小児の遺伝性脳変性疾患の脳波—2．汎発性脳硬化症及びGargoylism

ELECTROENCEPHALOGRAPHIC STUDIES ON HEREDODEGENERATIVE DYSORDERS OF CENTRAL NERVOUS SYSTEM IN CHILDREN : 2.DIFFUSE SCLEROSIS AND GARGOYLISM

　前編において，家族性黒内障性白痴と診断された症例について，脳波所見の概略を述べた。今回は，病理解剖学的，又は臨床的に，それに類似な所見を示す汎発性脳硬化症及び，Gargoylismの症例について得られた脳波所見を示し，黒内障性白痴において得られた所見と比較，検討を行つた所を述べる。

Electroencephalograms in 2 autopsied cases of diffuse sclerosis and 3 cases of Gargoyli-sm were presented.

The age of onset of the symptoms in a girl of diffuse sclerosis was 1 year and 3 months. She had 5 attacks of vomiting with acidosis lasting 10 to 20 days until 4 years and 5 months of age when she died. The serial EEG records in this case showed symmetrical theta or alpha waves of low ampulitude. High voltage theta or delta waves of more than 50 micro volt had never been found even in sleeping records throughout the cour-se of 3 years. Spike discharge or side diffe-rence was not detected. The main symptoms in another case, a boy, of diffuse sclerosis were generalized convulsions, massive myo-clonic seizures sometimes associated with laughing spells, and progressive lethargy. His EEG at the age of 5 months when his spells developed showed diffuse dysrhythmia composed of high voltage slow waves. The EEG at the age of 6 months revealed diffuse dysrhythmia intermixed with spikes or spi-ke-wave complex. In the EEG of 8 months, the spike-wave complexes tended to become grouped in paroxysms, and symmetrical spi-ke-wave complexes were observed dominantly in the parietal region.