Meinck H-M, Balint B
Neurologische Klinik, Universitätsklinikum Heidelberg, Im Neuenheimer Feld 400, 69120, Heidelberg, Deutschland.
UCL Institute of Neurology, Sobell Department of Motor Neuroscience and Movement Disorders, National Hospital of Neurology and Neurosurgery, Queen Square, London, UK.
Nervenarzt. 2018 Feb;89(2):207-218. doi: 10.1007/s00115-017-0480-2.
The identification of new variants of the stiff man syndrome (SMS) and of new, probably pathogenic neuronal autoantibodies has led to the concept of stiff man (or person) spectrum disorders (SPSD). This is an expanding group of rare chronic autoimmune inflammatory diseases of the central nervous system (CNS) that have in common the main symptoms of fluctuating rigidity and spasms with pronounced stimulus sensitivity. These core symptoms are mandatory and can be accompanied by a wide variety of other neurological signs. The SPSDs are associated with autoantibodies directed against neuronal proteins that attenuate excitability. Neither clinical phenotypes nor the course of SPSD correlate closely with the antibody status. The treatment of these diseases aims at maintaining mobility and is pragmatically oriented to the degree of impediment and comprises antispastic, anticonvulsant and immunomodulating or immunosuppressive medication strategies.
僵人综合征（SMS）新变体以及新的、可能具有致病性的神经元自身抗体的鉴定，催生了僵人（或僵人谱系）谱系障碍（SPSD）这一概念。这是一组不断扩大的罕见慢性中枢神经系统（CNS）自身免疫性炎症性疾病，其共同的主要症状是波动性僵硬和痉挛，并伴有明显的刺激敏感性。这些核心症状是必备的，还可能伴有各种各样的其他神经体征。SPSD与针对减弱兴奋性的神经元蛋白的自身抗体相关。SPSD的临床表型和病程均与抗体状态没有密切关联。这些疾病的治疗旨在维持活动能力，实际操作中以障碍程度为导向，包括抗痉挛、抗惊厥以及免疫调节或免疫抑制药物治疗策略。
