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Neonatal ophthalmoplegia with microfibers

A reversible myopathy?

Departments of Neurology and Pediatrics, Albany Medical College, Eleanor Roosevett Developmental Services of the New York State Department of Mental Hygiene and The Veteran's Administration Hospital, Albany, New York.

An infant born with marked hypotonia showed prompt regression of skeletal muscle weakness, but by 7 weeks of age had total external ophthalmoplegia. Biopsy of the gluteus muscle at 14 days showed marked variation in fiber size with a large proportion of very small fibers (less than 3 µ). By 10 months of age, biopsy of the vastus was virtually normal. The inferior oblique muscle was replaced by fibrous tissue containing a few remaining degenerating fibers. The child was normal at 2 years of age except for mild facial weakness and ophthalmoplegia. This syndrome may be the result of a reversible intrauterine process.

Address reprints to Dr. Hanson, Departmentof Neurology, The Neil Hellman Medical Research Building, Albany Medical College, Albany, NY 12208.

Accepted for publication December 1976.