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NMSP Exam 1 > Demyelinating/Degenerative/Genetic/Toxic Diseases > Flashcards

Demyelinating/Degenerative/Genetic/Toxic Diseases Flashcards

1
Q

What causes multiple sclerosis?

A
  • Autoimmune demyelinating disorder

- Genetics –> 15 fold higher incidence in first degree relative

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2
Q

What is seen in multiple sclerosis?

A
  • Distinct episodes of neurologic deficits, separated in time due to lesions of white matter separated in space
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3
Q

What do the lesions of MS cause?

A
  • Relapsing and remitting episodes of variable duration

- Neurological deficits are followed by gradual partial recovery

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4
Q

What happens to the relapses in MS?

A
  • Frequency tend to decrease during course, but there is a steady neurologic deterioration
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5
Q

What are some common neurological symptoms of MS?

A
  • Unilateral visual impairment –> frequently initial symptoms
  • Brainstem symptoms –> CN signs, ataxia, nystagmus, and internuclear ophthalmoplegia
  • Spinal cord symptoms –> Motor and sensory impairment of trunk and limbs, spasticity, and loss of bladder control
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6
Q

Who is most likely affected by MS?

A
  • Any age but rare in children

- Women are twice as often

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7
Q

What gene mutations are seen in MS?

A
  • DR2 –> genetic linkage of MS susceptibility

- IL-2 and IL-17 receptor genes

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8
Q

What are some environmental causes of MS?

A
  • Higher number of cases are seen farther away from the equator
  • May be due to lower levels of Vit D
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9
Q

How could chronic inflammation cause MS?

A
  • Disease initiated by CD4+TH1 and TH17 cells that react against self myelin antigens and secrete cytokines
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10
Q

What inflammatory cells are seen in MS?

A
  • TH1 cells –> secrete IFN gamma which activate macrophages (CD68+)
  • TH17 cells promote recruitment of leukocytes
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11
Q

What causes the plaque infiltrates in MS?

A
  • T cells –> mainly CD4+
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12
Q

What does MS look like grossly?

A
  • Multiple well circumscribed
  • Slightly depressed
  • Glassy, gray tan, irregularly shaped plaques
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13
Q

Where in the CNS does MS affect?

A
  • Adjacent to lateral ventricles
  • Corpus callosum
  • Optic nerves and chiasm
  • Brainstem
  • Ascending and descending fiber tracts
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14
Q

What is an active plaque in MS?

A
  • Ongoing myelin breakdown
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15
Q

What is seen with the active plaque in MS?

A
  • Abundant foamy macrophages contain lipid rich, PAS+ debris
  • Perivascular (small veins) inflammatory infiltrate at outer edge of plaque
  • Relative preservation of axons within plaque and depletion of oligodendrocytes
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16
Q

What is an inactive plaque in MS?

A
  • Quiescent

- Inflammation disappears

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17
Q

What is seen with an inactive plaque in MS?

A
  • No myelin
  • Decrease oligo and axons
  • Astrocyte proliferation and gliosis prominent
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18
Q

What is a shadow plaque in MS?

A
  • Border between NL and affected white matter not sharply circumscribed
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19
Q

What is seen in a shadow plaque in MS?

A
  • Abnormally thinned out myelin sheaths

- Partial and incomplete remyelination by surviving oligos

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20
Q

What does the CSF look like in MS?

A
  • Mildly elevated protein
  • Moderate pleocytosis in 1/3 cases
  • IgG increased
  • Oligoclonal IgG bands in immunoelectrophoresis
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21
Q

What is neuromyelitis optica?

A
  • Synchronous bilateral optic neuritis and spinal cord demyelination
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22
Q

Who is affected by neuromyelitis optica?

A
  • Women more than men

- 10-50% people with optic neuritis develop MS

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23
Q

What does the CSF look like in neuromyelitis optica?

A
  • Neutrophils
  • Increased opening pressure
  • Can be turbid
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24
Q

What occurs in neuromyelitis optica?

