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18 Cards in this Set
- Front
- Back
Spongiform Encephalopathy
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degeneration of neurons and vacuolization due to accumulations of prion proteins
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Prions
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infectious proteins
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Creutzfeldt-Jakob disease
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largely sporadic human prion disease; usually due to an inherited mutation of the prion gene, iatrogenic or dietary exposure to prions
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Amyloid Plaques
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accumulations of insoluble proteins or prions
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Demyelination
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the destruction of myelin
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Leukodystrophy
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the disruption of myelin metabolism
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Multiple Sclerosis (MS)
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a chronic demyelinating disease defined by multifocal demyelinated plaques; most common chronic CNS disease of young adults (USA).
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Optic Neuritis
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incomplete (blurred vision) or complete loss of vision due MS lesions of the optic nerve
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MS Dx and Tx
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Dx: See ↑ IgG in CSF with oligoclonal bands
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Internuclear Ophthalmoplegia
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a lesion of the medial longitudinal fasciculus (MLF); disorder of conjugate lateral gaze in which the affected eye fails to adduct when the contralateral eye abducts (w/ nystagmus); most commonly due to MS plaques when presenting in a young person
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Transverse Myelitis
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acute demyelination within the spinal cord causing weakness of one or both legs and numbness in the lower extremities
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Krabbe Disease (Globoid Cell Leukodystrophy)
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rapidly progressive, fatal, AR neurologic disorder caused by deficiency of beta-galactosidase; appears in infancy and is characterized by "globoid cells" in the white matter (macrophages containing undigested galactosylceramide)
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GBS
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Auto-AB for PNS myelin leads to progressive, ascending symmetric muscle weakness and low/absent DTR.
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cause of GBS
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often preceded by a flulike illness caused by campylobacter
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GBS pathophys
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Infection → Ab to PNS myelin → inflammation → degrade myelin → ↓ motor/sensory/autonomic function. Loss of patches of myelin decreases nerve transmission speed.
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GBS dx
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Clinical presentation and CSF - ↑ protein, nl cell count, Most survive and recover
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Compression neuropathy
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peripheral nerve is compressed w/in an anatomic compartment
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Carpal Tunnel Syndrome
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Affects median nerve. Numbness and paresthesias of the tips of the thumb and first two digits, women > men, bilateral is common.
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