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Journal of Child Neurology, Vol. 14, No. 5, 325-329 (1999)
DOI: 10.1177/088307389901400510

Alexander's Disease: Unique Presentation

Monique K. Gingold, MD

Departments of Pediatrics and Neurology

John B. Bodensteiner, MD

Departments of Pediatrics and Neurology

Sydney S. Schochet, MD

Departments of Neurology and Pathology

Margaret Jaynes, MD

Departments of Pediatrics and Neurology West Virginia University Health Sciences Center Morgantown, West Virginia

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 pathlogic 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 (J Child Neurol 1999;14:325-336).


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