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Juvenile Leigh's Encephalomyelopathy With Peripheral Neuropathy, Myopathy, and Cardiomyopathy

Department of Neurology, University of Connecticut Health Center, Farmington, Department of Pathology, University of Connecticut Health Center, Farmington

Edwin L. Zalneraitis, MD

Department of Neurology, University of Connecticut Health Center, Farmington, Department of Pediatrics, University of Connecticut Health Center, Farmington

Barry S. Russman, MD

Department of Pediatric Neurology, Newington Children's Hospital, Newington, CT

Morven C. Barwick, MD

Department of Neurology, University of Connecticut Health Center, Farmington

A child with typical histopathologic changes of Leigh's subacute necrotizing encephalomyelopathy presented with a chronic demyelinating neuropathy. During her 11-year course, she developed an unusual myopathy and cardiomyopathy in addition to many of the previously described manifestations of Leigh's disease. Despite an extensive evaluation, the biochemical basis of her condition was never identified. This case demonstrates another unique constellation of clinical alterations associated with subacute necrotizing encephalomyelopathy, and that chronic demyelinating neuropathy can be an important initial presentation of the disease. (J Child Neurol 1991;6:159-163).

Journal of Child Neurology, Vol. 6, No. 2, 159-163 (1991)
DOI: 10.1177/088307389100600214


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