A
  • Vascular deposition of immunoglobulin and complement

- Antibody to aquaporins –> maintain astrocyte foot processes

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25
Q

What is the long term treatment for neuromyelitis optica?

A
  • Attempt to decrease antibody burden via plasmapheresis
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26
Q

What can be used for acute attacks of neuromyelitis optica?

A
  • Glucocorticoids or plasma exchange
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27
Q

What is acute disseminated encephalomyelitis (ADEM)?

A
  • Perivenous encephalomyelitis

- Diffuse monophasic demyelinating disease that follows either viral infection, or rarely viral immunization

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28
Q

What are some symptoms of acute disseminated encephalomyelitis (ADEM)?

A
  • Follows 1-2 weeks after antecedent event
  • Headache
  • Lethargy
  • Coma
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29
Q

What is the morphology of ADEM?

A
  • Grayish discoloration around white matter vessels
  • Myelin loss with relative preservation of axons
  • Lesions are monophasic
  • Accumulation of lipid-laden macrophages
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30
Q

What is acute necrotizing hemorrhagic encephalomyelitis (ANHE)?

A
  • Fulminant syndrome of CNS demyelination

- Similar to ADEM

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31
Q

Who is most likely affected with ANHE?

A
  • Young adults and children

- Usually seen after a recent upper respiratory infection

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32
Q

What is the usual result seen with ANHE?

A
  • Fatal in most

- Survivors have significant deficits

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33
Q

What is central pontine myelinolysis?

A
  • Loss of myelin roughly symmetric pattern involving the basis pontis and portions of the pontine tegmentum
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34
Q

What is seen in central pontine myelinolysis?

A
  • Myelin loss without evidence of inflammation

- Neurons and axons well preserved

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35
Q

What are the symptoms of central pontine myelinolysis?

A
  • Acute paralysis –> may have locked in syndrome
  • Dysphagia
  • Dysarthria
  • Diplopia
  • LOC
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36
Q

What is the cause of central pontine myelinolysis?

A
  • Overly rapid correction of hyponatremia

- Severe electrolyte or osmolar imbalance

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37
Q

What is dementia?

A
  • Progressive loss of cognitive function independent of the state of attention
  • NOT A PART OF NORMAL AGING
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38
Q

What is seen in Alzheimer disease?

A
  • Insidious impairment of higher intellectual function with alterations in mood and behavior
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39
Q

What happens in late Alzheimer disease?

A
  • Progressive disorientation
  • Memory loss
  • Aphasia
  • All due to severe cortical dysfunction
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40
Q

When is Alzheimer disease usually seen?

A
  • After 60 usually

- Incidence doubles every 5 years

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41
Q

What is needed for a definitive diagnosis of Alzheimer disease?

A
  • Combination of clinical assessment and current radiologic methods allows accurate premortem diagnosis
  • Now possible to demonstrate Abeta deposition through imaging
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42
Q

How can Abeta be seen radiologically?

A
  • Using 18F-labeled amyloid-binding compounds
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43
Q

What can be seen in CSF in Alzheimer disease?

A
  • Presence of increased phosphorylated tau

- Reduced Abeta

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44
Q

What does the cortical atrophy look like in Alzheimer disease?

A
  • Global cortical atrophy
  • Widening of sulci
  • Frontal, temporal, and parietal lobes most prominently
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45
Q

What could be a compensatory side effect of Alzheimer disease?

A
  • Hydrocephalus ex vacuo due to decreased brain parenchyma
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46
Q

What are the two pathologic hallmarks of Alzheimer disease?

A
  • Amyloid plaques

- Neurofibrillary tangles

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47
Q

What helps correlate to the degree of dementia in AD?

A
  • Number of neurofibrillary tangles more than number of neuritic plaques
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48
Q

What are plaques in AD?

A
  • Deposits of aggregated Abeta peptides in the neuropil
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49
Q

What are the tangles in AD?

