Clinical Manifestations and Pathology of Progressive Myoclonus Epilepsy

Objective To explore the various types of progressive myoclonus epilepsy seizure characteristics,diagnostic strategies,and pathological features.Methods 12 cases of progressive myoclonus epilepsy were analyzed with the clinical characteristics,the routine laboratory examinations,the pathological examination by light and electron microscopy to extra cranial.Results 12 cases carried out routine examinations,neural electrophysiological examinations and physical examinations. The result showed that there 5 patients diagnosed with Neuronal Ceroid Lipofuscinoses,5 patients with MERRF,1 patient with Lafora Disease,1 patient with Unverricht-Lundborg disease.Conclusion Progressive myoclonus epilepsy is a group of rare myoclonus epilepsy syndrome. It can be early diagnosed and properly classified with detailed medical history,characteristics of the EEG,and physical examination of extra cranial tissue,especially electron microscopy examination.