両側脳内出血後いわゆるcortical deafnessのみられたモヤモヤ病の1例

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This paper presents one moyamoya case which developed so called cortical deafness following bilateral intracerebral hemorrhages. The patient is right handed 18 year-old girl whose moyamoya vessels were found incidentally when she had head-injury at 10 years of her age.<BR>She has had hemorrhagic episodes in two times. The first attack, on May, 30, 1976, was right putaminal hemorrhage and treated conservatively. The second one, on Oct., 16, 1979 was left temporal subcortical massive hemorrhage and removed the hematoma of 150g by craniotomy. On 7th postoperative day, she seemed to be fully awake, however, she did not respond to our call at all. On 42nd day, she could make contact only by means of writing, and still could not utter any useful words at all. The results of Standard Examination for Aphasia on Jan., 20, 1980, were as follows: words repeating, comprehension of languages, writing letters and sound differentiation were disturbed markedly; reading with loud voice and sense of the sound direction were disturbed slightly; spontaneous speech or writing and comprehension of a letter were normal. Several neuro-otological examinations were performed subsequently. The pure tone audiometry showed severe hearingloss on both sides on 42nd day after the second attack, but 6 months later it recovered to the point of minimum damage. The speech audiometry, however, pointed out her complete disability of discriminating speech bilaterally even on 6 months after the second attack. The auditory brain stem response by click sounds was normal. But the middle latency component was abnormal and no clear wave components were identified. All components of the waves of the slow vertex potential were obtained in almost normal patterns, but the latencies were delayed modelately.<BR>From the clinical features and otological examinations, it is presumed that she had so called cortical deafness in early stage, and then turned gradually to the auditory agnosia or pure word deafness. It is also suspected from CT scans and neuro-otological examinations that the responsible lesions to these symptomes are bilateral auditory radiations at sub-lenticular portion of the internal capsules and they do not include the medial geniculate bodies. In this case, not only the left transverse temporal gyrus (Heschl), but also the right one might have played some role in addition to the auditory radiation.
The Japanese Society on Surgery for Cerebral Strokeの論文

両側脳内出血後いわゆるcortical deafnessのみられたモヤモヤ病の1例

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