OVERLAP AXONAL POLYNEUROPATHY WITH IMMUNE MEDIATED NECROTISING MYOPATHY.

Ewe Jin Koh, Ming Lee Chin, Nor Aizan Ab Allah
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Abstract

Immune mediated necrotising myopathy (IMNM) is a rare autoimmune disease of the muscles belonging to the subset of the idiopathic inflammatory myopathies (IIM). This disease entity has classically been associated with myositis specific antibodies. The hallmark feature in clinching the diagnosis of IMNM would be a muscle biopsy showing muscle necrosis and regeneration in the absence of significant inflammatory infiltrates, interpreted in an appropriate clinical context. The term 'neuromyositis' was previously coined in the year 1893 to describe a concomitant polyneuropathy in patients with polymyositis or dermatomyositis. However, a combined polyneuropathy with IMNM has never been reported in previous literature. We describe a case of a 35-year-old gentleman who presented with a 5-day history of symmetrical bilateral lower limb pain and weakness. Despite a negative autoimmune work-up, his muscle biopsy was suggestive of IMNM. A nerve conduction study done had also revealed a superimposed non-length dependant axonal polyneuropathy. The patient had responded well to steroids and is now under remission. This case serves to highlight a rare entity of seronegative IMNM superimposed with an axonal polyneuropathy.

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轴突多发性神经病与免疫介导的坏死性肌病重叠。
免疫介导坏死性肌病(IMNM)是一种罕见的肌肉自身免疫性疾病,属于特发性炎症性肌病(IIM)的子集。这种疾病通常与肌炎特异性抗体有关。确定IMNM诊断的标志特征是肌肉活检显示肌肉坏死和再生,没有明显的炎症浸润,在适当的临床背景下解释。“神经肌炎”一词最初是在1893年创造的,用来描述多发性肌炎或皮肌炎患者的多发性神经病变。然而,在以前的文献中从未报道过多发性神经病变合并IMNM。我们描述了一个35岁的绅士谁提出了一个5天的历史对称双侧下肢疼痛和无力。尽管自身免疫检查呈阴性,但他的肌肉活检提示有IMNM。一项神经传导研究也显示了一种叠加的非长度依赖性轴突多发性神经病。患者对类固醇反应良好,目前病情有所缓解。本病例是一个罕见的血清阴性IMNM合并轴突多发性神经病的病例。
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