ABSTRACT
Relatamos três casos de síndrome de Isaacs, que apresentavam mioquímia clínica, cãibras, dificuldades para o relaxamento muscular, hipertrofia muscular e aumento da sudorese. A eletromiografia de agulha mostrou atividade muscular contínua involuntária, caracterizada como descargas mioquímicas. Os estudos da condução nervosa foram normais. Biópsia de músculo, realizado nos três casos, mostrou atrofia de fibras do tipo 2. Dois casos apresentaram melhora clínica com a utilização de carbamazepina e um com prednisona.
Subject(s)
Adult , Humans , Female , Adolescent , Fasciculation/pathology , Biopsy , Electromyography , Fasciculation/physiopathologySubject(s)
Adult , Fasciculation/physiopathology , Humans , Male , Muscle, Skeletal/physiopathology , Pain/physiopathologyABSTRACT
Characteristically continuous facial myokymia is a pathognomonic, exceedingly rare physical sign of intrinsic brain-stem lesions e.g. multiple sclerosis (where the myokymia lasts only for a few months), pontine glioma (where it is unremitting for years). The physiopathogenesis is unclear. Electromyographic patterns are characteristic. Therapy and prognosis are related to the basic aetio-pathological process. Only two out of 132 cases of intrinsic brain-stem lesions in the department of Neurosurgery, Seth G.s. Medical College, Bombay over a period of 3 decades, exemplify its rarity. These two cases are reported here and the relevant literature is reviewed.