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NEUROLOGY 1978;28:631
© 1978 American Academy of Neurology

Chlordecone intoxication in man

II. Ultrastructure of peripheral nerves and skeletal muscle

A. JULIO MARTINEZ, M.D., JOHN R. TAYLOR, M.D., PETER JAMES DYCK, M.D., SIDNEY A. HOUFF, M.D. and EDWARD ISAACS, M.D.

University of Pittsburgh School of Medicine,' Presbyterian-University Hospital (Dr. Martinez); The Medical College of Virginia, and Virginia Commonwealth University Departments of Pathology (Neuropathology) and Neurology, Richmond (Drs. Taylor, Muff. and Isaacs); and the Mayo Clinic and Mayo Foundation. Department of Neurology, Rodrester, Minnesota (Dr. Dyck).

Tremors, mental changes, opsoclonus, muscle weakness, gait ataxia, incoordination, and slurred speech developed in several employees in a Virginia chemical plant during the summer of 1975. Epidemiologic and clinical studies suggested that the chlorinated insecticide chlordecone (Kepone®) was responsible. Severity of symptoms seemed directly related to dose and duration of exposure. Five sural nerve and six muscle biopsy specimens were examined by light microscopy and electronmicroscopy. The sural nerves were also evaluated by computerized morphometry, which showed considerable decrease in the number of unmyelinated fibers and lesser abnormalities of myelinated fibers. Compared with the nerves of the control subjects, those of patients may have had an increase in Reich and Elzholz bodies, and a modest increase in endoneurial collagen. There were occasional "collagen pockets," stacks of Schwann cell cytoplasmic membranes, redundant Schwann cell cytoplasmic folds, and fewer unmyelinated axons. The skeletal muscles contained increased amounts of lipofuscin and lipid like droplets in subsarcolemmal areas and within intermyofibrillary spaces; the significance of this is unknown. Fiber size variability, type I predominance, and type grouping were present in three cases. All results strongly suggest that chlordecone is a neurotoxic agent predominantly affecting Schwann cells and unmyelinated fibers of peripheral nerves.

Requests for reprints should be addressed to Dr. A. Julio Martinez, Pathology Department (Neuropathology), University of Pittsburgh School of Medicine, Pittsburgh, PA 15261.

Accepted for publication August 4, 1977.




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[Abstract] [PDF]




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