A
  • Aggregates of the microtubule binding protein tau
  • Develop intracellularly
  • Persist extracellularly after neuronal death
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50
Q

What is the critical initiating event for the development of AD?

A
  • Abeta generation
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51
Q

What genetic locus has a strong influence on the risk of AD?

A
  • Ch 19 which encodes Apolipoprotein E
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52
Q

What is seen on histology in AD?

A
  • Neuritic plaques

- Diffuse plaques

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53
Q

What are neuritic plaques?

A
  • Focal spherical collections of dilated tortuous neuritic processes around amyloid core
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54
Q

Where are neuritic plaques seen?

A
  • Hippocampus
  • Amygdala
  • Neocortex
  • Reactive astrocytes and microglial in periphery
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55
Q

What stain can be used to visualize neuritic plaques?

A
  • Congo red due to amyloid core –> Abeta 40 and 42
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56
Q

How are diffuse plaques different from neuritic plaques?

A
  • No amyloid core –> Abeta 42
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57
Q

Where are diffuse plaques typically seen?

A
  • Superficial cortex
  • Basal ganglia
  • Cerebellar cortex
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58
Q

How is Down syndrome associated with AD?

A
  • The gene that encodes for APP is on Ch 21

- Causes early onset

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59
Q

What is seen with neurofibrillary tangles histologically?

A
  • Pyramidal nucleus
  • Round nucleus
  • Basophilic fibrillary structures –> silver stain
  • Persist after neuron death
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60
Q

What is granular degeneration in AD?

A
  • Small clear intraneuronal cytoplasmic vacuoles which contain argyrophilic granules
  • Normal to be found in aging but abundant in AD
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61
Q

What are hirano bodies in AD?

A
  • Elongated glassy eosinophilic bodies
  • Paracrystalline arrays of beaded filaments –> actin major component
  • Hippocampal pyramidal cells
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62
Q

What are frontotemporal lobar degenerations?

A
  • Heterogenous group of disorders with focal degeneration of frontal and/or temporal lobes
  • Share clinical features with AD but need to look postmortem to differentiate
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63
Q

What is pick disease (FTLD-Tau)?

A
  • Progressive dementia

- Mostly sporadic, but some familial forms identified

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64
Q

When does pick disease develop?

A
  • Early onset of behavioral changes with alterations in personality (frontal lobe signs) and language disturbances (temporal lobe signs)
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65
Q

What does pick disease look like?

A
  • Asymmetric atrophy of frontal and temporal lobes with sparing of the posterior 2/3 of superior temporal gyrus
  • Knife like thin gyri
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66
Q

What are pick cells and pick bodies seen in pick disease?

A
  • Pick cells –> swollen cells

- Pick bodies –> cytoplasmic filamentous inclusions that are weakly basophilic and stain strongly with silver

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67
Q

What is seen in progressive supranuclear palsy?

A
  • Progressive truncal rigidity with disequilibrium
  • Difficulty with voluntary eye movements –> including vertical gaze palsy progressing to difficulty with all eye movements
  • Nuchal dystonia
  • Pseudobulbar palsy and abnormal speech
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68
Q

When is the onset of progressive supranuclear palsy? Who does it affect most?

A
  • 5th-7th decades

- Men more than women

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69
Q

How fatal is PSP?

A
  • Fatal within 5-7 years of onset
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70
Q

What tangles are seen in PSP?

A
  • Globose neurofibrillary tangles –> 4R tau straight filaments
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71
Q

What are the clinical symptoms of parkinson’s disease?

A
  • Diminished facial expression
  • Stooped posture
  • Slowness of voluntary movement
  • Festinating gait
  • Rigidity
  • Pill rolling tremor
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72
Q

What is parkinson disease associated with?

A
  • Protein accumulation and aggregation
  • Mitochondrial abnormalities
  • Neuronal loss in the substantia nigra
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73
Q

What causes the hypokinetic movement disorders?

A
  • Loss of dopaminergic neurons from substantia nigra
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74
Q

What can help determine parkinson disease from other hypokinetic movement disorders?

