与信号识别颗粒抗体相关的肌病：疾病进展与神经学转归
Myopathy associated with antibodies to signal recognition particle: disease progression and neurological outcome.
作者信息
Suzuki Shigeaki, Hayashi Yukiko K, Kuwana Masataka, Tsuburaya Rie, Suzuki Norihiro, Nishino Ichizo
机构信息
Department of Neurology, Keio University School of Medicine, 35 Shinanomachi, Shinjuku-ku, Tokyo 160-8582, Japan.
出版信息
Arch Neurol. 2012 Jun;69(6):728-32. doi: 10.1001/archneurol.2011.1728.
OBJECTIVE
To characterize the clinical course of myopathy associated with antibodies to signal recognition particle (SRP), or anti-SRP myopathy.
DESIGN
Case series.
SETTING
Keio University Hospitals and National Institute of Neuroscience, National Center of Neurology and Psychiatry, Tokyo, Japan.
PATIENTS
We reviewed clinical features of 27 patients with anti-SRP myopathy and analyzed disease progression and neurological outcome.
MAIN OUTCOME MEASURES
Anti-SRP antibodies in serum were detected by RNA immunoprecipitation assay using extracts of K562 cells.
RESULTS
Of the 27 patients, 5 (19%) showed chronic progressive muscle weakness as well as atrophy of limbs and trunk muscles from a younger age with more severe neurological outcomes compared with the other 22 patients(81%) with the subacute form.
CONCLUSION
A subset of patients with anti-SRP myopathy can show a chronic progressive form associated with severe clinical deficits.
目的
描述与信号识别颗粒（SRP）抗体相关的肌病，即抗SRP肌病的临床病程。
设计
病例系列研究。
地点
日本东京庆应义塾大学医院及国立神经科学研究所、国立精神神经医疗研究中心。
患者
我们回顾了27例抗SRP肌病患者的临床特征，并分析了疾病进展和神经学转归。
主要观察指标
使用K562细胞提取物通过RNA免疫沉淀试验检测血清中的抗SRP抗体。
结果
27例患者中，5例（19%）表现为慢性进行性肌无力以及四肢和躯干肌肉萎缩，起病年龄较轻，与其他22例（81%）亚急性形式的患者相比，神经学转归更严重。
结论
一部分抗SRP肌病患者可表现为与严重临床缺陷相关的慢性进行性形式。
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