Hasegawa Y, Takahashi A
Department of Neurology, Nagoya National Hospital.
Nihon Rinsho. 1992 Apr;50(4):790-8.
Progressive or primary autonomic failure (AF) is a disease of unknown-etiology, and presents generalized and extensive autonomic disturbances because of selective neuronal degeneration in the whole of autonomic nervous system. AF is classified into three categories; (1) pure autonomic failure, without associated neurological disorders, (2) AF with Parkinson's disease (PD), and (3) AF with multiple system atrophy (Shy-Drager syndrome) (Bannister, 1988). AF with PD is pathologically characterized by neuronal cell degeneration in the intermediolateral column and the substantia nigra, together with Lewy bodies mainly in the pigmented nuclei in the brain stem. Patients with PD occasionally develop syncope or dizziness due to orthostatic hypotension and/or postprandial hypotension as well as urorectal disturbances as the initial symptoms, and are followed by parkinsonism. Levodopa is usually effective for parkinsonism but the prognosis is rather poor. AF with PD could be regarded as a form of 'the Lewy body disease' according to Kosaka's clinicopathological entity (1984).
进行性或原发性自主神经功能衰竭（AF）是一种病因不明的疾病，由于整个自主神经系统的选择性神经元变性，表现为全身性和广泛性的自主神经功能紊乱。AF分为三类：（1）单纯自主神经功能衰竭，无相关神经系统疾病；（2）伴有帕金森病（PD）的AF；（3）伴有多系统萎缩（Shy-Drager综合征）的AF（班尼斯特，1988年）。伴有PD的AF的病理特征是中间外侧柱和黑质的神经元细胞变性，同时路易小体主要出现在脑干的色素核中。PD患者偶尔会因体位性低血压和/或餐后低血压以及泌尿直肠功能紊乱而出现晕厥或头晕等初始症状，随后出现帕金森综合征。左旋多巴通常对帕金森综合征有效，但预后相当差。根据小坂的临床病理实体（1984年），伴有PD的AF可被视为“路易体病”的一种形式。
