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Late Onset Ataxia, Rigidity, and Peripheral NeuropathyA Familial Syndrome With Variable Therapeutic Response to Levodopa
Dewey K. Ziegler, MD;
R. Neil Schimke, MD;
John J. Kepes, MD;
Donald L. Rose, MD;
George Klinkerfuss, MD
Arch Neurol. 1972;27(1):52-66.
Abstract
A family had a polymorphous neurological syndrome transmitted in autosomal dominant fashion. The clinical syndrome is one of late onset cerebellar ataxia, muscular rigidity, bradykinesia, dysarthria, fasciculations, muscle atrophy, and spasticity appearing in various combinations in affected individuals. Pathological studies on one case have revealed degeneration in spinocerebellar tracts, Purkinje cells and dentate nuclei of cerebellum, dorsal root ganglion cells, and cauda equina nerve roots. Peripheral neuropathy was present in several cases, as evidenced by demyelinating changes on sural nerve biopsy, and by characteristic changes in nerve conduction times and electromyography. Such changes on occasion preceded clinical signs of peripheral nerve disease. Several cases showed a therapeutic response to levodopa; only the symptom of rigidity was alleviated.
Author Affiliations
Kansas City, Kan; St. Louis
From the Department of Medicine, sections of neurology (Dr. Ziegler) and genetics (Dr. Schimke), departments of pathology (Dr. Kepes) and physical medicine (Dr. Rose), Kansas University Medical Center, Kansas City, Kan, and the Department of Neurology, Washington University, St. Louis (Dr. Klinkerfuss).
Footnotes
Accepted for publication March 2, 1972.
Reprint requests to Section of Neurology, University of Kansas Medical Center, Rainbow Blvd at 39th St, Kansas City, Kan 66103 (Dr. Ziegler).
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