cause of death for someone with Friedrich’s Ataxia?

A

Cardiac causes- in addition to degeneration of different spinal cord tracts, there are electrical conductance and structural anomalies that lead to death

70
Q

Which spinal tracts are destroyed in Friedreich’s ataxia?

A

Posterior columns, spinocerebellar columns, and corticospinal tract

+ atrophy of dorsal roots,

71
Q

Name the three Tau-opathies

A

Alzheimer’s, Pick’s disease, some FTLD’s

72
Q

Name the three poly gluatamine diseases

A

Huntington’s Disease
Spinocerebellar ataxia
Friedreich’s ataxia

73
Q

Name the three alpha-synucleinopathies

A

Parkinson’s Disease
Lewy Body Disease
Multiple Systems Atrophy

74
Q

Name a TDP43-opathy

A

one of the FTLD

75
Q

What is mixed dementia?

A

Alzheimer’s disease + vascular dementia

76
Q

Vascular amyloidosis increases the risk of developing what?

A

Hemorrhagic Strokes

77
Q

What is vascular dementia

A

Vascular dementia is a decline in thinking skills caused by conditions that block or reduce blood flow to the brain, depriving brain cells of vital oxygen and nutrients

78
Q

What are pathological findings associated with vascular dementia?

A

Multiple strokes, or a single stroke in a strategic site (thalamus, angular gyrus, ACA territory)

Small vessel disease

79
Q

What causes Wernicke’s syndrome?

A

Thiamine deficiency

80
Q

What 4 areas of the brain are primarily affected by alcohol toxicity?

A
  • Wernicke’s area (due to thiamine deficiency)
  • Central pontine myelinolysis
  • Cortical atrophy
  • Atrophy of the cerebellar vermis
81
Q

What is the triad of WErnicke’s encephalopathy?

A

Oculomotor abnormalities (ophthalmoplegia, nystagmus)

Cerebellar dysfunction
Altered Mental status

82
Q

What is Korsakoff’s psychosis?

A

Loss of recent memory compensated by confabulation

83
Q

What area of the brain is damaged leading to the symptoms of Wernicke’s encephalopathy?

A

Mammillary body

-it becomes hemorrhagic and atrophic

MHD- Neuro (19 decks)

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