title: EMG study in the diagnosis and differential diagnosis of lipid storage myopathy.
abstract: Clinical, electromyographic and pathological features were studied in 18 patients with lipid storage myopathy (group I) and 18 patients with polymyositis and dermatomyositis (group II). The results showed a remarkable lower spontaneous activity(SA) incidence (14%) in group I than that (55%) in group II; 46% and 34% short-duration motor unit potentials(MUAPs) with polyphasic potentials and 74% and 71% short-duration MUAPs without polyphasic potentials respectively; the percentages of increased polyphasic MUAPs were same in the two groups. The reduced or pathologic interference patterns accounted for 61% in the group I and 50% in group II. Increased CPK, LDH and HBD were also found in both of them. It is suggested that the lipid storage myopathy may be diagnosed when patients have muscle weakness and myalgia with short-duration and low-amplitude and polyphasic MUAPs without or with occasional spontaneous activities, and increased CPK, LDH and HBD.
authors.affiliation: Department of Neurology, Peking Union Medical College Hospital, CAMS & PUMC, Beijing 100730.
authors.forename: L
authors.initials: L
authors.lastname: Cui
authors.forename: X
authors.initials: X
authors.lastname: Tang
authors.forename: Q
authors.initials: Q
authors.lastname: Zhang
authors.forename: B
authors.initials: B
authors.lastname: Li
authors.forename: H
authors.initials: H
authors.lastname: Du
authors.forename: Y
authors.initials: Y
authors.lastname: Guo
mesh_terms: D000293:Adolescent; D000328:Adult; D002331:Carnitine / Q000172:deficiency; D002334:Carnitine O-Palmitoyltransferase / Q000172:deficiency; D002648:Child; D002675:Child, Preschool; D003882:Dermatomyositis / Q000175:diagnosis; D003937:Diagnosis, Differential; D004576:Electromyography; D005260:Female; D006801:Humans; D008064:Lipidoses / Q000175:diagnosis*; D008297:Male; D008875:Middle Aged; D017285:Polymyositis / Q000175:diagnosis*
chemical_list: D002334:Carnitine O-Palmitoyltransferase; D002331:Carnitine