Inagaki T, Ishino H, Iijima M, Seno H, Kikumoto O, Yoshinaga J, Matsuura H, Tachiyama Y
Department of Psychiatry, Shimane Medical University, Izumo, Japan.
No To Shinkei. 1996 Nov;48(11):1037-45.
An autopsy case of progressive supranuclear palsy (PSP) with degeneration of the fronto-pontine tracts of the midbrain and pons, and without grumose degeneration of the dentate nucleus is reported. A 72-year-old woman was suffering from dysarthria and gait disturbance. Moderate dementia was noted and gradually worsened. Pyramidal and extrapyramidal signs and cerebellar ataxia were not observed. Eye movements were fully preserved. Brain CT showed cerebellar atrophy. Three years later, she was unable to stand or move, and became mutistic. At the age of 75, she died suddenly. The duration of her illness was approximately 4 years. Clinical diagnosis was LCCA (late cortical cerebellar atrophy). Neuropathological examination revealed gliosis of the deep layers of the cerebral cortex around the precentral gyrus, fronto-pontine tracts degeneration (posterior part of the anterior crus, genu and anterior part of the posterior crus of the internal capsule, cerebral peduncles of the midbrain, pontine base and pyramis of the medulla oblongata). Also, atrophy of the pons and marked degeneration of the superior colliculi and substantia nigra were observed. Neurofibrillary tangles (NFTs) and glial fibrillary tangles (GFTs) were found in the subcortical nuclei. These findings were almost consistent with PSP. However, the following differed from those of previously reported typical PSP cases: firstly, mild gliosis in the reticular formation of the midbrain; secondly, few NFTs in the pontine nuclei and superior colliculi and; thirdly, no grumose degeneration in the dentate nucleus. In addition, clinical symptoms of the present case are not consistent with PSP. Therefore, we concluded this case to be an atypical PSP both clinically and neuropathologically.
报告一例进行性核上性麻痹（PSP）的尸检病例，该病例中脑和脑桥的额桥束发生变性，齿状核无颗粒状变性。一名72岁女性患有构音障碍和步态障碍。存在中度痴呆且逐渐加重。未观察到锥体束和锥体外系体征以及小脑共济失调。眼球运动完全保留。脑部CT显示小脑萎缩。三年后，她无法站立或活动，变得缄默。75岁时，她突然死亡。病程约4年。临床诊断为LCCA（晚期皮质小脑萎缩）。神经病理学检查发现中央前回周围大脑皮质深层胶质增生，额桥束变性（内囊前肢后部、膝部和后肢前部、中脑大脑脚、脑桥基底部和延髓锥体）。此外，观察到脑桥萎缩以及上丘和黑质明显变性。在皮质下核中发现神经原纤维缠结（NFTs）和胶质纤维缠结（GFTs）。这些发现与PSP基本一致。然而，以下方面与先前报道的典型PSP病例不同：首先，中脑网状结构轻度胶质增生；其次，脑桥核和上丘中NFTs较少；第三，齿状核无颗粒状变性。此外，本病例的临床症状与PSP不符。因此，我们得出结论，该病例在临床和神经病理学上均为非典型PSP。
