Brown P, Marsden C D
National Hospital for Neurology and Neurosurgery, and the MRC Human Movement and Balance Unit, Institute of Neurology, London WC1N 3BG, UK.
J Neurol. 1999 Aug;246(8):648-52. doi: 10.1007/s004150050425.
Rigidity in the setting of continuous motor unit activity at rest can be caused by a variety of central and peripheral conditions. A central origin is suggested by the presence of painful reflex spasms. Focal spinal lesions and infective causes are relatively easily excluded through imaging, microbiological and serological studies. There then remain a group of patients who may have the classical 'stiff-man syndrome' or a related syndrome. When strict diagnostic criteria are used, patients with the stiff man syndrome uniformly have axial rigidity, and about 90% are found to have antibodies against glutamic acid decarboxylase. Treatment response and prognosis are excellent. Stiff persons with 'plus' signs, particularly those with rigidity of a distal limb, are unlikely to have the classical stiff man syndrome. They have a poorer treatment response and prognosis. Some have a paraneoplastic aetiology, while a non-malignant autoimmune basis seems likely in others. Those in whom post-mortem pathology findings are available usually are seen to have had an encephalomyelitis with prominent involvement of the grey matter. Clinically, stiff persons with 'plus' signs may be divided into three groups according to the aggressiveness of the pathology and its relative distribution. Encephalomyelitis with rigidity follows a relentless subacute course, leading to death within 3 years. Chronic cases may present with predominantly brainstem involvement, including generalised myoclonus (the 'jerking stiff person syndrome') or spinal cord involvement, dominated by stiffness and spasm in one or more limbs (the 'stiff limb syndrome').
静息时持续运动单位活动情况下的僵硬可由多种中枢和外周疾病引起。疼痛性反射痉挛提示中枢起源。通过影像学、微生物学和血清学研究相对容易排除局灶性脊髓病变和感染性病因。然后剩下一组可能患有典型“僵人综合征”或相关综合征的患者。使用严格的诊断标准时，僵人综合征患者均有轴性僵硬，约90%被发现有抗谷氨酸脱羧酶抗体。治疗反应和预后良好。有“附加”体征的僵硬患者，尤其是远端肢体僵硬的患者，不太可能患有典型的僵人综合征。他们的治疗反应和预后较差。一些患者有副肿瘤病因，而另一些患者似乎有非恶性自身免疫基础。那些有尸检病理结果的患者通常可见有脑脊髓炎，灰质受累明显。临床上，有“附加”体征的僵硬患者可根据病理的侵袭性及其相对分布分为三组。伴有僵硬的脑脊髓炎呈持续的亚急性病程，3年内导致死亡。慢性病例可能主要表现为脑干受累，包括全身性肌阵挛（“抽搐性僵人综合征”）或脊髓受累，以一个或多个肢体的僵硬和痉挛为主（“僵肢综合征”）。
