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Neuro 2 Exam 2 > DSA 31 Demyelinating Diseases > Flashcards

DSA 31 Demyelinating Diseases Flashcards

1
Q

identify: autoimmune demyelinating disorder with distinct clinical episodes separated in time due to white matter lesions separated in space.

A

multiple sclerosis

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2
Q

identify: this disease is initiated by Th1 and Th17 cells that react against myelin antigens and secrete cytokines.

A

multiple sclerosis

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3
Q

where do most plaques commonly occur in MS?

A

adjacent to lateral ventricles

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4
Q

identify: ongoing myelin breakdown, abundant macrophages with lipid-rich PAS-positive debris. also preservation of axons and depletion of oligodendrocytes.

A

active plaque in MS

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5
Q

where do active plaques usually form?

A

centered on small veins

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6
Q

identify: little/no myelin, decreased oligodendrocyte nuclei. instead, astrocyte proliferation and gliosis.

A

inactive plaque in MS

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7
Q

what is a shadow plaque in MS?

A

border between normal and affected white matter is not sharply circumscribed

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8
Q

identify: CSF has mild increase in proteins, moderate pleocytosis, increased IgG leves, oligoclonal IgG bands.

A

multiple sclerosis

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9
Q

what is neuromyelitis optica?

A

syndrome with synchronous (or near synchronous) bilateral optic neuritis and spinal cord demyelination

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10
Q

identify: this syndrome has antibodies against aquaporin 4 which then injure astrocytes via complement-dependent mechanism.

A

neuromyelitis optica

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11
Q

what is the genetic linkage in MS?

A

HLA-DR2

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12
Q

what demographic is predominantly affected by MS?

A

young adults, mostly women, 20s-30s

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13
Q

what is the clinical presentation of MS?

A

scanning speech, incontinence, INO, nystagmus–think SIIN. can also have optic neuritis

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14
Q

identify: diffuse demyelinating disease occurring after viral infection or (rarely) viral immunization. no focal deficits in presentation.

A

acute disseminated encephalomyelitis

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15
Q

identify: fulminant demyelinating syndrome that affects young adults and children after upper respiratory infection.

A

acute necrotizing hemorrhagic encephalomyelitis

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16
Q

identify: acute disorder with myelin damage without inflammation in basis pontis and pontine tegmentum → spastic paresis.

A

central pontine myelinolysis

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17
Q

identify: this disease is commonly associated with rapid correction of hyponatremia. can also occur due to electrolyte abnormalities or after liver transplant.

A

central pontine myelinolysis

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18
Q

what is the most common cause of dementia in the elderly?

A

Alzheimer’s disease

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19
Q

what is the gross morphology of Alzheimer’s disease?

A

cortical atrophy with narrow gyri and wide sulci, especially in the frontal, temporal, and parietal lobes

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20
Q

what is the significance of the apolipoprotein e4 allele in alzheimer’s disease?

A

associated with greater risk and younger age

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21
Q

what is the significance of Aß peptides?

A

they readily aggregate and can be directly neurotoxic → synaptic dysfunction

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22
Q

in amyloid precursor protein processing, sequential cleavage by _______ (alpha/beta/gamma) secretase and _______ (alpha/beta/gamma) secretase results in Aß generation → amyloid fibrils.

A

beta, gamma

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23
Q

in amyloid precursor protein processing, cleavage by _______ (alpha/beta/gamma) secretase → non-amyloidogenic peptide fragments.

A

alpha

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24
Q

identify: round, homogeneous neuronal cytoplasmic inclusions that stain intensely with silver stain.

A

Pick bodies

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25
Q

which lobes are spared in Pick disease?

A

parietal and occipital

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26
Q

what is the clinical progression of Pick disease?

A

behavior and language symptoms arise early, eventually progresses to dementia

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27
Q

identify: group of disorders that share clinical features including progressive deterioration of language and changes in personality corresponding to temporal and frontal lobe degeneration.

A

frontotemporal dementias

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28
Q

identify: disorder with loss of vertical gaze, truncal rigidity, disequilibrium, loss of facial expression, and mild dementia.

A

progressive supranuclear palsy

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29
Q

what demographic is primarily affected by progressive supranuclear palsy?

