Electroencephalographic Studies in Hereditary Myopathy.

Recent reports by a number of authors have suggested that electroencephalographic (E.E.G.) abnormalities are often found in patients with progressive muscular dystrophy. Winfield, Britt, and Raskind (1958) reported the finding of nine abnormal records in 12 children with muscular dystrophy subjected to E.E.G. study. The abnormalities consisted of the pattern designated by Gibbs and Gibbs (1952) as 14 and 6 per second positive spikes. Wayne and Browne-Mayers (1959), in a study of 57 dystrophic patients of whom 21 were adults and 36 were children, found a total incidence of definite abnormality in 17 cases. The changes consisted of generalized abnormalities of various kinds and there was no correlation between the severity of the E.E.G. abnormalities and the clinical severity of the dystrophy. Ascione, Matano, and Serra (1959) found E.E.G. changes in five boys with muscular dystrophy, all in the same sib-ship. Thechanges consisted ofgeneralized abnormalities which developed during photic stimulation and hyperventilation. Perlstein, Gibbs, Gibbs, and Stein (1960) reported a large series of examinations on patients suffering from what the authors referred to as various forms of myopathy. Unfortunately, not all of their 101 patients were in fact cases of muscular dystrophy. Included in this series were 22 cases of amyotonia congenita; this is a clinical diagnosis, the syndrome commonly embracing primary neuropathic disorders such as infantile spinal musuclar atrophy (Werdnig-Hoffman disease), as well as various myopathic disorders. A further nine patients are reported to have had 'probable myopathies' but no further details are given. Of the 70 remaining patients, eight were suffering from facioscapulohumeral dystrophy and 62 were children with pseudohypertrophic dystrophy. In these two groups the incidence of abnormalities in the E.E.G. was 45% (28 patients) in the children and 37% (three patients) in the group with facioscapulohumeral dystrophy. The most commonly observed abnormalities were 14 and 6 per second positive spikes and these were seen particularly in the children. Di Gruttola, Fanuele, and Berni Canani (1961) studied 12 patients, all children with pseudohypertrophic muscular dystrophy. They found E.E.G. abnormalities in 10 patients, the changes consisting of slow dominant rhythms and generalized excesses of slow activity; they did not observe any 14 or 6 per second positive spikes.