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NEUROLOGY 1977;27:971
© 1977 American Academy of Neurology

Ophthalmoplegia as a sign of metabolic disease in the newborn

JOHN T. MacDONALD, M.D. and PHYLLIS K. SHER, M.D.

Department of Neurology, University of Miami Medical Center, Miami, Florida.

Fluctuating ophthalmoplegia in the neonatal period is unusual. We report two infants, one with branched-chain keto-aminoaciduria, the other with nonketotic hyperglycinemia, who demonstrated varying degrees of ophthalmoplegia. In one, the abnormalities of extraocular motility best correlated with elevations of serum leucine. In the other case, increased CSF glycine was implicated. Varying ophthalmoplegia during the neonatal period should alert the clinician to consider an underlying metabolic disorder.

Reprint requests should be addressed to Dr. Sher, 4440 Sheridan St., Emerald Hills Medical Square, Hollywood, FL 33201.

Accepted for publication March 14, 1977.






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