Jog M S, Lambert C D, Lang A E
Division of Neurology, St. Michael's Hospital, University of Toronto, Ontario, Canada.
Can J Neurol Sci. 1992 Aug;19(3):383-8.
The stiff-person syndrome is a disorder of persistent, painful muscle contractions predominately affecting the axial musculature. We describe a patient with this disorder and review its pathophysiology. Molecular biologic and immunologic techniques have recently added to the understanding of the mechanism of this disorder. Association with diseases such as diabetes, vitiligo and hypothyroidism have strengthened the auto-immune nature of this syndrome. Auto-antibodies against glutamic acid decarboxylase (GAD), an intraneuronal enzyme, have been implicated in the etiology of this unique disease. Therapeutic intervention with agents such as benzodiazepines that modify central GABAergic activity have demonstrated significant benefit in patients with stiff-person syndrome.
僵人综合征是一种以持续性、疼痛性肌肉收缩为特征的疾病，主要影响轴性肌肉组织。我们描述了一名患有这种疾病的患者，并对其病理生理学进行了综述。分子生物学和免疫学技术最近增进了我们对这种疾病机制的理解。与糖尿病、白癜风和甲状腺功能减退等疾病的关联强化了该综合征的自身免疫性质。针对神经元内酶谷氨酸脱羧酶（GAD）的自身抗体与这种独特疾病的病因有关。使用如苯二氮䓬类等可改变中枢γ-氨基丁酸能活性的药物进行治疗干预，已证明对僵人综合征患者有显著益处。
