Imai H
Department of Neurology, Juntendo University School of Medicine.
Rinsho Shinkeigaku. 1993 Dec;33(12):1307-9.
Akinesia, the core symptom of parkinsonism, is a comprehensive term and a disorder characterized by poverty, slowness and also freezing of movement. Parkinson (1817) in his original essay pointed out the festinating gait. Festination is a tendency to speed up in parallel with a loss of normal amplitude of repetitive movement (petit pas, micrographia and inaudible speech). Freezing is a breakdown of repetitive voluntary movement emerging through festination or suddenly. Freezing of gait occurs either at the start (start hesitation), through festination of gait or suddenly when turning or going through the narrow doorway. Narabayashi and the present author reported the first 2 cases with pure akinesia or freezing symptom without rigidity or tremor and unresponsive to L-DOPA therapy as a new condition at the 14th annual meeting of this Society, 1973. Kinésie paradoxal was always accompanied by this type of akinesia. HVA level in the cerebrospinal fluid was not below normal. The author suggested that the main pathological structures of the condition are different from Parkinson's disease. L-threo-DOPS, a synthetic norepinephrine (NE) precursor, had a mild-to-moderate effect on some cases with freezing, and the NE hypothesis for freezing was proposed (Narabayashi). 35 cases with this condition have been known in our clinic to date. Slowly progressive supranuclear ophthalmoplegia appeared later in some patients, several of whom were clinically and two were pathologically diagnosed as progressive supranuclear palsy. The nosological position and responsible lesion sites of this condition are discussed.
运动不能是帕金森综合征的核心症状,是一个综合术语,指的是一种以运动减少、缓慢以及运动冻结为特征的病症。帕金森(1817年)在其最初的论文中指出了慌张步态。慌张步态是指随着重复性运动正常幅度丧失(小步、小写症和言语低微)而加速行走的倾向。运动冻结是通过慌张步态或突然出现的重复性随意运动的中断。步态冻结可发生在起始时(起始犹豫)、通过慌张步态或在转身或穿过狭窄门口时突然出现。Narabayashi和笔者在1973年本学会第14届年会上报告了首例无强直或震颤、对左旋多巴治疗无反应的单纯运动不能或运动冻结症状的病例,作为一种新病症。反常运动常伴有这种类型的运动不能。脑脊液中的高香草酸水平不低于正常。笔者认为该病症的主要病理结构与帕金森病不同。L-苏糖酸多巴胺(L-threo-DOPS),一种合成去甲肾上腺素(NE)前体,对一些运动冻结病例有轻度至中度疗效,并提出了运动冻结的NE假说(Narabayashi)。截至目前,我们诊所已确诊35例该病症患者。一些患者后来出现了缓慢进展的核上性眼肌麻痹,其中几例经临床诊断,两例经病理诊断为进行性核上性麻痹。本文讨论了该病症的疾病分类位置和责任病变部位。