Barontini F, Cappelli L, Marconi G
Riv Patol Nerv Ment. 1978 Jul-Aug;99(4):195-206.
A case of Progressive Supranuclear Palsy (P.S.P.) of early onset (38 years) is described, with somewhat atypical features: the patient was oligophrenic, had no pseudobulbar signs, but there was some muscular atrophy, particularly of the shoulders girdle. On examination there was a slight cortical and cerebellar atrophy on the air encephalogram. Electromyography showed signs of damage to the lower motor neurone which was confirmed by muscular biopsy. In the literature a muscular atrophy in association with P.S.P. is rarely reported clinically although in some cases studied histologically there was degeneration in the nuclei of the cranial and spinal motor nerves. The present case may be an example of such widespread degeneration.
本文描述了一例早发型（38岁）进行性核上性麻痹（P.S.P.）病例，具有一些非典型特征：患者智力发育迟缓，无假性球麻痹体征，但存在一定程度的肌肉萎缩，尤其是肩胛带肌萎缩。气脑造影检查显示轻度皮质和小脑萎缩。肌电图显示下运动神经元受损迹象，肌肉活检证实了这一点。在文献中，虽然在一些组织学研究的病例中发现颅神经和脊神经核有变性，但临床上很少报道与进行性核上性麻痹相关的肌肉萎缩。本病例可能是这种广泛变性的一个例子。
