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Progressive Myoclonus Epilepsy With Lafora Inclusion BodiesII. Studies of Ultrastructure
D. Louise Odor, PhD;
Richard Janeway, MD;
Larry A. Pearce, MD;
J. R. Ravens, MD;
Winston-Salem, NC
Arch Neurol. 1967;16(6):583-594.
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Lafora's AFORA'S disease is a rare form of myoclonus epilepsy.1 It is characterized by myoclonus, generalized convulsions, and progressive mental deterioration which begin during adolescence. Death within four to ten years has been the inevitable outcome in all reported cases.
This report describes the ultrastructure of the inclusion bodies found in cerebral cortical biopsy specimens from two patients with Lafora's disease. The ultrastructure of liver and gastrocnemius muscle are also briefly described. Detailed clinical, genetic, histopathologic, and biochemical data have been separately reported2-4 so that only brief clinical reports are given here.
Report of Cases
CASE 1.—A 19-year-old white boy, product of the equivalent of double-first cousin consanguineous parentage, had developed normally until age 17. Generalized convulsions began and soon were associated with diffuse asynchronous myoclonus, mental deterioration, and dystaxia. A cerebral cortical biopsy showed neuronal inclusion bodies of the Lafora type. Biochemical assays of glycogen and
. . . [Full Text PDF of this Article]
Author Affiliations
From the departments of anatomy, neurology, and pathology, Bowman Gray School of Medicine, Winston-Salem, NC.
Footnotes
Submitted for publication Sept 15, 1966; accepted Dec 3.
Read in part at the meeting of the American Neurological Association, Atlantic City, NJ, June 14-16, 1965.
Reprint requests to Department of Neurology, Bowman Gray School of Medicine, Winston-Salem, NC 27103 (Dr. Janeway).
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