Binswanger型脳症の臨床病理学的研究

特に若年発症非高血圧症群の存在について

抄録

In 1894, Binswanger for the first time reported on a group of diseases, which he distinguished from general paralysis of the insane despite similarity in clinical picture, and which he designated as “die Abgrenzung der allgemeinen progressiven Paralyse”. Later, Alzheimer regarded this pathological group as a special type of cerebral arteriosclerosis, and since then the denomination of encephalitis subcorticalis progressiva or Binswanger's disease has become prevalent, and many case reports on it have been published. There remains, however, some doubts as to whether it can be regarded as a perfectly distinct entity.
The authors performed studies on the following questions : 1) Does Binswanger's encephalopathy manifest a definite clinical findings amenable to diagnosis? Especially can it be differrentiated from the general cerebral arteriosclerotic psychosis? 2) Is the vascular lesion in this disease really identical with that of arteriosclerosis? Is there no possibility that it may be a vasculitis? 3) From what vascular lesion does Binswanger's encephalopathy result? In other word, what is the morphopathogenesis of this disease?
To elucidate these questions, 7 cases of Binswanger's disease were classified into 3 groups : Group a includes typical Binswanger's encephalopathy; group b, malignant hypertensive cases; and group c, non-hypertensive cases with onset in younger ages. The results were as follows :
i) Group a can surely be classified as Binswanger's encephalopathy under a special category on the basis of particular clinical symptoms and pathological findings. And this type can be estimated from the clinical symptoms with a considerably high probability.
ii) As to group b, cases of malignant hypertension accompanied by marked neuropsychiatric symptoms sometimes manifest strong subcortical lesion.
iii) The cerebral white matter lesion of Binswanger's encephalopathy is caused by the chronic anoxia and edematous necrosis in it, and they can be explained either as induced by particular damage to intracerebral artery or by extracerebral damage, such as injury to the heart or to the common carotid artery.
iv) As to group c, the special target of the present study, the cerebral parenchymal lesion exhibits that of Binswanger's encephalopathy, whereas the vascular lesion is more of angitis rather than of arteriosclerosis. The authors, however, had strong impression that this angitis, different from the one belonging to the conventional category, may chiefly be confined to the brain. Sourander et al. of Sweden suggested the presence, in a family line, of a new disease, which he designated as hereditary multi-infarct dementia, and which manifests a vascular lesion perfectly similar to that of group c. The authors, placing more emphasis on the cerebral lesion, classifies it under the category of Binswanger's encephalopathy, but they want to regard its vascular lesion not as arteriosclerosis, but as necrotic arteritis contrary to the prevalent classification.
v) It is a problem of today, whether one should regard group c as a new nosological entity as Sourander did. The authors estimated, as an etiologic factor, the presence of some congenital mesenchymal disorder, but its elucidation must await electron microscopic, fluorescent antibody technique and other examinations.