Terao Y, Takeda K, Sakuta M, Nemoto T, Takemura T, Kawai M
Department of Neurology, Japanese Red Cross Medical Center, Tokyo.
Eur Neurol. 1993;33(6):409-15. doi: 10.1159/000116985.
Two cases of pure progressive autonomic failure (PAF) are presented. A postmortem study of one case (case 2) showed a pathology resembling that of Parkinson's disease. Marked cell loss was noted in the substantia nigra, nucleus ceruleus, and intermediolateral column of the spinal cord, while cell loss in the sympathetic ganglion was not remarkable. This case may be an exceptionally rare case of late-onset PAF in which autonomic failure was mainly ascribed to preganglionic (and central) pathology, although autonomic function tests suggested postganglionic sympathetic disorder in both cases.
本文报告了两例纯进行性自主神经功能衰竭（PAF）病例。对其中一例（病例2）进行的尸检研究显示，其病理表现类似于帕金森病。在黑质、蓝斑核和脊髓中间外侧柱观察到明显的细胞丢失，而交感神经节中的细胞丢失并不显著。尽管自主神经功能测试表明两例均存在节后交感神经功能障碍，但该病例可能是一例极为罕见的迟发性PAF，其自主神经功能衰竭主要归因于节前（和中枢）病变。