A

men older than 50 years

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30
Q

true or false: progressive supranuclear palsy does not have MAPT mutations

A

true

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31
Q

identify: neurons exibit tau (-) ubiquitin (+) inclusions in dentate gyrus and superficial temporal and frontal lobes.

A

FTD without tau pathology

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32
Q

what is vascular dementia?

A

multifocal infarction and injury due to hypertension, atherosclerosis, or vasculitis

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33
Q

identify: disease of the elderly characterized by extrapyramidal rigidity.

A

corticobasal degeneration

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34
Q

what is clinical presentation of corticobasal degeneration?

A

asymmetric motor disturbances and sensory cortical dysfunction

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35
Q

in corticobasal degeneration, what is seen in the motor, premotor, and anterior parietal cortices?

A

neuronal loss, gliosis, and ballooned neurons

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36
Q

in corticobasal degeneration, what is seen in astrocytes and oligodendrocytes?

A

tau immunoreactivity present in astrocytes (tufted astrocytes) and coiled bodies in oligodendrocytes

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37
Q

in corticobasal degeneration, what is seen in the substantia nigra and locus coeruleus?

A

loss of pigmented neurons and agyrophilic inclusions

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38
Q

what is the morphology of Parkinson’s disease?

A

pallor of substantia nigra, lewy bodies composed of alpha-synuclein filaments

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39
Q

what is the pathogenesis of Parkinson’s disease?

A

dopamine deficiency from strial neurons which project to the striatum and stimulate muscular movements

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40
Q

what is the clinical presentation of Parkinson’s disease?

A

think TRAPS–tremor, rigidity, akinesia/bradykinesia, postural instability, shuffling gait

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41
Q

what is the significance of when dementia occurs in the history of Parkinson’s disease?

A

dementia is a common feature of late disease. if it occurs early, this suggests Lewy body dementia → dementia, hallucinations, Parkinsonian features

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42
Q

identify: group of disorders characterized by atrophy in specific CNS regions associated with glial tubular cytoplasmic inclusions composed of alpha-synuclein, ubiquitin, and alpha-beta crystallin.

A

multiple system atrophy

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43
Q

identify: Parkinsonism, atrophy of substania nigra and striatum

A

striatonigral degeneration

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44
Q

identify: atrophy of cerebellar peduncles, basis pontis, and inferior olives

A

olivopontocerebellar atrophy

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45
Q

what is clinical presentation of olivopontocerebellar atrophy?

A

cerebellar ataxia, eye and somatic movement abnormalities, dysarthria, rigidity

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46
Q

identify: autonomic dysfunction with loss of sympathetic neurons of intermediolateral column of spinal cord.

A

Shy-Drager syndrome

47
Q

what is the pathogenesis of Huntington’s disease?

A

autosomal dominant mutation of HD gene which encodes huntingtin protein. trinucleotide repeat disorder (more than 35 copies CAG)

48
Q

Hungtington’s disease involves degeneration of which neurons?

A

GABAergic neurons in the caudate nucleus

49
Q

what is morphology of Huntington’s disease?

A

atrophy of caudate, putamen, and frontal lobes.

enlarged ventricles.

gliosis

50
Q

true or false: motor features precede cognitive features in Huntington’s disease.

A

true

51
Q

what is the age of onset for Huntington’s disease? what is the onset related to?

A

age onset: 35-45. related to number of CAG repeats

52
Q

what is the inheritance pattern of Friedreich ataxia?

A

autosomal recessive

53
Q

what is the clinical presentation for Friedreich ataxia?

A

wheelchair within 5 years. gait ataxia, hand clumsiness, dysarthria, depressed DTRs, sensory loss. also likely to develop cardiomyopathy–think of my frat brother Freidreich stumbles and falls but has a big heart.

54
Q

what is the genetic cause for Friedreich ataxia?

A

GAA repeat in gene coding frataxin (inner mitochondrial membrane protein involved in iron regulation) on chromosome 9. decreased frataxin is associated with generalized mitochondrial dysfunction

55
Q

identify: axonal loss and gliosis in posterior columns of the spinal cord, distal corticospinal, and spinocerebellar tracts

A

Friedreich ataxia

56
Q

what structures are degenerated in Friedreich ataxia?

