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Neuro II > Path: Demyelinating/Neurodegenerative and Metabolic Dz > Flashcards

Path: Demyelinating/Neurodegenerative and Metabolic Dz Flashcards

1
Q

What are the signs of Wernicke encephalopathy; caused by?

How can it be alleviated?

A
  • Thiamine (B1) deficiency
  • Acute; psychotic symptoms or opthalmoplegia
  • May reverse w/ Thiamine
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2
Q

Mutations in the gene encoding ______ are a more common cause of autosomal dominant PD

A

LRRK2 (leucine-rich repeat kinase 2)

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3
Q

In the absence of a toxic or other known etiology the presumptive diagnosis of PD can be based on the presence of the central triad of parkinsonism, which includes?

How is this diagnosis confirmed?

A

1) Tremor
2) Rigidity
3) Bradykinesia
- Confirmed by symptomatic response to L-DOPA replacement therapy

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4
Q

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

A

Gray matter is relatively spared

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5
Q

When is the onset of Ataxia-Telangiectasia?

What are the common symptoms?

A
  • Ataxic-dyskinetic syndrome beginning in early childhood w/ recurrent sinopulmonary infections and unsteadiness in walking
  • Later on, dysarthria and eye movement abnormalities
  • Subsequent development of telangiectasias in the conjunctiva and skin along w/ immunodeficiency
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6
Q

B12 deficiency causes what?

A

- Subacute combined degeneration of the spinal cord –> defect in myelin formation

  • Degeneration of both ascending and descending spinal tracts
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7
Q

Which disease is characterized by extrapyramidal rigidity, asymmetric motor disturbances (jerking movements of limbs), and imparied higher cortical functioning?

A

Corticobasal Degeneration (CBD)

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8
Q

What chromosome is the HTT gene encoding the protein huntingtin found on?

A

Chromosome 4p16.3

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9
Q

What is the characteristic pattern of atrophy seen in Pick disease?

A
  • Asymmetric, atrophy of the frontal and temporal lobes w/ sparing of the posterior 2/3 of superior temporal gyrus
  • Reduction of gyri to a wafer-thin (“knife-edge”) appearance
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10
Q

Central pontine myelinolysis (aka osmotic demyelination disorder) most commonly arises when?

A
  • 2-6 days after rapid correction of hyponatremia
  • Low to high, the pons will die
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11
Q

What is the diagnostic hallmark of PD and what is the major component of this diagnostic indicator?

A

Lewy body; major component is α-synuclein

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12
Q

What is the clinical presentation of Central pontine myelinolysis (aka osmotic demyelination disorder)?

A
  • Rapidly evolving quadriplegia, which may be fatal
  • “Locked-in” syndrome, in which patients are fully conscious yet unresponsive
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13
Q

Cerebral amyloid angiopathy (CAA) is an almost invariable accompaniment of _______

A

AD

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14
Q

Which chromosome is the mutated ATM gene associated with Ataxia-Telangiectasia located on?

Function of this gene?

A
  • Chromosome 11q22-q23
  • Encodes kinase critical for repair of double-stranded DNA breaks
  • Fails to remove cells with DNA damage
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15
Q

Which missense mutation and on what gene is the most common cause of ALS in the US?

A

A4V mutation of SOD1 on chromosome 21

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16
Q

Structures of which lobe are involved earliest in the AD and are usually severely atrophied in the later stages?

A

Medial temporal lobe, including hippocampus, entorhinal cortex and amygdala

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17
Q

Diffuse plaques seen in AD have no _____ and are predominantly made up of Aβ____

A

Diffuse plaques seen in AD have no​ amyloid core and are predominantly made up of Aβ 42

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18
Q

Loss of axons and gliosis are seen where in Friedreich Ataxia?

A
  • Posterior columns
  • Corticospinal tracts
  • Spinocerebellar tracts
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19
Q

Friedreich ataxia is associated with what expansion?

A

GAA trinucleotide repeat of gene on chromosome 9q13 that encodes frataxin

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20
Q

What is seen morphologically in an active plaque of a patient with MS?

