Abstract
Intelligence scale (WISC) and electroencephalographic record (EEG) were investigated on the 88 patients of progressive muscular dystrophy in Shimoshizu Hospital. Age ranged from 6 to 16 and disability stage was I to III, mainly II. Types of disease were as follows: Duchenne 80, limb-girdle 4, facioscapulohumeral 1, congenital 2, myotonic 1.
Distribution of intelligence quotient (IQ) was similar to normal control except the lowest one (IQ under 79), which was observed in 38% of all cases.
EEG were recorded two to three times about one patient and sleep induction was performed by ingestion of γ-hydroxy butyric acid syrup. The results of EEG examination of muscular dystrophy were as follows: normal 11.2%, borderline 28.8%, abnormal 60.0% in Duchenne; abnormal 1/4 in limb-girdle, 0/1 in facioscapulohumeral, 1/2 in congenital, 1/1 in myotonic and 4/16 (23%) in healthy control. Abnormal EEG findings were negative spike 31, spike & wave complex 9, 14 & 6c/s positive spikes 14, sharp wave 10, theta (sporadic or burst) 57, delta (sporadic or burst) 29, fast rhythm 10, low voltage 11, etc. in Duchenne dystrophy. Fourteen & 6c/s positive spikes were rare in others and healthy controls.
High incidence of intellectual and EEG abnormalities in the muscular dystrophy may be due to the undetermined disturbance in the brain which is caused by the same genetic defect for the dystrophic change in muscle.
