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NEUROLOGY 1986;36:872
© 1986 American Academy of Neurology

Fatal metabolic acidosis, hyperglycemia, and coma after steroid therapy for Kearns-Sayre syndrome

Richard G. Curless, John Flynn, Brian Bachynski, Jocelyn B. Gregorios, Paul Benke and Robert Cullen

University of Miami, Departments of Neurology, Ophthalmology, Pathology, and Pediatrics, Miami Children's Hospital, Division of Neurology, Miami, FL.

Two adolescent boys with Kearns-Sayre syndrome (progressive external ophthalmoplegia, heart block, elevated CSF protein, and ragged-red muscle fibers) developed lethargy, increasing somnolence, polydipsia, polyphagia, and polyuria after a brief course of steroid therapy. Both had hyperglycemia and acidosis. Nonketotic, lactic acidosis was present in one and ketosis in the other. Severe respiratory failure developed, and both patients died. Postmortem revealed fatty infiltration of the pancreas in addition to a diffuse spongiform encephalopathy.

Address correspondence and reprint requests to Dr. Curless, University of Miami School of Medicine, Department of Neurology, Mailman Center D-820. P.O. Box 016820, Miami, FL 33101.

Presented in part at the thirty-seventh annual meeting of the American Academy of Neurology, Dallas, TX, April 1985.

Accepted for publication October 17, 1985.




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