Stiff-Man Syndrome and Variants
Stiff-Man Syndrome and Variants
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DOI：
10.1001/archneurol.2011.991
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发表时间：
2012-02-01
影响因子：
--
通讯作者：
Pittock, Sean J.
中科院分区：
文献类型：
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作者：
McKeon, Andrew;Robinson, Maisha T.;Pittock, Sean J.
背景：关于僵硬人综合征（SMS）（经典形式或其变体）的发病率或长期治疗反应和结果的信息很少。目的：综合描述一组SMS患者的特征。设计：观察性研究。地点：明尼苏达州罗切斯特市梅奥诊所。患者：99例经典SMS与疾病变体患者，谷氨酸脱羧酶65kd异构体（GAD65）抗体血清阳性和血清阴性。主要结局指标：神经学、自身免疫、血清学和肿瘤学结果；治疗;以及1984年1月至2008年12月的结果。结果：中位随访时间为5年（范围0-23年）。79例患者（典型SMS 59例，部分SMS 19例，进行性脑脊髓炎伴强直和肌阵挛[PERM] 1例）GAD65抗体血清阳性。其中67%（79人中的53人）患有至少一种共存的自身免疫性疾病，4%（79人中的3人）患有癌症。典型SMS患者初始评估时的GAD65抗体值（中值为623 nmol/L）显著高于部分SMS患者（中值为163 nmol/L） (P
Background: Little information is available about the incidence of stiff-man syndrome (SMS) (the classic form or its variants) or about long-term treatment responses and outcomes.Objective: To comprehensively describe the characteristics of a cohort of patients with SMS.Design: Observational study.Setting: Mayo Clinic, Rochester, Minnesota.Patients: Ninety-nine patients with classic SMS vs variants of the disorder, both glutamic acid decarboxylase 65 kD isoform (GAD65) antibody seropositive and seronegative.Main Outcome Measures: Neurological, autoimmune, serological, and oncological findings; treatments; and outcomes between January 1984 and December 2008.Results: The median follow-up duration was 5 years (range, 0-23 years). Seventy-nine patients (59 having classic SMS, 19 having partial SMS, and 1 having progressive encephalomyelitis with rigidity and myoclonus [PERM]) were GAD65 antibody seropositive. Sixty-seven percent (53 of 79) of them had at least 1 coexisting autoimmune disease, and 4% (3 of 79) had cancer. GAD65 antibody values at initial evaluation were significantly higher among patients with classic SMS (median value, 623 nmol/L) than among patients with partial SMS (median value, 163 nmol/L) (P