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Clinical and Ultrastructural Abnormalities

Dennis M. D. Landis, MD; Roger N. Rosenberg, MD; Story C. Landis, PhD; Lawrence Schut, MD; William L. Nyhan, MD, PhD

Arch Neurol. 1974;31(5):295-307.

Abstract
Thirty-three persons in the Schut-Swier kindred at risk for autosomal dominant olivopontocerebellar degeneration have been examined and nine new cases identified. These affected individuals manifested severe cerebellar deficits; abnormalities of cortical, bulbar, and spinal motor neurons; and posterior column dysfunction. Cerebellar biopsy material from two patients revealed extensive progressing loss of Purkinje cells, degeneration of cortical afferents, and variable loss of granule cells. Vermiform tubules that resemble paramyxovirus nucleocapsids were found in association with crystalline inclusions in both biopsy specimens.

Author Affiliations
From the departments of medicine (Dr. D. Landis), neurosciences (Drs. Rosenberg and S. Landis), and pediatrics (Drs. Schut and Nyhan), School of Medicine, University of California at San Diego, La Jolla, Calif. Dr. Dennis Landis is now with the Laboratory of Experimental Pathology, National Institute of Arthritis, Metabolism, and Digestive Diseases, Bethesda, Md. Dr. Rosenberg is now with the Department of Neurology, Southwestern Medical School, University of Texas at Dallas. Dr. Story Landis is now with the Laboratory of Neuropharmacology, Division of Special Mental Health Research, Saint Elizabeth's Hospital, Washington, DC.

Footnotes
Accepted for publication Jan 23, 1974.

Reprint requests to Department of Neurology, Southwestern Medical School, University of Texas at Dallas, Dallas, TX 75235 (Dr. Rosenberg).

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