A
  • Response to L-dopa

- If there is no response, than it is not PD

75
Q

What gene is in mutated in PD?

A
  • SNCA –> encodes for alpha-synuclein (Ch 5)
76
Q

What may be the cause in autosomal recessive forms of PD?

A
  • Mitochondrial dysfunction

- Genes that encode for DJ-1, PINK1, and PARKIN

77
Q

What is the most common cause of AD PD?

A
  • LRRK2
78
Q

Where in the brain becomes more pale in PD?

A
  • Substantia nigra

- Locus ceruleus

79
Q

What are the lewy bodies seen in parkinson disease?

A
  • Could be single or multiple cytoplasmic, eosinophilic round-elongated inclusions that have a dense core surrounded by a pale halo
80
Q

What is dementia with lewy bodies?

A
  • Parkinson disease that is accompanied by cognitive dysfunction
81
Q

What are the symptoms of dementia with lewy bodies?

A
  • Fluctuating course
  • Hallucinations
  • Prominent frontal signs
82
Q

What is lewy neuritis?

A
  • Dystrophic processes that contain alpha-synuclein aggregated protein
83
Q

What symptoms are seen in corticobasal degeneration?

A
  • Extrapyramidal rigidity
  • Asymmetric motor disturbances
  • Impaired higher cortical function
84
Q

Where does corticobasal degeneration affect in the brain?

A
  • Cerebral cortex instead of the brainstem and deep gray matter like PSP
85
Q

What is the most specific pathologic finding in CBD?

A
  • Tau-positive threads in gray and white matter
86
Q

What is multiple system atrophy?

A
  • Sporadic disorder that affects a number of different systems in the brain
87
Q

What three systems are involved in multiple system atrophy?

A
  1. Striatonigral circuit –> parkinsonism
  2. Olivopontocerebellar circuit–> ataxia
  3. ANS –> autonomic dysfunction (orthostatic hypotension)
88
Q

How is Huntington disease inherited?

A
  • AD

- Polyglutamine trinucleotide repeat –> CAG

89
Q

What is Huntington disease?

A
  • Progressive movement disorder and dementia
90
Q

What movements are seen in Huntington disease?

A
  • Chorea
91
Q

What is chorea?

A
  • Jerky, hyperkinetic, sometimes dystonic movements involving all parts of the body
92
Q

When does Huntington disease present? Who does it affect?

A
  • Onset is 4th-5th decade

- More repeats mean the younger the onset

93
Q

How does anticipation affect Huntington disease?

A
  • Repeat expansions during spermatogenesis leads to earlier onset
94
Q

What causes the motor symptoms in HD?

A
  • Loss of medium spiny striatal neurons which leads to dysregulation of basal ganglia
95
Q

What causes the cognitive changes in HD?

A
  • Neuronal loss from cortex
96
Q

What happens to the brain in HD?

A
  • Striking atrophy of caudate nucleus then later, the putamen
  • Globus pallidus secondarily atrophied
  • Atrophy in frontal lobes –> less in parietal
  • Severe loss of striatal neurons –> most marked in caudate nucleus
97
Q

What are the seven types of polyglutamine disease?

A
  • SCA1, SCA2, SCA3, SCA6, SCA7, SCA17
98
Q

What is the polyglutamine disease similar to?

A
  • HD since it is linked to expansion of a CAG repeat
99
Q

What are the five types of expansion of noncoding repeats?

A
  • SCA5, SCA8, SCA10, SCA12, SCA31, and SCA36
100
Q

What is the inheritance of friedreich ataxia?

A
  • AR

- GAA trinucleotide repeat

101
Q

What protein is mutated in friedreich ataxia?

A
  • Frataxin
102
Q

When does friedreich ataxia present?

A
  • Begins in first decade with gait ataxia followed by hand clumsiness and dysarthira
103
Q

What symptoms are seen in friedreich ataxia?

