Progressive Supranuclear Palsyについて

PROGRESSIVE SUPRANUCLEAR PALSY:A REVIEW OF THE LITERATURE WITH PRESENTATION OF ONE CASE

I．緒言

　私たちは最近progressive supranuclear palsy （以下PSPと略）の1剖検例を経験した^19）。この症例では，動眼神経核と脊髄前角にグリオーゼを認めたことが従来の報告例にくらべて特異であつたが，その臨床像を要約すると，死亡時71歳の男子で，69歳に発病してよく転倒するようになつた。反射性よりも随意性運動に対する垂直注視麻輝（「人形の目」試験陰性），輻輳麻痺，言語緩徐，嚥下困難，筋強剛，感情失禁，眼輪筋反射，下顎反射，四肢腱反射の亢進，バビンスキー反射，痴呆，夜間のせん妄状態を呈し，1年3カ月の経過で死亡した。

　病理像を要約すると，脳重1,210g，前頭葉極に萎縮，側脳室前角，第3脳室の中等度の拡大がある。病変の主座は両側性に，淡蒼球（第2,3図），ルイ氏体（第2，3図），蓋板上丘（第8図），赤核，黒核（第4,5図），青斑核，中脳網様体（第4,5図），中心灰白質，動眼神経核（第4,5図），オリーブ核，歯状核（第7図）とそのHilus （第6図），後索核，三叉神経脊髄路核，脊髄前角にあり，神経細胞の脱落，脱落，グリオーゼをみた。またアルッハイマー神経原線維変化を淡蒼球，ルイ氏体（策10図），中脳網様体，黒核，青斑核，赤核，橋（第9図），オリーブ核に認めた（第1図）。

1) The literature on progressive supranuclear palsy was reviewed with presentation of one case.

2) The total number of published autopsy cases of progressive supranuclear palsy with vertical gaze palsies amounts to 25. (Chavany et al, Steele et al, David et al, Cambier et al, Behrman et al, Blumenthal et al, Rizzuto et al, Jellinger, Steele, Man'nen et al, and Ishino et al)

3) The cases without gaze palsy are reported by Brusa, Verhaart, Weinmann, Anzil, and Neumann.

4) Symptoms of these 25 cases started in the sixth (17cases) and seventh (7cases) decade. There was a steady progressive course leading to death in 3 to 7 years in about 70% of the 25 cases. The shortest course was 1 year and 3 months, while the longest 12 years. As to loss of conjugate vertical gaze, paralysis of upward gaze was fre-quently observed and, when associated with loss of ocular convergence, formed Parinaud's syndrome. Fifteen cases revealed impairment of ocular conver-gence. At the later stage paralysis of horizontal gaze was observed in 15 cases. Extension and dystonic rigidity of the neck was one of the com-monest symptoms, but was absent in 6 cases. Dysarthria and dysphagia were reported in almost all cases, while emotional incontinence (forced cry-ing and laughing) was recorded in 4 cases. Dementia was seen in almost all cases, but of minor degree. Restlessness, increasing irritability, confusion, and delirious state in the night were also recorded. Deep tendon reflexes were hyperactive in almost all cases, while Babinski's sign was noted in 12 cases. In 17 cases with spinal cord verification, only 3 cases showed slight to moderate degeneration of the pyramidal tracts.

5) The pathological findings of the cases without paralysis of conjugate gaze had a striking re-sembrance to those reported by Steele et al.