Progressive Subcortical Gliosisの1剖検例

AN AUTOPSIED CASE OF PROGRESSIVE SUBCORTICAL GLIOSIS

I．はじめに

　1949年Neumann^11）は，それまでにAlzheimer病やPick病として報告されていた症例のなかから神経病理学的に非定型例を整理し，これらの症例をPick病のII型，とした。その後，かかる症例群の神経病理学的所見の特徴から，1967年にはprogressive subcortical glio—sis^12）の概念が提唱された。以来，症例報告が散発的にみられるが，まだ稀な疾患とされ，わが国では数例の報告をみるのみである。本症例群が病理学的にしか診断され得ないことも，症例数が少ない理由の1つと考えられる。いかに臨床的に診断するか，ということも臨床医にとつては目前の課題であるが，本症例群が従来の初老期痴呆群のなかで，1つの疾患単位として存在し得るか否かも大きな課題である，と考える。

　著者らは，progressive subcortical gliosisの定型例と考えられる症例を経験したので，上記の問題点について若干の考察を行ないたい。

A case of a 44-year-old male with progr-essive subcortical gliosis was presented. The clinical manifestations included mental activity, difficulty in attention, euphoria and inability to work. The impairement of memory was not evi-dent and his personality was fairly well preserved. Neurological examination showed no abnormality. The course was progressive without remission and the patient died with pneumonia approximatelyfifteen months after the onset of the disease. La-boratory tests revealed unremarkable except for slight degree of glucosuria and elevated F. B. S.. Electroencephalography and carotid angiography were within normal.

Neuropathological finding revealed that brain weighed 1200g. and moderate degree of atrophy on frontal to parietotemporal lobes was evident. Occipital lobe was not involved. Microscopic exa-mination revealed such changes as fibrillary gliosis in the cerebral white matter and grey matter inthe spinal cord. The lesion in the cerebral white matter was predominent in the frontal lobe. Stri-atum and brain stem also showed mild degree of fibrillary gliosis. No senility related change such as senile plaque, granulovacuolar degeneration and Alzheimer's neurofibrillary tangle was found.

The clinicopathologic findings in this case sup-ported a diagnosis of progressive subcortical gliosis which was first described by Neumann in 1949.