A
  • DTRs depressed or absent –> but extensor plantar reflex is positive
  • Joint position and vibratory sense is impaired
  • Sometimes loss of pain, temperature, and light touch
  • Pes cavus and kyphoscoliosis
104
Q

What does friedreich ataxia do to the heart?

A
  • Increases cardiac arrhythmias and congestive heart failure
105
Q

What is the cause of death most often seen in friedreich ataxia?

A
  • Intercurrent pulmonary infections and cardiac disease
106
Q

What is ataxia-telangiectasia?

A
  • AR

- Ataxic-dyskinetic syndrome that begins in early childhood

107
Q

What symptoms are seen in ataxia-telangiectasia?

A
  • Telangiectasias in CNS, conjunctiva, skin of face, neck, and arms
  • Many develop lymphoid neoplasms
  • Immunodeficiency –> recurrent sinopulmonary infections
108
Q

What gene is mutated in ataxia-telangiectasia?

A
  • ATM gene on Chr 11
109
Q

What does a mutated ATM gene do?

A
  • Increased sensitivity to Xray induced chromosome abnormalities
  • Fails to remove cells with DNA damage
110
Q

What happens in ALS?

A
  • Loss of lower motor neurons in spinal cord and brainstem

- Loss of upper motor neurons in cerebral cortex

111
Q

Who is most likely affected with ALS?

A
  • Seen in men more than women

- Usually around the fifth decade

112
Q

What mutations are the most common cause of familial ALS?

A
  • SOD1 –> 20%
  • C9ORF72 –> 40%
  • TDP-43 and FUS proteins
113
Q

How does ALS affect the CNS?

A
  • Anterior roots are thin
  • Precentral gyrus is atrophic
  • Decreased number of anterior horn neurons
114
Q

What do the neurons contain in ALS?

A
  • PAS+ cytoplasmic inclusions –> Bunina bodies
115
Q

What are some early symptoms of ALS?

A
  • Asymmetric weakness of hands –> dropping objects or difficulty with fine motor tasks
  • Cramping and spasticity of arms and legs
  • Fasciculations
  • Recurrent pneumonia
  • Progressive muscular atrophy
  • Primary lateral sclerosis
116
Q

What is progressive bulbar palsy?

A
  • Degeneration of lower brainstem
  • Cranial motor nuclei occurs early and progresses rapidly
  • Deglutination and phonation difficulties (speaking and swallowing)
117
Q

What is spinal and bulbar muscular atrophy?

A
  • X-linked polyglutamine repeat expansion disease

- Repeat in first exon of the androgen receptor

118
Q

What does the repeat do in spinal and bulbar muscular atrophy?

A
  • May see androgen insensitivity
  • Gynecomastia
  • Testicular atrophy
  • Oligospermia
119
Q

What is seen in spinal and bulbar muscular atrophy?

A
  • Distal limb amyotrophy and bulbar signs
  • Atrophy and fasciculations of the tongue and dysphagia
  • Associated with degeneration of LMNs in the spinal cord and brainstem
120
Q

What happens in neuronal storage diseases?

A
  • Mostly AR

- Defect in catabolism of sphingolipids, mucopolysaccharides or mucolipids –> causes accumulation of substrate

121
Q

What do the defects in neuronal storage diseases cause?

A
  • Loss of cognitive function

- Possible seizure activity

122
Q

What are leukodystrophies?

A
  • Myelin abnormalities

- Generally lack neuronal storage defects

123
Q

What happens in leukodystrophies?

A
  • Diffuse involvement of white matter

- Deterioration of motor skills, spasticity, hypotonia, or ataxia

124
Q

What are mitochondrial encephalomyopathies?

A
  • Oxidative phosphorylation disorders

- Mutations in mitochondrial or nuclear genomes

125
Q

What do mitochondrial encephalomyopathies involve?

A
  • Gray matter and skeletal muscle
126
Q

What gene is involved in Tay Sachs?

A
  • HEXA
127
Q

What enzyme is involved in Tay Sachs?

A
  • Hexosaminidase A
128
Q

What accumulates and never goes away in Tay sachs?

