両側脳室周囲，特に尾状核脳梁角（Wetterwinkel）部に連続性の嚢胞性変化を認めた多発性硬化症の1例

An Autopsy Case of Multiple Sclerosis with Bilateral Continuous Cystic Lesions along Lateral Ventricles and Caudate-Callosal Angles (Wetterwinkel)

　全経過約6年で死亡した多発性硬化症の53歳女性の剖検所見を報告した。新旧の脱髄巣が大脳皮質下白質，脳幹，視神経，脊髄に散在した以外に，本例では，広汎な連続性の嚢胞性病巣が，尾状核脳梁角（Wetterwinkel）を含めた両側脳室に接してほぼ対称性に認められ，稀な症例と思われた。このように広汎な嚢胞性病巣がこの部位に出現した機序として急性増悪の繰り返しにより発生した局所の浮腫，循環障害や血液脳関門の破壊による血液成分の脳実質内への侵入によって壊死組織が長い経過中に倉食細胞などにより吸収，清掃され生じたものと考えた。また副腎皮質ステロイドなどによる免疫抑制も1つの要因となったものと考えられた。

A 47-year old female had a fever about 39°C of unknown origin for 2 days. Soon she developed pain in the bilateral lower extremities followed by gait disturbance and vesicorectal disorder. Prednisolone was administered with an improvement. However, she developed paresthesia in the upper extremities 1 month later, and then gradually paraplegia another 5 month later. Nystagmus, painful tonic spasm, facial spasm, and visual disorder also appeared. These symptoms repeatedly exacerbated and remit-ted with administration of prednisolone.

We examined this patient at age 53. CBC, blood chemistry, urinalysis, ECG and chest X-ray were normal. Serum IgG and IgA level were decreased. CSF protein content and IgG level were remarkablyincreased. EEG showed diffuse 0 activities. MRI studies revealed high intensity signals in the putamen, deep frontal and periventricular white matter region.

Pulse therapy of methylprednisolone was perfor-med effectively for several times. She died of respi-ratory and heart failure 6 years after the onset. Autopsy revealed bilateral continuous cystic lesions along the lateral ventricles extending from the fron-tal tips of anterior horns to the occipital tips of posterior, and further, to the temporal tips of lateral horns ; the caudate-callosal angeles (Wetterwin-kel) were more severely and widely affected bilater-ally. There were also old and fresh demyelinated lesions scattered in the cerebral white matter, brain-stem, cerebellum, and spinal cord. Although this case is considered to have typical MS from clinical and pathological findings, there have been only a few reports of MS with such continuous cystic lesions in the cerebral hemispheres as seen in this case.