A

CN 8, CN 9, CN 10, CN 12, dentate nucleus,Purkinje cells of superior vermis, and dorsal root ganglia

57
Q

what is the presentation of Friedreich ataxis in childhood?

A

presents with kyphoscoliosis

58
Q

what is the inheritance pattern of ataxia telangiectasia?

A

autosomal recessive

59
Q

what is the clinical presentation of ataxia telangiectasia?

A

present in childhood with cerebellar dysfunction, telangiectactic lesions in the skin and conjunctiva. hypoplastic lymph nodes and thymus → immunodeficiency

60
Q

what is the mutation involved in ataxia telangiectasia?

A

mutation in ATM

61
Q

what degeneration is seen in ataxia telangiectasia?

A

cerebellar, granule, and Purkinje cells lost. degeneration of dorsal columns, spinocerebellar tract, and anterior horn cells (differs from Friedreich ataxia)

62
Q

identify: congenital degeneration of anterior horns of spinal cord → LMN lesion.

A

spinal muscular atrophy

63
Q

what is the clinical presentation of spinal muscular atrophy?

A

floppy baby, marked hypotonia and tongue fasciculations

64
Q

what is the inheritance pattern for spinal muscular atrophy?

A

autosomal recessive

65
Q

true or false: ALS has sensory and oculomotor deficits.

A

false, ALS is a motor neuron disease

66
Q

what is the clinical presentation of ALS?

A

upper motor neuron loss → spasticity, brisk DTRs

lower motor neuron loss → muscles atrophy

brainstem motor neuron loss → abnormal speech, impaired swallowing, later respiratory failure

67
Q

what is the inheritance pattern of bulbospinal atrophy?

A

x-linked

68
Q

identify: atrophy associated with androgen insensitivity (→ gynecomastia), testicular atrophy, and oligospermia (→ male infertility).

A

bulbospinal atrophy

69
Q

what is the genetic cause for bulbospinal atrophy?

A

expansion of CAG/polyglutamine trinucleotide repeat in androgen receptor gene. intranuclear receptor aggregation

70
Q

what drug when given to ALS patients increases survival by decreasing presynaptic glutamate release?

A

riluzole

71
Q

identify: lysosomal storage disorders characterized by neuronal accumulation of lipofuscin. results in blindness, mental and motor deterioration, seizures.

A

neuronal ceroid lipofuscinoses

72
Q

what causes Krabbe disease?

A

deficiency of galactocerebrosidase → stiffness and weakness by 3-6 months of age.

73
Q

identify: glycolipid-engorged macrophages around blood vessels.

A

globoid cells, characteristic of Krabbe disease

74
Q

what causes metachromatic leukodystrophy?

A

deficiency of arylsulfatase → sulfatides cannot be degraded and accumulate in the lysosomes of oligodendrocytes

75
Q

what is the inheritance pattern of metachromatic leukodystrophy?

A

autosomal recessive

76
Q

identify: myelin loss and gliosis with macrophages containing metachromatic material.

A

metachromatic leukodystrophy

77
Q

identify: myelin loss and adrenal insufficiency attributale to the inability to catabolize long chain fatty acids. atrophy of the adrenal cortex is present

A

adrenal leukodystrophy

78
Q

what will present in the serum of patient with adrenal leukodystrophy?

A

high VLCFA

79
Q

describe the genetics of Pelizaeus-Maerzbacher disease.

A

X-linked inheritance, mutations in genes encoding myelin proteins

80
Q

identify: myelin may be in the hemispheres. patches may remain giving a tigroid appearance to tissue sections stained for myelin.

A

Pelizaeus-Merzbacher disease

81
Q

identify: megalocephaly, severe mental deficits, blindness, and signs/symptoms of white matter injury beginning in early infancy. spongy degeneration of the white matter

A

Canavan disease

82
Q

identify: accumulation of N-acetylaspartic acid due to loss of function mutation in the gene encoding the deacetylating enzyme aspartoacetylase located on chromosome 17.

A

Canavan disease

83
Q

what is the clinical presentation of lehigh syndrome?

A

arrest of psychomotor development. feeding problems. seizures, extraocular palsy, and weakness of hypotonia.