A
  • Abundant macrophagescontaininglipid-rich, PAS-positive debris
  • Perivascular (small veins) inflammatory infiltrate (mononuclear) at outer edge of plaqe
  • Relative preservation of axons within plaque and depletion of oligodendrocytes
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21
Q

Accumulation of what is seen in FTLD-tau?

A
  • Accumulation of tau and NOT Aβ are characteristic
  • Smooth contoured inclusions —> Pick bodies
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22
Q

When is the onset of Friedreich Ataxia and how does it manifest?

How do the symptoms progress and what do most affected individuals develop?

A
  • First decade of life beginning with gait ataxia, followed by hand clumsiness and dysarthria
  • DTRs are depressed or absent, but extensor plantar reflex is typically present
  • Joint position and vibratory senses impaired
  • Most will develop pes cavus and kyphoscoliosis

- Cardiomyopathy later in life associated w/ arrhythmia and CHF

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23
Q

What is progressive musclar atrophy vs. primary lateral sclerosis in terms of findings in patients w/ ALS?

A
  • Progressive muscular atrophy: applied to uncommon cases where LMN involvement predominates
  • Primary lateral sclerosis: cases where UMN involvement predominates
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24
Q

What is the age of onset most commonly seen with Huntington Disease?

A

Fourth and fifth decades (30-50 yo)

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25
Q

A higher number of (plaques/tangles) correlates better with the degree of dementia seen in AD?

A

Number of neurofibrillary tangles correlates better with the degree of dementia

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26
Q

What are Pick Cells vs. Pick Bodies?

How do Pick bodies stain?

A
  • Pick cells = swollen cells
  • Pick bodies = cytoplasmic, round to oval, filamentous inclusions that are weakly basophilic and stain strongly with silver
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27
Q

What is Bergmann gliosis and who is it seen in?

A
  • Advanced cases of chronic alcoholism
  • Loss of purkinje cells and proliferation of adjacent astrocytes = Bergmann gliosis
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28
Q

Single or multiple cytoplasmic, eosinophilic, round to elongated inclusions that often have a dense core surrounded by a pale halo describes what feature found in PD?

A

Lewy Bodies

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29
Q

Inclusions containing SOD1, TDP-43, and FUS are associated with that neurodegenerative disease?

A

ALS

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30
Q

Which MHC halotype increases risk for developing MS?

A

HLA-DR2

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31
Q

Wernicke encephalopathy is characterized by foci of hemorrhage and necrosis where?

With time there is infiltration by?

A

- Mammillary bodies and walls of the 3rd and 4th ventricles

  • Hemosiderin-laden macrophages
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32
Q

Metachromatic Leukodystrophy can be observed using what stain?

What is a sensitive method of establishing diagnosis?

A
  • Toluidine blue
  • Metachromatic material in the urine = sensitive
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33
Q

What is the most severe form w/ the earliest onset of Spinal Muscular Atrophy?

Onset and prognosis?

A
  • SMA type 1, Werdnig-Hoffmann disease
  • Onset during first year of life w/ death by 2
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34
Q

What type of hydrocephalus may be seen with AD?

A

Hydrocephalus ex vacuo

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35
Q

Which neurodegenerative disorder is characterized by loss of myelin in a roughly symmetric pattern involving the basis pontis and portions of the pontine tegmentum, including myelin loss WITHOUT evidence of inflammation?

A

Central pontine myelinolysis (aka osmotic demyelination disorder)

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36
Q

Which condition is characterize by synchronous bilateral optic neuritis and spinal cord demyelination?

A

Neuromyelitis optica (aka Devic disease)

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37
Q

Demyelinating diseases of the CNS are acquired conditions characterized by preferential damage to ________ w/ relative preservation of ______.

A

Demyelinating diseases of the CNS are acquired conditions characterized by preferential damage to myelin w/ relative preservation of axons.

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38
Q

Which Ig is found in increased levels in the CSF of patients with MS?

What is seen on immunoelectrophoresis?

