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67 Cards in this Set
- Front
- Back
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Necrosis in the hippocampus in CA-1 sector is indicative of what condition?
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Hypoxic encephalopathy
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Thiamine deficiency affects what structures in the brain?
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Brainstem
Cerebellum Mamillary bodies Thalamus Vestibular & ocular neurons |
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Vacuolization, loss of myelin and axons is indicative of what vitamin deficiency?
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B12 (cyanocobalamin)
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What affect does lead have on young children?
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Affects immature endothelial cells in the brain of the young
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What neurological effects does mercury have?
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Affects ribosomes of granule cells in cerebellum
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What neurological affect does the JC virus have?
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Attacks oligodendroglial cells in the body
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Locked in syndrome is significant of what condition?
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Central pontine myelinitis
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What causes central pontine myelinitis?
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Usually occurs when patients are rapidly treated for hyponatremia
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A 30 year old woman comes in with scanning speech and seems like she is drunk. What do you suspect?
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Multiple sclerosis
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What finding would you see in the CSF of a patient with MS?
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Increased gamma globulin
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What genetic predisposition is associated with MS?
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DR2 HLA haplotype
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What is the gross pathology of MS?
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Yellow-tan, thick, gelatinous periventricular plaques
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What will an LP show in a patient with MS?
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Increased lymphocytes
Oligoclonal Igg bands Myelin basic protein |
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Interferon beta and Natalizumab are used to treat what disease?
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MS
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What deficiency is found in metachromatic leukodystrophy?
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Arylsulfatase
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What deficiency is found in Krabbe's disease?
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Galactocerebrosidase
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What pathology is seen in Krabbe's disease?
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Granulocytes around blood cells
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What abnormality is seen in adrenoleukodystrophy?
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Abnormal beta oxidation of long chain fatty acids
Coenzyme A is not added properly |
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Spongy degeneration and Alzheimer type II astrocytes is seen in what disease?
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Spongy leukodystrophy (Canavan's disease)
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What abnormality is seen in spongy leukodystrophy (Canavan's disease)
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Decreased aspartoacylase so n-acetylaspartic acid builds up
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A patient comes in with megalencephaly, severe mental deficits and blindness. What do you suspect?
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Spongy leukodystrophy
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A patient comes in with megalencephaly, psychomotor retardation that has been progressive, and seizures. What do you suspect?
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Alexander's disease
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Abnormal accumulation of what substance is seen in Alexander's disease?
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GFAP
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Pathology shows Rosenthal fibers with abnormal GFAP. What do you suspect?
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Alexander's disease
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Pathology shows alpha b-crystallin -- long pink deposits with a granular core. What do you suspect?
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Alexander's disease
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How does Guillan Barre and botulism differ in terms of the spread of paralysis?
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Guillan Barre is ascending paralysis
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How does the diphtheria toxin cause demyelination?
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Prevents Schwann cells from synthesizing myelin
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Sensory impairment and a foot deformity is a consideration for what hereditary neuropathy?
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Charcot-Marie-Tooth
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A small child has developmental delays as well as atrophy of limb and trunk muscles. What do you consider as a ddx?
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HMSN type 3 (Dejerine Sottas)
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What demyelinating diseases have palpable/hypertrophic changes (onion bulbs)?
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Chronic idiopathic demyelinating polyneuropathy
Dejerine Sottas Refsum's disease |
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Adrenoleukodystrophy and Refsum's disease affects what cell organelle?
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Peroxisome
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A patient in their 40s comes in with pigmentary degeneration of their retina, and chronic distal and symmetrical sensorimotor neuropathy. What is a ddx?
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Refsum's disease
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What is the pathogenesis of chronic idiopathic demyelinating polyneuropathy (CIDP)?
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Abnormal immune response
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At what age does Charcot-Marie Tooth usually appear?
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First or second decade
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At what age does Refsum's disease usually appear?
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Second or third decade
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What is the difference between Charcot-Marie-Tooth and Dejerine Sottas in terms of inheritance?
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Charcot-Marie-Tooth is autosomal dominant
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Selective frontal and temporal atrophy is characteristic of what disease?
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Pick's disease
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What are common causes of death of patients with Alzheimer's?
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Pneumonia
Cardiovascular accident MI Pulmonary embolus |
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What susceptibility gene is seen in sporadic Alzheimer's?
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ApoE gene
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What do neurofibrillary tangles look like?
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"flame shaped" cytoplasmic inclusions
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What are neurofibrillary tangles composed of?
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Paired filaments that are twisted around each other-abnormal neurofilament protein, abnormal phosphorylated tau, ubiquitin
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What genes and chromosomes are involved in familial Alzheimer's?
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Presenilin 1 (chromosome 14)
Presenilin 2 (chromosome 1) |
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In what brain structures is there decreased Ach in Alzheimer's disease?
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Neocortex and hippocampus
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What nucleus is the major source of Ach to hippocampus and neocortex?
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Nucleus of Meynert (substantia inominata)
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What is abnormal about amyloid precursor protein (APP) in patients with dementia or diseases that present with dementia?
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APP usually undergoes alpha cleavage. Beta cleavage results in AB amyloid that aggregate into amyloid fibrils
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What are some of the mechanisms of neuronal degeneration seen in neurodegenerative diseases?
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Protein accumulation
Oxidative stress Apoptosis Cytokines Excitotoxicity |
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What is the role of tau protein in cells?
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Helps stabilize tubulin--found in microtubules--cytoskeleton and cellular transport
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Where do neuritic plaques usually accumulate?
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Neutropil
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What are neuritic plaques composed of?
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Abnormal neurites (ends of degenerating axons), microglia, astrocytes, amyloid core
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What MUST be found in order to diagnose Parkinson's?
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Lewy bodies?
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What do Lewy bodies look like?
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Glassy, round, eosinophilic cytoplasmic inclusions
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What do Lewy bodies consist of?
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Alpha synuclein, ubiquitin
Sphingomyelin |
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What gene is abnormal/involved in familial cases of ALS?
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Cu/Zn superoxide dismutase gene (SOD-1) -- involved in regulating intracellular free radicals
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Ballooning of motor neurons or axons is found in what motor system disease?
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ALS
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What is the pathology of Werdnig Hoffman?
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Loss of anterior horns in spinal cord (floppy baby)
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Hypertrophic cardiomyopathy is found in what hereditary ataxia?
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Friedreich's ataxia
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Why is iron found in the heart in Friedreich's ataxia?
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Frataxin protein is abnormal--important for mitochondrial iron regulation --free radical damage
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Friedreich's ataxia has degeneration in what areas of the CNS?
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Cerebellum and spinal cord
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Combined subacute degeneration (Vit B12 neuropathy) has the same spinal cord degeneration as what disease?
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Friedreich's ataxia
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Megaloblastic anemia is associated with what vitamin deficiency?
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B12 (cyanocobalamin)
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What symptom is the first to appear in mercury poisoning?
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Ataxia
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What is a key cofactor in the synthesis of dopamine?
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Iron
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What are some positive symptoms of schizophrenia?
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Hallucinations
Delusions Bizarre behavior |
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What are some negative symptoms of schizophrenia?
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Ambivalance
Social withdrawal Flat affect Attention difficulties Autistic behaviors |
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What kind of tumors do children get?
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Pilocytic astrocytoma
Medulloblastoma Ependymoma |
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What kind of tumors do adults get?
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Fibrillary astrocytomas
Metastases Meningiomas |
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What is the most common primary brain neoplasm?
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Glioblastoma multiforme
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