84
Q

identify: bilateral damage with vascular proliferation and spongiform changes involving the midbrain, periventricular gray matter, pontine tegmentum, thalamus, and hypothalamus.

A

lehigh syndrome

85
Q

what is characteristic of leigh syndrome?

A

lactic acidemia

86
Q

where does the mutation occur in mitochondrial encephalopathy, lactic acidosis, and strokelike episodes (MELAS)?

A

tRNAs

87
Q

what is the clinical presentation of myoclonic epilepsy and ragged red fibers (MERRF)?

A

patients have myoclonus (seizure disorder), evidence of myopathy, and ataxia

88
Q

identify: sporadic disorder most often associated with large mitochondrial DNA deletion/rearrangement. degeneration of cerebellum is most common.

A

Kearn-Sayre syndrome

89
Q

identify: neurologic symptoms with evidence of hepatic dysfunction. hepatitis and bile duct proliferation.

A

Alpers disease

90
Q

what is clinical progression of Alpers disease?

A

severe seizures followed by developmental delay, hypotonia, ataxia, and cortical blindness

91
Q

what causes Wernicke-Korsakoff syndrome?

A

thiamine (B1) deficiency which can result from alcoholism or gastric disease

92
Q

describe Wernicke encephalopathy.

A

suddent onset of psychosis and/or ophthalmic symptoms (e.g. nystagmus). focal hemorrhage and necrosis of mammilary bodies and walls of 3rd and 4th ventricles.

93
Q

describe Korsakoff syndrome.

A

chronic, can follow Wernicke encephalopathy. memory disturbance and confabulation. lesions in dorsomedial nucleus of thalamus.

94
Q

what does B12 deficiency cause?

A

subacute combined spinal cord degeneration

95
Q

what is the clinical presentation of B12 deficiency?

A

slight ataxia with lower extremity parethesias. spastic weakness and pareplegia (progresses later in the disease)

96
Q

what is the morphology of B12 deficiency?

A

vacuolar swelling of myelin which affects the ascending and descending tracts starting at the mid-thoracic cord

97
Q

identify: atrophy and granule cell loss in anterior cerebellar vermis → Bergmann gliosis (Purkinje cell dropout and astrocyte proliferation).

A

ethanol

98
Q

identify: this toxic disorder causes intractable nausea, confusion, convulsions, and rapid onset of coma. delayed effects are papilledema and headaches.

A

radiation

99
Q

what is the effect of methanol on the retina?

A

degeneration of ganglion cells → blindness

100
Q

which cells are sensitive to hypoglycemia?

A

large cerebral pyramidal cells, hippocampal pyramidal cells, and Purkinje cells

101
Q

in hyperglycemia, what are the physical manifestations of hyperosmolar state?

A

dehydration, confusion, stupor, and eventually coma

102
Q

what is the cause of hepatic encephalopathy?

A

liver failure

103
Q

identify: Alzheimer type II cells evident in cortex and basal ganglia in response to injury.

A

hepatic encephalopathy

104
Q

what is the most common damage seen in carbon monoxide poisoning?

A

bilateral necrosis of the globus pallidus

105
Q

in combined methotrexate and radiation induced injury, where are focal areas of coagulative necrosis often seen?

A

adajcent to lateral ventricles

106
Q

how will neuromyelitis optic differ from MS on MRI?

A

NMO will not show plaques in the brain particularly around periventricular areas, which is characteristic of MS.

107
Q

what is the pathogenesis of multiple system atrophy?

A

alpha-synuclein accumulation kills oligodendroglial cells

108
Q

what is the age of onset for Friedreich’s ataxia?

A

childhood

109
Q

what findings are not a hallmark of ALS?

A

lymphocytic and macrophage infiltrates

110
Q

what is the etiology of Pick disease?

A

abnormality of tau protein

111
Q

what is the clinical presentation of frontotemporal lobar degenerations (FTLDs)?

A

altered personality, behavior, and speech (aphasia) precede memory loss

112
Q

what is a possible partially effective treatment for Wernicke-Korsakoff syndrome?

A

vitamin B1–will likely help the acute Wernicke portion of the syndrome

113
Q

what is the clinical presentation of Huntington’s disease?

A

choreiform movements, aggression, depression, dementia

Neuro 2 Exam 2 (16 decks)

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