Thursday, November 02, 2006

Alexander Disease

Alexander Disease                                         40

General: Rare degenerative neurologic disorder characterized by diffuse demyelination in the presence of Rosenthal fibers; cases may resemble multiple sclerosis; neuroradiologic findings include increased cerebellar white matter hyperintensity and diffuse periventricular signal hyperintensities.

Ocular: Impaired smooth pursuit; gaze-evoked horizontal nystagmus; slowed saccades; ocular myoclonus.

Clinical: In infants, hydrocephalus, spasticity, and seizures; in juveniles and adults, bulbar palsy and hyperreflexia, intermittent neurologic dysfunction.

Reichard EAP, Ball WS, Bove KE. Alexander disease: a case report and review of the literature. Pediatr Pathol Lab Med 1996; 16:327-343.

Russo LS, Aron A, Anderson PJ. Alexander's disease: a report and reappraisal. Neurology 1976; 26:607-614.

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