title: Myoclonus.
abstract: Genetic studies have suggested homogeneity between the Baltic-type and Mediterranean-type progressive myoclonic epilepsy. Magnetoencephalography was applied to elucidate the mechanism underlying the giant evoked responses in cortical reflex myoclonus. A new concept of negative myoclonus mediated by cortical reflex mechanism was proposed. Cortical myoclonus was demonstrated in various neurodegenerative or metabolic disorders, such as presenile or senile dementia, olivopontocerebellar atrophy, and myoclonus epilepsy associated with ragged-red fibres. Myoclonus in corticobasal degeneration is especially noteworthy because it has clinical and electrophysiological features of cortical reflex myoclonus, but its latency is shorter compared with the conventional cortical reflex myoclonus. Clinical features of 'palatal myoclonus' were reported by the name of 'palatal tremor'.
authors.affiliation: Department of Brain Pathophysiology, Kyoto University School of Medicine, Japan.
authors.forename: H
authors.initials: H
authors.lastname: Shibasaki
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D001933:Brain Stem / Q000503:physiopathology; D002531:Cerebellum / Q000503:physiopathology; D002540:Cerebral Cortex / Q000503:physiopathology; D002648:Child; D004252:DNA Mutational Analysis; D004569:Electroencephalography; D004831:Epilepsies, Myoclonic / Q000235:genetics / Q000503:physiopathology*; D005071:Evoked Potentials / Q000502:physiology; D005808:Genes, Recessive / Q000235:genetics; D006801:Humans; D015810:Linkage Disequilibrium; D008875:Middle Aged; D009849:Olivopontocerebellar Atrophies / Q000235:genetics / Q000503:physiopathology; D010641:Phenotype; D012018:Reflex / Q000502:physiology; D013116:Spinal Cord / Q000503:physiopathology