Alexander's disease: unique presentation.

Abstract:

:Subacute necrotizing encephalomyelopathy (Leigh syndrome) refers to a nebulous disease entity characterized by lactic acidosis, a wide variety a clinical manifestations, and a consistent conglomeration of pathologic findings. Several abnormalities in metabolism have been delineated in association with Leigh syndrome, but many cases have no identified metabolic abnormality. We report a case that clinically, metabolically, and neuroradiologically appeared to be Leigh syndrome. In addition, our patient exhibited other unusual clinical findings, including ocular motility abnormalities. Neuropathologically, however, the diagnosis of Alexander's disease was confirmed. A review of the literature failed to find other cases of Alexander's disease reported with the metabolic abnormalities and clinical manifestations with which our patient presented.

journal_name

J Child Neurol

authors

Gingold MK,Bodensteiner JB,Schochet SS,Jaynes M

doi

10.1177/088307389901400510

subject

Has Abstract

pub_date

1999-05-01 00:00:00

pages

325-9

issue

5

eissn

0883-0738

issn

1708-8283

journal_volume

14

pub_type

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