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甲基强的松龙治疗HIV相关杆状肌病的临床、组织学及放射学反应

Clinical, histological and radiological responses to methylprednisolone in HIV-associated rod myopathy.

作者信息

Silva André M S, Mendonça Rodrigo H, Moreno Cristiane A M, Estephan Eduardo P, Helito Paulo V P, Carvalho Mary S, Zanoteli Edmar

机构信息

Department of Neurology, Medical School of the University of São Paulo, São Paulo, Brazil.

Department of Radiology, Medical School of the University of São Paulo, São Paulo, Brazil.

出版信息

Neuromuscul Disord. 2017 Aug;27(8):756-759. doi: 10.1016/j.nmd.2017.05.008. Epub 2017 May 12.

Abstract

Skeletal muscle involvement as a neurologic manifestation in individuals with HIV is rare, especially as rod myopathy. We describe a 41-year-old male with HIV infection who presented progressive proximal muscle weakness and limb-girdle atrophy. A muscle magnetic resonance image showed bilateral fatty infiltration and post-contrast enhancement in the arm and thigh muscles. The muscle biopsy revealed intracytoplasmic aggregates with appearance of nemaline rod bodies with Gomori trichrome staining and electron microscopy in most fibers. The patient underwent six cycles of intravenous methylprednisolone pulses, presenting clinical improvement. Post-treatment muscle biopsy showed fewer nemaline bodies and muscle magnetic resonance image depicted a pronounced reduction of muscular edema. These findings corroborate that deposition of nemaline bodies in these patients might be related to an immune response triggered by the virus.

摘要

骨骼肌受累作为HIV感染者的一种神经表现较为罕见,尤其是作为杆状体肌病。我们描述了一名41岁的HIV感染男性,他出现进行性近端肌无力和四肢带肌萎缩。肌肉磁共振成像显示双侧手臂和大腿肌肉脂肪浸润及造影剂后强化。肌肉活检显示,在大多数纤维中,经Gomori三色染色和电子显微镜检查可见胞质内聚集物,外观呈棒状体。该患者接受了六个周期的静脉注射甲泼尼龙冲击治疗,临床症状有所改善。治疗后的肌肉活检显示棒状体减少,肌肉磁共振成像显示肌肉水肿明显减轻。这些发现证实,这些患者中棒状体的沉积可能与病毒引发的免疫反应有关。

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