Meinck H M
Sektion Klinische Neurophysiologie, Neurologische Universitätsklinik Heidelberg.
Nervenarzt. 1991 Dec;62(12):762-5.
The discovery of autoimmune processes in the stiff-man syndrome (SMS) not only raises questions concerning the syndrome itself, but may also lead to new insights into pathogenetic principles of neurological disorders. Autoantibodies against GAD, the GABA synthesising enzyme, may become a helpful (though not specific) diagnostic tool, and furthermore may serve as a plausible explanation for both the symptoms of the syndrome and the delayed development of type I diabetes mellitus. However, it remains unexplained why autoimmunity against such widespread inhibitory transmitter systems should induce a syndrome which by definition is confined to only a few symptoms, and for which the majority of neurological signs are regarded as exclusion criteria. It is therefore hypothesised that SMS is part of a broad spectrum of encephalomyelopathies with autoimmunity against GABAergic neurones in common, but with a heterotopic manifestation. Progressive encephalomyelitis with rigidity may be an extreme variant within this spectrum.
僵人综合征（SMS）中自身免疫过程的发现不仅引发了关于该综合征本身的问题，还可能为神经疾病的发病机制原理带来新的见解。针对γ-氨基丁酸（GABA）合成酶谷氨酸脱羧酶（GAD）的自身抗体可能成为一种有用的（尽管不具有特异性）诊断工具，而且还可以为该综合征的症状以及1型糖尿病的延迟发病提供合理的解释。然而，针对如此广泛的抑制性递质系统的自身免疫为何会引发一种从定义上讲仅局限于少数症状且大多数神经体征被视为排除标准的综合征，这一点仍无法解释。因此，有人提出假设，僵人综合征是广泛的脑脊髓病谱的一部分，这些脑脊髓病共同具有针对GABA能神经元的自身免疫，但表现各异。进行性僵硬性脑脊髓炎可能是这一谱中的一个极端变体。
