CONTINUOUS MUSCLE-FIBER ACTIVITY (ISAACS SYNDROME) IN INFANCY - A REPORT OF 2 CASES

Authors
THOMAS NH HECKMATT JZ RODILLO E RANSLEY YF DUBOWITZ V
Citation
Nh. Thomas et al., CONTINUOUS MUSCLE-FIBER ACTIVITY (ISAACS SYNDROME) IN INFANCY - A REPORT OF 2 CASES, Neuromuscular disorders, 4(2), 1994, pp. 147-151
Citations number
21
Categorie Soggetti
Neurosciences,"Clinical Neurology
Journal title
Neuromuscular disorders → ACNP
ISSN journal
09608966
Volume
4
Issue
2
Year of publication
1994
Pages
147 - 151
Database
ISI
SICI code
0960-8966(1994)4:2<147:CMA(SI>2.0.ZU;2-4
Abstract
Two cases of continuous muscle fibre activity in infancy are reported. Both infants were severely affected and died from respiratory failure . Treatment with phenytoin and carbamazepine produced only temporary i mprovement. Electrophysiological and pharmacological evidence suggests that the site of the lesion in infancy, as in older patients, is in t he terminal nerve endings or motor end plate. Continuous muscle fibre activity occurring in infancy seems to be more severe than in older ch ildren or adults and seems refractory to treatment.