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Immune Disorders

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Rasmussen’s syndrome is an autoimmune disease involving one hemisphere, with cortical inflammation and atrophy, and thus this is a secondary myoclonus. Pathophysiology is unknown. Despite the identification of antiglutamate receptor antibodies in some children, immune-modulating therapies do not have long-term benefit and only hemispherectomy is curative.66 A case has also been described after gliomatosis cerebri in two children.67 Some authors have suggested that EPC be used to designate cases with cortical origin, and myoclonia continua be used for those originating elsewhere in the nervous system.68