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抄録 Creutzfeldt-Jakob (C-J)症候群の発病から増悪期にかけての主たる精神症状は急速に進行する痴呆であるとするのが一般的である。その内容をより明らかにする目的で,臨床・病理学的に確認された自験5例および過去の報告例20例のC-J症候群について,発病初期の精神症状を主としてその時間経過に注目して検討した。
その結果,精神症状発症よりはじめて専門医を受診するまでの期間が,自験5例の平均は3.3±2.5ヵ月,報告例のそれは1.5±1.5カ月と短かく,比較のため調べたAlzheimer病では2.5±1.5年であった。自験例のうち全経過が112カ月と極端に長かった1例でも発症より初回受診までの期間は7カ月であった。また,この長期生存例を除く自験4例と報告例20例について,発症より初回受診まで,発症より失外套状態に達するまで,失外套状態の侍続,および全経過期間をみると,それぞれ2.4±1.5ヵ月と1.5±1.1ヵ月,4.3±3.3ヵ月と3.9±3.6ヵ月,4.0±3.7ヵ月と3.2±2.5ヵ月および8.3±6.2ヵ月と8.6±4.8ヵ月であり,差はなかった。自験5例は多少とも意識障害の特徴をあわせ持っており,その経過が亜急性であることと共に,C-J症候群の精神症状は亜急性脳症候群とするのがより妥当であろう。
The cardinal mental symptom in the developing stage of Creutzfeldt-Jakob syndrome is well known as rapidly progressive dementia. In this study, the characteristics of "rapidly progressive dementia" in Creutzfeldt-Jakob syndrome were investigated. Data was obtained by surveying our own observa-tions on 5 cases, and 20 additional cases from Japanese literature, with special attention on the temporal profile of these mental symptoms.
Five patients with C-J syndrome had at least one of the symptoms as seen in dementia, i. e. disorientation, memory disturbance or disturbance of verbal understanding. At the early stage, all of these patients also showed indifference to the surroundings or to themselves, or apparent conti-nuity with delirium or fluctuation of symptoms. These characteristics were considered to have close relationship with consciousness disturbance.
Of the temporal factors investigated during the developing stage, the following were noteworthy :periods from the onset of mental symptoms to the first psychiatric consultation were much shorter in 5 observed cases (3.3±1.1 months) and in 20 liter-ature cases (1.5±1.1 months) than in 10 cases with Alzheimer's disease (2.5±1.5 years). Even in Case 4, which had a very long survival course (112 months), the period from the onset to the first consultation was 7 months.
Most of our patients exclusive of Case 4 steadily progressed and finally reached to apallic state in 2 to 9 months (mean:4.3±3.3). The apallic state of these 4 patients lasted O. 1 to 8 months (mean : 4.0±3.7). The temporal profile of 20 cases in literature was similar to that of our own observa-tions.
The nature and the course of mental symptoms in observed and literature cases with C-J syndrome might be said to represent "subacute brain syn-drome intermediate form between consciousness disturbance and dementia.
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