A
  • IgG
  • Oligoclonal IgG bands
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39
Q

What is the prognosis of Huntington Disease?

A

Relentlessly progressive and uniformly fatal, average course of 15 years

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40
Q

Which T cells are the major players in causing damage to the myelin in persons w/ MS?

A

CD4+ TH1 and TH17

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41
Q

Which chromosome contains the ApoE locus?

A

Chromosome 19

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42
Q

Clinical features of PD can be remembered with mnemonic TRAPS, which includes?

A

T = tremor (“pill rolling” tremor at rest)

R = rigidity

A = akinesia and bradykinesia

P = postural instability (stooped posture)

S = shuffling gait (festinating gait)

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43
Q

Internculear opthalmoplegia (INO) often seen in MS is due to damage to what?

A

Medial longitudinal fasciculus (MLF)

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44
Q

What is progressive bulbar palsy (Bublar ALS) associated with some patients with ALS?

Prognosis?

A
  • Some pts will have degeneration of the lower brainstem cranial motor nuclei occuring early and rapidly progessing
  • Problems with deglutination and phonation dominate; clinical course is inexorable during 1-2 year period; 50% alive at 2 years
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45
Q

Which disease is characterized by distinct episodes of neuro deficits separated in time due to white matter lesion that are separated in space

A

Multiple Sclerosis

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46
Q

What is commonly found in the CSF of patients with Neuromyelitis Optica?

A

White cells, often including neutrophils

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47
Q

What are the CSF findings in MS?

A
  • Midly elevated protein
  • Moderate pleocytosis in 1/3 cases
  • IgG increased
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48
Q

What 2 brain inclusions are associated with Parkinsons Disease?

A
  • Tau
  • α-synuclein
49
Q

Radiation may induce tumors years after therpay, and include what 3 types?

A
  • Sarcomas
  • Gliomas
  • Meningiomas
50
Q

What are Hirano Bodies and what is their major component?

A
  • Elongated glassy eosinophilic bodies
  • Actin = major component
51
Q

What are the initial signs/symptoms of B12 deficiency?

A
  • Bilateral symmetrical numbness, tingling, and slight ataxia in the lower extremities
  • May progress to include spastic weakness of the lower extremities
52
Q

How are the diagnostic glial cell inclusions in MSA stained?

A

Silver stain; show glial inclusions mainly in oligodendrocytes containing α-synuclein and ubiquitin

53
Q

Clusters of tau-positive processes around astrocytes (“astrocytic plaques”) and the presence of tau-positive threads in gray and white matter are specific pathologic findings for which disease?

A

Corticobasal Degeneration (CBD)

54
Q

Which 3 diseases do Tau deposits appear in without the appearance of Aβ?

A

1) Frontotemporal lobar degeneration (Picks disease)
2) Progressive supranuclear palsy
3) Corticobasal degeneration

55
Q

Glucose deprivation (hypoglycemia) initially causes selective injury to which areas of the brain?

A
  • Large pyramidal neurons of cerebellar cortex
  • Hippocampus; especially Sommer sector (CA1)
  • Purkinje cells of the cerebellum
  • If severe, PSEUDOLAMINAR necrosis of the cortex
56
Q

What is the age of onset for ALS and the earliest symptoms?

Symptoms progress to?

A
  • Asymmetric weakness of hands – dropping objects + difficulty performing fine motor tasks
  • Cramping + spasticity of the arms and legs
  • Fasciculations
  • Respiratory infections
57
Q

Acute necrotizing hemorrhagic encephalomyelitis (AKA acute hemorrhagic leukoencephalitis of Weston Hurst) is almost invariable preceded by a recent episode of?

Who is most at risk?

A
  • Upper respiratory infection (URI)
  • Young adults and children
58
Q

In Huntington Disease there is striking atrophy of?

A

Caudate nucleus; globus pallidus may atrophy secondarily

59
Q

What is the prognosis of Ataxia-Telangiectasia and how do most of these patients die?