A
  • GM2 gangliosides
129
Q

What happens in Tay sachs?

A
  • Kid is fine for the first year and then degenerating mentally and physically until year 2 or 3
130
Q

What is seen in Tay sachs?

A
  • Cherry red spots in maculae
131
Q

What defect is seen in Krabbe disease?

A
  • Galactocerebroside B galactosidase
132
Q

How does galactocerebroside toxic?

A
  • The accumulation is not toxic

- An alternative catabolic pathway removes a fatty acid generating galactosylsphingosine

133
Q

What does galactosylsphingosine do?

A
  • Toxic to oligodendroglia and astrocytes
134
Q

What does Krabbe disease do?

A
  • Rapidly progressive and fatal

- Motor signs (stiffness and weakness) with gradually worsening difficulties feeding

135
Q

What does histology look like in Krabbe disease?

A
  • Loss of myelin and oligodendroglia in the CNS and PNS

- Neurons and axon relatively spared

136
Q

What is a unique diagnostic feature of Krabbe disease?

A
  • Aggregation of engorged macrophages in parenchyma and around blood vessels
137
Q

What is the defect in metachromatic leukodystrophy?

A
  • Arylsulfatase A –> cerebroside sulfate accumulates (toxic to white matter)
138
Q

What is seen in the late infantile and juvenile form of metachromatic leukodystrophy?

A
  • Late infantile most common
  • Motor symptoms and progress gradually
  • Death in 5-10 years
139
Q

What is seen in the adult form of metachromatic leukodystrophy?

A
  • Psychiatric and cognitive initially

- Motor later

140
Q

What is the treatment of metachromatic leukodystrophy?

A
  • Bone marrow stem cell transplant beneficial if started before neurological deficits
141
Q

What does histology look like in metachromatic leukodystrophy?

A
  • Demyelination with resulting gliosis

- Macrophages scattered in white matter –> have vacuolated cytoplasm

142
Q

What can happen in the brain in metachromatic leukodystophy?

A
  • Brain stained with metachromatic dye
143
Q

How is adrenoleukodystrophy inherited? What gene?

A
  • X-linked recessive

- ABCD1 gene

144
Q

What happens in adrenoleukodystrophy?

A
  • Progressive myelin loss of the CNS and PNS with adrenal insufficiency
145
Q

What happens to the adrenals in adrenoleukodystophy?

A
  • Very long chain fatty acids accumulate (VLCFAs)
146
Q

What happens in the early onset of adrenoleukodystrophy?

A
  • More rapid course

- Young boy presents with behavioral changes and adrenal insufficiency

147
Q

What happens in the late onset of adrenoleukodystrophy?

A
  • Adults slowly progressive for decades

- Peripheral nerves predominantly

148
Q

What is the inheritance of pelizaeus-merzbacher disease?

A
  • X-linked

- PLP and DM20

149
Q

What is pelizaeus-merzbacher disease?

A
  • Invariably fatal leukodystrophy
150
Q

What are some early symptoms in pelizaeus-merzbacher disease?

A
  • Pendular eye movements
  • Hypotonia
  • Choreoathetosis
  • Pyramidal signs
151
Q

What are some late symptoms in pelizaeus-merzbacher disease?

A
  • Spasticity
  • Dementia
  • Ataxia
152
Q

What do tissues look like when stained for myelin in pelizaeus-merzbacher disease?

A
  • Tigroid pattern
153
Q

What causes canavan disease?

A
  • Loss of function mutation in deacetylating enzyme = aspartoacylase
154
Q

What accumulates in canavan disease?

A
  • N-acetylaspartic acid
155
Q

What happens in canavan disease?

A
  • Spongy degeneration of white matter
156
Q

What are the symptoms of canavan disease?

A
  • Megalencephaly
  • Severe mental deficits
  • Blindness
  • Signs of white matter injury
157
Q

What causes alexander disease?

A
  • Gain of function in gene encoding glial fibrillary acid protein (GFAP)
158
Q

What accumulates in Alexander disease?

