B&b: Quiz 2: Myelin Disorders

Front 1

Spongiform Encephalopathy

Back 1

degeneration of neurons and vacuolization due to accumulations of prion proteins

Front 2

Prions

Back 2

infectious proteins

Front 3

Creutzfeldt-Jakob disease

Back 3

largely sporadic human prion disease; usually due to an inherited mutation of the prion gene, iatrogenic or dietary exposure to prions

Front 4

Amyloid Plaques

Back 4

accumulations of insoluble proteins or prions

Front 5

Demyelination

Back 5

the destruction of myelin

Front 6

Leukodystrophy

Back 6

the disruption of myelin metabolism

Front 7

Multiple Sclerosis (MS)

Back 7

a chronic demyelinating disease defined by multifocal demyelinated plaques; most common chronic CNS disease of young adults (USA).

Front 8

Optic Neuritis

Back 8

incomplete (blurred vision) or complete loss of vision due MS lesions of the optic nerve

Front 9

MS Dx and Tx

Back 9

Dx: See ↑ IgG in CSF with oligoclonal bands

Front 10

Internuclear Ophthalmoplegia

Back 10

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

Front 11

Transverse Myelitis

Back 11

acute demyelination within the spinal cord causing weakness of one or both legs and numbness in the lower extremities

Front 12

Krabbe Disease (Globoid Cell Leukodystrophy)

Back 12

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)

Front 13

GBS

Back 13

Auto-AB for PNS myelin leads to progressive, ascending symmetric muscle weakness and low/absent DTR.

Front 14

cause of GBS

Back 14

often preceded by a flulike illness caused by campylobacter

Front 15

GBS pathophys

Back 15

Infection → Ab to PNS myelin → inflammation → degrade myelin → ↓ motor/sensory/autonomic function. Loss of patches of myelin decreases nerve transmission speed.

Front 16

GBS dx

Back 16

Clinical presentation and CSF - ↑ protein, nl cell count, Most survive and recover

Front 17

Compression neuropathy

Back 17

peripheral nerve is compressed w/in an anatomic compartment

Front 18

Carpal Tunnel Syndrome

Back 18

Affects median nerve. Numbness and paresthesias of the tips of the thumb and first two digits, women > men, bilateral is common.