Daroff R B
Yale J Biol Med. 1987 Mar-Apr;60(2):119-22.
Progressive supranuclear palsy (PSP) was originally described in 1964. Although some contended it was merely a variant of Parkinson's disease, a specific electron microscopic finding of straight, rather than twisted, filaments in the neurofibrillary tangles established PSP as a distinct entity. The almost pathognomonic early clinical finding of paralysis of downward gaze is due to lesions involving the lateral portions of the rostral interstitial nucleus of the medial longitudinal fasciculus. Recent neurochemical studies have identified both a decrease in central dopamine and acetylcholine. The etiology of PSP is unknown, and the therapy is generally ineffective.
进行性核上性麻痹(PSP)最初于1964年被描述。尽管有些人认为它仅仅是帕金森病的一种变体,但神经原纤维缠结中直的而非扭曲的细丝这一特定电子显微镜发现确立了PSP为一种独特的疾病实体。早期几乎具有诊断意义的向下凝视麻痹的临床发现是由于涉及内侧纵束嘴侧间质核外侧部分的病变所致。最近的神经化学研究发现中枢多巴胺和乙酰胆碱均减少。PSP的病因尚不清楚,治疗通常无效。