Department of Anesthesiology, Tri-Service General Hospital, National Defense Medical Center, Taipei, Taiwan.
Department of Pathology, Tri-Service General Hospital, National Defense Medical Center, Taipei, Taiwan.
Acta Neurol Taiwan. 2023 Dec 30;32(4):212-217.
Anti-signal recognition particle (SRP) myopathy is a subtype of immune-mediated necrotizing myopathy. It rarely presents with extramuscular features, involving the skin, lung, and heart. This paper presents a case of anti-SRP myopathy associated with sensorimotor polyneuropathy.
A 33-year-old woman with no history of systemic disease presented to our hospital with weakness and numbness of the lower limbs for 1 year. Electromyography and nerve conduction study (NCS) revealed combined myopathy and axonal sensorimotor polyneuropathy. Blood examination revealed increased levels of serum muscle enzymes and anti-SRP antibodies. T1-weighted magnetic resonance imaging revealed diffuse muscular hyperintensities in the thighs, indicative of fatty replacement. She was administered methylprednisolone pulse therapy, followed by oral prednisolone and azathioprine. Muscle power increased, and serum muscle enzyme levels decreased significantly. Subsequent NCS performed 2 years later revealed persistent axonal degeneration in the lower limbs.
Anti-SRP myopathy can present with sensorimotor polyneuropathy. Thus, the possibility that the same pathological process affected the skeletal muscles and peripheral nerves should be considered.
抗信号识别颗粒（SRP）肌病是免疫介导的坏死性肌病的一个亚型。它很少出现肌肉外特征，涉及皮肤、肺和心脏。本文报告了一例抗 SRP 肌病合并感觉运动性多发性神经病。
一名 33 岁女性，无系统性疾病史，因下肢无力和麻木 1 年来我院就诊。肌电图和神经传导研究（NCS）显示合并肌病和轴索性感觉运动性多发性神经病。血液检查显示血清肌肉酶和抗 SRP 抗体水平升高。T1 加权磁共振成像显示大腿弥漫性肌肉高信号，提示脂肪替代。给予甲泼尼龙脉冲治疗，随后口服泼尼松龙和硫唑嘌呤。肌肉力量增加，血清肌肉酶水平显著下降。2 年后进行的后续 NCS 显示下肢仍存在持续性轴索性变性。
抗 SRP 肌病可出现感觉运动性多发性神经病。因此，应考虑同一病理过程影响骨骼肌和周围神经的可能性。