A
  • Relentlessly progressive –> death early in second decade
  • Many develop lymphoid neoplasms (often T cell leukemias), gliomas, and carcinomas
60
Q

What are the clinical features of Progressive Supranuclear Palsy (PSP)?

A
  • Trunchal rigidity, disequilibrium w/ frequent falls and difficulty w/ voluntary eye movements
  • Also can have: nuchal dystonia, pseudobulbar palsy, and a mild progressive dementia
61
Q

What are the pathologic findings in the CNS following exposure to high doses of radiation (>10 Gy)

A
  • Large areas of coagulative necrosis, primairly in white matter, and adjacent edema
  • Vessels will have thickened walls w/ intramural fibrin-like material
62
Q

What occurs to someone suffering from clinical hyperglycemia and what precautions must be taken clinically?

A
  • Pt becomes dehydrated and develops confusion, stupor, and eventually coma
  • Fluid depletion must be corrected slowly; otherwise, severe cerebral edema may follow

*From high to low, the brain may blow!

63
Q

1% of chronic alcoholics will develop a clinical syndrome with what findings?

A
  • Truncal ataxia
  • Unsteady gait
  • Nystagmus
64
Q

What are the inclusions found in the brain of patients who die of CTE?

A

Tau

65
Q

Repeat expansions of CAG associated with Huntington Disease occur when?

A

Spermatogenesis; paternal transmission associated w/ early onset in next generation

66
Q

Which disease has a clinical pattern noted to be Parkinsonian w/ abnormal eye movements?

A

Progressive Supranuclear Palsy (PSP)

67
Q

What is a frequent initial manifestation of MS?

A

Unilateral visual disturbances due to involvement of the optic nerve (optic neuritis, retrobulbar neuritis)

68
Q

The mnemonic for MS is SINS, what are each of these clinical findings?

A

S = scanning speech

I = intention tremor (incontinence and INO)

N = nystagmus

69
Q

What type of response in the CNS accompanies hepatic encephalopathy?

Critical mediators?

Which cells appear in the basal ganglia and cerebral cortex?

A
  • Impaired liver function accompanied by glial response in the CNS
  • Mediators = ELEVATED ammonia levels as well as proinflammatory cytokines
  • Alzheimers type II cells
70
Q

In which condition do various cells, including Schwann cells in DRG and peripheral nerves, endothelial cells, and pituicytes show bizarre enlargement of the nucleus to 2-5x normal size and are referred to as amphicytes?

A

Ataxia-Telangiectasia

71
Q

Which chromosome is the gene encoding APP located on and why is this significant?

A
  • Chromosome 21
  • Lies in the down syndrome region; patients with down syndrome usually develop Alzheimers around age 40
72
Q

What is typically the terminal event in a patient with AD?

A

Intercurrent disease, often pneumonia

73
Q

A 40 yo woman who presents with chief complaint of unilateral disturbance should raise red flags for which disease until proven otherwise?

A

MS

74
Q

What is Progressive Supranuclear Palsy and when is it commonly seen?

What is the prognosis?

A
  • A Tauopathy (does not contain Aβ)

- Onset between 5th and 7th decades, with males 2x more affected

  • Often fatal within 5-7 years
75
Q

Huntington Disease patients are at an increased risk of suicide, but the most common cause of death is?

A

Intercurrent infection

76
Q

Progressive disease marked by loss of UMN in cerebral cortex and LMNs in spinal cord and brainstem w/ evidence of toxic protein accumulation

A

Amyotrophic lateral sclerosis (ALS)

77
Q

In chronic alcoholics who suffer from cerebellar dysfunction where is atrophy and loss of granule cells mainly seen?

A

Anterior vermis

78
Q

What is the pathologic hallmark of PSP?

A

Tau-containing inclusion in neurons and glia

79
Q

Some patients with clinically diagnosed FTLD have inclusions that contain ______, but DO NOT contain Tau

A

TDP-43

80
Q

Huntington Disease is the prototype of?

A

Polyglutamine trinucleotide repeat expansion diseases (CAG)

81
Q

What are the focal spherical collections of dilated tortous neuritic processes (dystrophic neurites) around an amyloid core seen in AD?

