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 Neuroljournal_title
Journal of child neurologyauthors
Gingold MK,Bodensteiner JB,Schochet SS,Jaynes Mdoi
10.1177/088307389901400510subject
Has Abstractpub_date
1999-05-01 00:00:00pages
325-9issue
5eissn
0883-0738issn
1708-8283journal_volume
14pub_type
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