Suzuki Shigeaki, Hayashi Yukiko K, Kuwana Masataka, Tsuburaya Rie, Suzuki Norihiro, Nishino Ichizo
Department of Neurology, Keio University School of Medicine, 35 Shinanomachi, Shinjuku-ku, Tokyo 160-8582, Japan.
Arch Neurol. 2012 Jun;69(6):728-32. doi: 10.1001/archneurol.2011.1728.
To characterize the clinical course of myopathy associated with antibodies to signal recognition particle (SRP), or anti-SRP myopathy.
Case series.
Keio University Hospitals and National Institute of Neuroscience, National Center of Neurology and Psychiatry, Tokyo, Japan.
We reviewed clinical features of 27 patients with anti-SRP myopathy and analyzed disease progression and neurological outcome.
Anti-SRP antibodies in serum were detected by RNA immunoprecipitation assay using extracts of K562 cells.
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.
A subset of patients with anti-SRP myopathy can show a chronic progressive form associated with severe clinical deficits.
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。
病例系列研究。
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。
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。
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。
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。
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。
