Foix-Alajouanine病の1例

A Case of Foix-Alajouanine's Disease

I．緒言

　Foix-Alajouanine^6）は，亜急性壊死性脊髄炎（myelitenécrotique subaigue）の2症例を記載し，臨床的には，痙性，のち弛緩性，筋萎縮を伴う対麻痺，初め解離性，後に全知覚障害，髄液の蛋白―細胞解離を示し，亜急性，進行性に経過，一，二年で死亡するが，病理学的には，腰仙部を主とし胸髄に及ぶ，灰白質にいちじるしい壊死傾向をもつ脊髄炎と脊髄内外の血管壁の著明な肥厚を示す内中膜血管炎（endo-mésovascularite）をこの疾患の特徴とした。

　その後，その病因として，感染，中毒，アレルギーなどが想定されてきたが，Foix-Alajouanine自らは，感染説をとりながら，病因的には血管病変を重視し，一次性と考えた。ついでLhermitte^l1）らは，血管病変をこの疾患の本質として，脊髄炎の名称をしりぞけ，かわりにg1iose angéio-hypertrophiqueの名をあげた。Greenfield^7）らも血管病変を原発性とし，以後多くの報告は，血管原発説をとつている。しかし，その発生機序については意見が分かれている。

　In a patient, 49 year-old, male, paresthesia of the gluteal region and weakness of the left foot were firstly noted. Subsequently he developed gradual paraplegia with moderate muscular atrophy, transversal sensory disturbances (L1) and bladder disturbance. Finally sepsis appeared. The total duration of illness was 4 years and 10 months.

　The postmortem examination revealed haeman. gioma racemosum venosum of the spinal ves. sels and necrosis of the spinal cord. Extramedullary haemangioma, from the superior thoracic segment to the sacrar, was associated with marked dilatation and great thickening of the wall, where inflammatory infiltration was not found. Small intramedullary vessels, from the middle thoracic to the asacrar, increased and showed convolution or glomerulus-formation and thickening and hyalinisation of the walls. Medullary changes, in parallel with the changes of the intramedullary vessels, showed rarefaction with demyelinisation and plasmatic infiltrations necrosis, but nerve cells were almost intact. These changes were identical with Foix-Alajouanine's disease.

　The pathogenesis of these disease is discussed. The necrosis of the parenchyma of the spinal cord appeared to be secondary to the vascular lesion, i.e. haemangioma racemosum venosum with degenerated walls.