Progressive supranuclear palsyの2症例—特にその臨床症状と気脳断層写所見について

PROGRESSIVE SUPRANUCLEAR PALSY: REPORT OF TWO CASES, WITH SPECIAL REFERENCE TO ITS CLINICAL MANIFESTATIONS AND PNEUMOENCEPHALO-TOMOGRAPHIC FINDINGS

I．はじめに

　Progressive supranuclear paisy （以下PSPと略す）は1964年Steele,Richardson＆Olszewski²³）により報告されて以来，その特徴的な臨床症状と病理所見より独立疾患単位として認められ，以後，外国では多数の症例が報告されており，本邦においてもその臨床例^{8,12,13,19,25）}や剖検例^{9,15,16,17）}が相次いで発表されている。

　本疾患の診断には臨床所見以外には有効な補助診断法はないとされていたが，1974年にBentson＆Keesey^2）は6例のPSP全例において気脳断層写により，上丘および中脳被蓋の萎縮像を確認し，その診断的価値につき論じた。

Two cases of progressive supranuclear palsy werereported with special reference to their clinicamanifestations and pneumoencephalo-tomographicfindings.

Case 1. A 68 year old woman was found to havehyperextended neck posture approximately one yearprior to admission, followed gradually by difficultyin speaking and swallowing. Neurological exami-nation revealed expressionless face, hyperextendedneck with marked rigidity, prominent dementia,slight emotional lability and dysarthria, in additionto right hemiparesis due to old cerebral infarction(Fig. 1). Her extraocular movements were abolishedvertically with absent Bell's phenomenon andslightly preserved oculo-cephalic responses, andwere also limited horizontally (Fig. 2).

Case 2. A 60 year old man was noted somechange in his character about one year prior toadmission. A few months later he started to havedifficulty in lid-opening, slow physical activity andpoor memory, which were gradually getting worse.Neurological examination disclosed expressionlessface, marked blepharospasms, short neck withmarked rigidity, slight dementia and mild rigiditywith cogwheel phenomenon in both upper ex-tremities (Fig. 4A). His extraocular movementswere absent vertically, and moderately limitedhorizontally (Fig. 5A). Bell's phenomenon waspreserved. He was placed on L-DOPA with gradualimprovements of eye movements at the maximumdoses of 3.6gm (Fig. 5B). Thereafter, his neuro-logical status progressively deteriorated to the pointthat he was totally bedridden and almost immobilewith marked extensor rigidity of his neck approxi-mately 4 years after the onset of the illness (Fig.4B).

Pneumoencephalo-tomograms were performed inthese two cases by using Mimer III (40% magni-fication factor) for measuring superior and inferiorcolliculi, midbrain tegmentum and pons afterBentson and Keesey. The results were summarizedin Table 1. The atrophy of superior colliculi andmidbrain tegmentum was evident in both Case 1(Fig. 3) and Case 2 (Fig. 6). These findings werecompatible with pathological evidences seen inpreviously reported cases of progressive supra-nuclear palsy, and proved the importance of pneumo-encephalo-tomography as the premortem diagnostictests as proposed by Bentson and Keesey in 1974.