The ultrastructural cytochemical study of a brain biopsy and the biochemical study of a muscle biopsy from a 20-year-old girl with typical familial progressive myoclonic epilepsy of Lafora body type yielded the following findings: No evidence of acid phosphatase activity was observed in the great majority of the Lafora bodies, indicating that they do not undergo a hydrolytic degradative process. Both filamentous and amorphous components of Lafora bodies were stained with silver proteinate, a method to detect polysaccharides, and both were digested after incubation with \g=g\-amylase,suggesting that both components of Lafora bodies are polyglucosans, probably in a different state of aggregation. The biochemical study of the muscle revealed twice the normal concentration of glycogen but no defect was found in the activities of the main enzymes of the glycogenosynthetic and glycogenolytic pathways. (25:483-493, 1971)
Myasthenia gravis (MG) is a complex autoimmune neurologic disorder of unknown etiology, characterized by fluctuating skeletal muscle weakness most commonly involving the muscles of the head, neck, and upper extremities. Autoantibodies directed against acetylcholine receptors on the postjunctional membrane decrease the numbers of functional acetylcholine receptors and cause membrane alterations, resulting in neuromuscular transmission failure. Diagnosis is established by history and physical examination, the "Tensilon Test," and acetylcholine receptor antibody titers. Treatment modalities include drug therapy, thymectomy, and plasmapheresis. The drugs most commonly employed are anticholinesterases, corticosteroids, and immuno-suppressive agents. Cyclosporine and intravenous immunoglobulin are promising investigational treatments. The purpose of the article is to review current concepts in the pathophysiology, immunopathology, diagnosis, and treatment of MG. Special emphasis is placed on the autoimmune form of the disease and the drugs employed in its management. Standard regimens as well as some experimental treatment modalities are reviewed.
scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.