肝レンズ核変性症（Wilson病）の脳波

ELECTROENCEPHALOGRAPHIC STUDY OF HEPATOLENTICULAR DEGENERATION (WILSON'S DISEASE)

はじめに

　いわゆる「肝性昏睡」や肝脳変性疾患特殊型の脳波所見については，すでに多くの知見が公けにされているが，それに較べれば肝レンズ核変性症（以下，HLDと略す）での報告は乏しいようである。われわれは，剖検によりHLDの確定された1例を含む2家系につき，その脳波所見を述べる。

Electroencephalograms were studied of three patients (8 records), including a proband (A. K.), her younger brother (T. K.) and a case (M. I.) from another pedigree, who were clinically diagnosed as hepatolenticular degeneration (Wilson's disease). EEGs of three members (3 records) belonging to the proband's family were also considered, even though they did not exhibit the symptom of this disease. The proband (A. K.) was verified at autopsy that she was a victim of this disease.

The EEGs taken from the three patients (Case 1; A. K., Case 2 ; T. K., Case 6 ; M. I.) were ab-normal, but did not show any pattern character-istic of this illness. In the records of Cases 1 and 2, slow waves appeared more predominantly in re-lation with their progresses of clinical exacerbara-tion. Moreover, in the records of Case 1, mu rhythm and 7 Hz positive spikes were observed from time to time in the course of clinical observation for one and a half years, and subharmonic driving was occasionally induced by photic stimulation.

Discussion was made on the present findings in comparison with the earlier ones in literatures, and possible correlation was considered between the EEG abnormalities and the macroscopic changes found in the brain of the autopsied proband.