A
  • Rosenthal fibers around blood vessels, subpial and subependymal zones
159
Q

What is MELAS?

A
  • Mitochondrial encephalomyopathy, Lactic acidosis, and stroke like episodes
160
Q

What is the most prevalent mutation in MELAS?

A
  • MTTL1 TRNAleucine
161
Q

What symptoms are seen in MELAS?

A
  • Recurrent episodes of acute neurologic dysfunction
  • Cognitive changes
  • Muscle involvement with weakness and lactic acidosis
  • Stroke like episodes that do not correspond well to specific vascular territories
162
Q

What is MERRF?

A
  • Myoclonic epilepsy and ragged red fibers
163
Q

What mutation is seen in MERRF?

A
  • TRNA distinct from MELAS
164
Q

What symptoms are seen in MERRF?

A
  • Myoclonus
  • Seizure disorder
  • Evidence of myopathy
  • Ataxia
165
Q

What stain is used to see the ragged red fibers in MERRF?

A
  • Modified gomori trichrome stain
166
Q

What symptoms are seen in Leigh syndrome?

A
  • Lactic acidemia
  • Arrest of psychomotor development
  • Feeding problems
  • Seizures
  • Extraocular palsies and weakness with hypotonia
167
Q

What is seen on histology in Leigh syndrome?

A
  • Multifocal moderately symmetric regions of destruction of brain tissue with spongiform appearance and vascular proliferation
168
Q

What is Kearn-Sayre syndrome?

A
  • Sporadic disorder, often associated with a large mitochondrial DNA deletion/rearrangement
169
Q

What symptoms are seen in Kearn-Sayre syndrome?

A
  • Cerebellar ataxia
  • Progressive external ophthalmoplegia
  • Pigmentary retinopathy
  • Cardiac conduction deficits
170
Q

What does the histology look like for Kearn-Sayre syndrome?

A
  • Spongiform change in gray and white matter

- Neuronal loss most evident in the cerebellum

171
Q

What happens in the brain in Wernicke encephalopathy?

A
  • Hemorrhage and necrosis in the mamillary bodies
172
Q

What happens histologically in B12 deficiency?

A
  • Swelling of myelin in axons both of the ascending tracts of the posterior columns and descending pyramidal tracts
173
Q

What areas of the brain are affected by hypoglycemia?

A
  • Sommer’s sector of hippocampus

- Purkinje cells of cerebellum

174
Q

What is seen in the brain during hypoglycemia?

A
  • Pseudolaminar necrosis –> also selective for the large pyramidal neurons of cerebral cortex
175
Q

What is hyperglycemia usually associated with?

A
  • Ketoacidosis or hyperosmolar coma
176
Q

What happens to the patient in hyperglycemia?

A
  • Become dehydrated and develop confusion, stupor, and eventually coma
177
Q

What is hepatic encephalopathy?

A
  • Cellular response in CNS that is primarily glial due to elevated ammonia levels
178
Q

Where in the brain does carbon monoxide affect?

A
  • Layers III and IV of cerebral cortex
  • Sommer’s sector
  • Purkinje cells
  • Bilateral necrosis of globus pallidi
179
Q

What does CO interact with?

A
  • Heme of cytochrome C oxidase
180
Q

What does methanol do?

A
  • Preferentially attacks the retina
  • Degeneration of retinal ganglion cells –> causing blindness
  • Selective bilateral putaminal necrosis and focal white matter necrosis
181
Q

What is the major metabolite in methanol poisoning?

A
  • Formate
182
Q

What does ethanol do?

A
  • Directly toxic or secondary to nutritional deficits
183
Q

What signs are seen in ethanol over intake?

A
  • Truncal ataxia
  • Unsteady gate
  • Nystagmus
184
Q

How is ethanol toxic to the brain?

A
  • Atrophy and loss of granule cells predominantly in the anterior vermis
  • Loss of purkinje cells and proliferation of adjacent astrocytes

NMSP Exam 1 (12 decks)

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