Creutzfeldt-Jakob病の1症例

A CASE OF CREUTZFELDT-JAKOB'S DISEASE

I．緒言

　Creutzfeldt-Jakob病（C-J病と略す）は急速に進行する痴呆を中核とした精神症状と，錐体路症状，錐体外路症状，下位運動ニューロン障害など多彩な神経症状を示しつつ，数ヵ月〜1年前後という急速な経過で死亡する疾患である。本病の原因，本態はなお不明であるが最近，種々の新しい方法を用いた研究がなされるようになつた。すなわちFriedeら（1964）による組織化学的研児，Gonatasら（1965）による電子顕徽鏡的研究，Suzukiら（1966）による脳の化学的研究などである。しかしこれらの努力にもかかわらず本病の原因はなお明らかにされていない。一方，本病は従来考えられていたほど，きわめてまれな疾患ではないように思われる。Siedler　＆Malamud^1）によると，1963年までの欧米の本病およびその近縁疾患の報告例は104例であり，わが国の本病報告例数は1961年の王丸ら^2）の報告以来例をかぞえる。最近，われわれも臨床的に本病と診断することができ，病理学的にも定型的なC-J病に属することを確かめ得た症例を経験した。C-J病の本態の解明にはなお症例の集積を必要とすると思われ，そのためには臨床診断の能力をいつそう高めなければならない。以下にわれわれの経験した症例の臨床と病理を報告し，本病の臨床診断について考察したいと思う。

This paper presents the clinical and pathological findings of 51-year-old housewife, who died 10 months after the onset of illness, which was a weakness of lower extremities and a difficulty in walking. The subacute illness characterized clinically by. a progressive mental deterioration with a hal-luzination and an illusion, muscular rigidity, tremor, atrophy with fasciculation in all extremities, gen-eralized hyperactive deep reflexes and positive Ba-binski's sign. At the latter stage she went into a state of akinetic mutism.

The electroencephalopram was characterized by a periodic appearance of generalized bursts of high voltage slow waves, and the pneumoencephalogram showed the slight dilatation of the third and the anterior part of lateral ventricles, and the dilatation of sulci in frontal lobes.

On autopsy, the brain weighed 1120 g., there was slight atrophy of the cerebral convolutions, from the frontal to the parietal regions on gross inspection. Histological examination revealed the widespread degeneration of nerve cells of verying intensity throughout the gray matter of the central nervous system. In the cerebral cortex, it was marked in frontal, parietal and temporal lobes and diminished in occipital lobes. The neuronal changes included the shrinkage, sclerosis, edematous change, axonal reaction and a loss of neurons. A widespread astrocytic proliferation and hypertrophy were noted. The neuronal and glial changes in similar nature were seen in the basal ganglia, thalamus, pons, medulla oblongata and spinal cord. Cerebellum wasnot affected. The axonal reaction in cell body was also seen in substantia nigra, ambiguus nuclei, hy-poglossal nuclei and anterior horn cells in spinal cord as well as in Betz's cell in precentral gyrus. Neuronophagia and glia rosetts were very poor.

The reported case is classified in the typical Creutzfeldt-Jakob's disease clinically and pathologi-cally, and is the twelfth case to be reported in the literature in Japan. The clinical diagnosis of Creutzfeldt-Jakob's disease and the differential dia-gnosis to subacute spongiform encephalopathy were discussed.