How are they stained?

A
  • Neuritic (senile) plaques
  • Amyloid core can be stained w/ congo red; dominant component of amyloid core = Aβ
82
Q

What is the morphology of the LNs, thymus, and gonads in Ataxia-Telangiectasia?

A

Hypoplastic

83
Q

All forms of spinal muscular atrophy (SMA) are associated with what genetic mutation?

Common clinical course?

A
  • Disruption of SMN1 (usually deletion)
  • Marked loss of LMN –> progressive weakness
84
Q

Methanol toxicity preferentially damages what structure?

Which structures in brain may be damaged?

A
  • Retina
  • Degeneration of retinal ganglion cells may cause blindess
  • Selective bilateral necrosis of the putamen
85
Q

What is the primary pathologic event occuring in MSA?

A

Glial cytoplasmic inclusions (aka α-synuclein inside of oligodendrocytes)

86
Q

First gene to be identified as a cause of autosomal dominant PD encodes _______

A

α-synuclein

87
Q

The vasuclar amyloid seen in CAA is predominantly of which type?

A

Aβ40

88
Q

Neurofibrillary tangles are insoluble and apparently resistant to clearance in vivo, thus remaining visible in tissue sections as _______ or _______ tangles long after the death of the parent neuron.

A

“Ghost” or “Tombstone” tangles

89
Q

How are Frontaltemporal Lobar Degenerations (FTLDs) distinguished from AD in term of clinical manifestations?

A

Changes in personality, behavior, and language come BEFORE changes in memory

90
Q

Although similar to MS, how does Acute Disseminated Encephalomyelitis (ADEM) differ?

When does it occur and what are the clinical manifestations?

A
  • Occurs in younger patients w/ an abrupt onset and may be rapidly fatal
  • Is a DIFFUSE monophasic demyelinating disease occuring after a viral infection or viral immunization (rare)
  • Signs and symptoms develop 1-2 weeks after the antecedent infection as headache, lethargy, and coma
  • In contrast to MS, all of the lesions look similar – monophasic

*MS has focal findings w/ considerable variance in the size of lesions

91
Q

In PSP there is widespread neuronal loss in what areas of the brain?

What type of tangles are found in these areas and what does ultrastructural analysis reveal?

A
  • Globus pallidus, subthalamic nucleus, substantia nigra, colliculi, periaqueductal gray matter, and dentate nucleus of the cerebellum
  • Contain globose fibrillary tangles
  • Straight filaments composed of 4R tau
92
Q

Generation of which peptide aggregates are the critical initiating event for the development of AD?

A

Aβ first and then tau

93
Q

Genes for which interleukin receptors have been associated with an increased risk in developing MS?

A

IL-2 and IL-7

94
Q

Infarction of which artery is associated with UMN-type weakness and cortical-type sensory loss; contralateral hemiplegia initially?

A

Anterior Cerebral Artery (ACA)

95
Q

In Metachromatic Leukodystrophy there is an accumulation of what?

What does this accumulation lead to?

A

Sulfatides —> white matter injury, inhibiting differentiation of oligodendrocytes, inflammatory response from microglia and astrocytes

96
Q

Multiple System Atophy (MSA) is characterized by cytoplasmic inclusions of ________ in oligodendrocytes (glial cells)

A

Multiple System Atophy (MSA) is characterized by cytoplasmic inclusions of α-synuclein in oligodendrocytes

97
Q

Which parts of the brain will contain neuritic (senile) plaques associated w/ AD?

A

Hippocampus, amygdala, and neocortex

98
Q

How long do patients with Friedreich Ataxia live and what are common causes of death?

A
  • Most patients are wheelchair-bound within 5 years and live to about 40-50 yo
  • Intercurrent pulmonary infections and cardiac death = cause of death
99
Q

Grossly how does the brain of a patient with AD look; where are the effects most pronounced?

Which compensatory change is seen?

