Creutzfeldt-Jakob病の1剖検例—初老期—退行期亜急性脳萎縮の理解への1寄与

An Autopsy Case of Creutzfeldt-Jakob Disease: A Contribution to the Understanding of Presenile-involutive Subacute Encephalopathies.

I．まえがき

　退行期と初老期の精神病は，精神神経科領域中最もdunkelな分野と考えられる。なかでもCreutzfeldt-Jakob病（以下C-J病と略す）は臨床症状，脳組織病理所見ともに複雑，多彩で，病因不明であり，文献中にもときどきいろいろの混乱がみられる。最近私たちは白木教授とともに亜急性海綿状大脳萎縮（subakute generalisierte spongiöse Atrophie mit dyskinetischem Endstadium，H. Jacob 1960；以下亜急性海綿状萎縮と略す）の2剖検例について研究し^＊＊＊，初老期ないし老年期の精神病の理解という広い視野から考察をおこなつた。その後さらに新しい文献を読み，またCreutzfeldt-Jakob病の1剖検例を経験した，その1例をここに報告し，あわせてC-J病と亜急性海綿状萎縮とを主としたいわゆる"Präsenil-involutive subakute myoklonisch-dyskinetische Encephalopathienの臨床，病理について，先にあげた亜急性海綿状萎縮に関する論文でおこなつた考察を敷延し，この種諸疾患の病理的鑑別診断と，臨床症状群としての特徴について整理を試み，理解の一助ともなればとねがうしだいである。

　In the present case a 29-year-old housewife progressed from initial symptom of abrupt mental deterioration accompanied with fever, pharyngitis, dysphagia to a mental state fluctuating from akinetic mutism to delirious confusion with quadriparesis in flexion, rheumatoid arthritis, paratonic rigidity, dysarthria, and continuous myoclonic-dyskinetic jerks of the upper part of her body. Infrequent grand mal seizures and a passing eruption with bulla on axillary and chest skin were also observed. At the end stage these abnomal movements disappeared. The total course of illness was 5 months.

　Significant data of clinical examinations were : CRP +5, ASLO 500, erythrocyte sedimentation rate (average) 80, blood serum gamma grobulin 60%, A/G 0.5, CoR. 5, CaR. 18, BSP (45') 2.5%, liquor negative, fundus oculi negative, low voltage EEG.

　At post mortem examination the patient appeared to have died of bronchopneumonia. Brain weighed 980g, and was small as the whole, without any macroscopic focal changes. Histological examination disclosed a diffuse degenerative changes in nerve cells including sclerosis, "primäre Reizung", severe cell changes, vacuole formation, edematous change and slight loss of neurons in the cerebral cortex. Cortical and subcortical diffuse astrocytic and microglial proliferations, neuronophagia, glia rosetts and klasmatodendrosis were also encountered. Regional order of severity in the hemisphere was: precentral; subiculum; frontal; occipital; temporal lobe; insula. The changes were predominant in the deep part of the cortex. Milder changes of similar nature were found in the basal ganglia, thalami, dentate nuclei, and olivary nuclei. "Axonal bodys" were encountered in Gollis nuclei, and the pigment granules in the reticular zone of substantia nigra increased in quantity.

　Clinical and pathologic characteristics of "presenil-involutive subacute myoclonic-dyskinetic encephalopathies" centering Creutzfeldt-Jakob diseases were discussed.