Takeuchi M, Sasaki S, Ito A, Osawa M, Kobayashi I, Maruyama S
Department of Neurology, Tokyo Women's Medical College, Japan.
No To Shinkei. 1991 Sep;43(9):863-7.
Clinical and pathologic findings of an autopsy case of progressive supranuclear palsy (PSP) with a 7 year clinical course are described. The patient exhibited clinical findings of typical PSP, cerebellar signs and rhythmical myoclonus that was about 2 Hz and synchronous in the eyes, palate, and pharynx, which is so called palatal myoclonus. Pathological findings compatible with those in PSP i.e. loss of nerve cells, neurofibrillary tangles (NFT), and gliosis were found in the globus pallidum, thalamus, subthalamic nucleus, substantia nigra, locus coeruleus, nucleus of Raphe, reticular formation, dentate nucleus, and inferior olives. Nerve cells in the nucleus basalis were preserved. Distinctive findings included marked degeneration of the dentate nucleus, prominent hypertrophy of the inferior olives, and atrophy and subcortical gliosis of the frontal lobe. Hypertrophy of the inferior olives and palatal myoclonus represent an unusual PSP. It is presumed hypoxic injury unmasked the palatal myoclonus in this setting of dentate nucleus and inferior olivary complex degeneration.
描述了一例病程7年的进行性核上性麻痹（PSP）尸检病例的临床和病理发现。患者表现出典型PSP的临床症状、小脑体征以及频率约为2Hz且在眼睛、软腭和咽部同步的节律性肌阵挛，即所谓的腭肌阵挛。在苍白球、丘脑、底丘脑核、黑质、蓝斑、中缝核、网状结构、齿状核和下橄榄核中发现了与PSP相符的病理表现，即神经细胞丢失、神经原纤维缠结（NFT）和胶质增生。基底核中的神经细胞得以保留。独特的发现包括齿状核明显变性、下橄榄核显著肥大以及额叶萎缩和皮质下胶质增生。下橄榄核肥大和腭肌阵挛代表了一种不常见的PSP。据推测，在这种齿状核和下橄榄复合体变性的情况下，缺氧损伤使腭肌阵挛得以显现。