A
  • Cortical atropy = widening of sulci, narrowing of gyri
  • Most pronounced in: frontal, temporal, and parietal lobes
  • Compensatory ventricular enlargement –> Hydrocephalus ex vacuo
100
Q

Dementia with Lewy Bodies has which characteristc features?

A
  • Fluctuating course (cognition/alertness)
  • Hallucinations
  • Prominent frontal signs

*HaLewycinations

101
Q

In dementia with lewy bodies there is depigmentation of the substantia nigra and locus ceruleus w/ relative preservation of which structures?

A

Cortex, hippocampus, and amygdala

102
Q

Which 2 spinocerebellar degeneration disorders are Autosomal Recessive?

A
  1. Friedreich ataxia
  2. Ataxia Telangiectasia
103
Q

What are characteristic morphological findings on the spinal cord and brain in ALS?

A
  • Anterior roots are thin
  • Reduction in anterior horn neurons
  • Precentral motor gyrus in the cortex may be atrophic
104
Q

Infarction of which artery is associated with contralateral homonymous hemianopia?

A

Posterior Cerebral Artery

105
Q

Mutations in SOD1 associated with ALS are loss or gain of function?

Leads to?

A
  • Gain of function associate w/ mutant SOD1 protein
  • Forms aggregates
106
Q

How do neurofibrillary tangles appear in pyramidal neurons vs. in rounder cells?

A
  • Pyramidal = elongated “flame” shape
  • Rounder cells = “globose;” basket weave of fibers around nucleus
107
Q

Neuromyelitis optica is due to antibodies against?

Major channel of which cell?

A

Aquaporin-4; major water channel of astrocytes

108
Q

Carbon monoxide poisoning causes selective injury to which areas of the CNS?

A
  • Layers III and V of the cerebral cortex
  • Sommer sector of hippocampus
  • Purkinje cells
  • BILATERAL NECROSIS of the globus pallidus may also occur
109
Q

Neurofibrillary tangles are seen best with which stain?

A

Bielschowsky stain (silver stain)

110
Q

FTLDs are one of the more common causes of?

A

Early onset dementia

111
Q

What are the 3 distinct neuroanatomic circuits commonly involved in MSA; what does disruption of each lead to?

A
  1. Striatonigral: parkinsonism
  2. Olivopontocerebellar: ataxia
  3. ANS: autonomic dysf. w/ orthostatic hypotension as prominent component
112
Q

What do the remaining neurons in ALS contain?

A

PAS-positive cytoplasmic inclusions called Bunina bodies (remnants of autophagic vacuoles)

113
Q

Which cerebral disease is ALS most often associated with?

A

FTLD; most often w/ TDP-43 inclusions

114
Q

What is a characteristic morpological finding in the substantia nigra and locus ceruleus of patients with PD?

A

Pallor (loss of pigmentation) of the substantia nigra and locus ceruleus

115
Q

Plaques are deposits of aggregated ______ peptides in the _______

Tangles are aggregates of the ________ binding protein tau

A

Plaques are deposits of aggregated Aβ peptides in the neuropil

Tangles are aggregates of the microtubule binding protein tau

116
Q

The 2 loci identified as causes of the majority of early-onset familial AD encode what?

A

Presenilin-1 and Presenilin-2

117
Q

What is Korsakoff syndrome and the main signs/symptoms?

Is damage reversible?

Caused by?

A
  • Disturbances of short term memory and confabulation
  • Irreversible
  • Thiamine deficiency –> Chronic alcohol; also gastric disorders (carcinoma), chronic gastritis or persistent vomiting
118
Q

What is the significance of Transient Ischemic Attacks (TIA)?

A
  • Are a warning sign for potentially larger ischemic injury to the brain
  • Neurological emergency!
  • 15% of TIA pts will have a stroke causing persistent deficits within 3 months; HALF of those within the first 48 hrs
119
Q

Which demyelinating disease is associated with lesions that are firmer than the surrounding white matter and contain circumscribed, depressed, glassy, grey-tan, irregularly shaped plaques?

A

MS

Neuro II (11 decks)

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