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Congenital retardation and central motor defect with later evolution of seizure disorder, orofacial dysplasia, and amyotrophy

A clinicopathologic report

From the Departments of Neurology and Neurological Surges (Neurology), Pathology, and Pediatrics, Washington University School of Medicine, St. Louis, and the Departments of Pediatrics and Neurology, University of Colorado School of Medicine, Denver.

Retarded mental and motor development was observed during the first year of life of a full-term female who had been delivered with difficulty. Generalized psychomotor seizures and dysplastic "acromegalic" facial changes began to develop when she was 8 years old, and generalized amyotrophy developed over the next several years. The course was generally progressive, and she died at age 31. A variety of clinical laboratory studies were nondiagnostic. Autopsy findings showed normal brain weight and normal-appearing cerebral hemispheres, a peculiar atrophy of the inferior portion of the cerebellar hemispheres, a demyelinating process in the dorsal columns of the cervical and thoracic spinal cord, and lateral column and motor neuron degeneration. The pathogenesis of the syndrome is undetermined.

Dr. Landau's address is Department of Neurology and Neurological Surgery, Barnes Hospital Plaza, 660 So. Euclid Ave., Box B111, St. Louis, MO 63110.

Received for publication December 19, 1975.

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				W. J. Peacock, L. A. Staudt, D. Burke, E. Pierrot-Deseilligny, P. D. Neilson, W. A. Herndon, I. Rapin, W. M. Landau, C. C. Hunt, A. Askenasi, et al. Selective Posterior Rhizotomy: Further Comments J Child Neurol, April 1, 1991; 6(2): 173 - 184. [PDF]