Neurology Department, National Institute of Neurology, Tunis, Tunisia.
Neurol Sci. 2011 Aug;32(4):727-9. doi: 10.1007/s10072-011-0653-x. Epub 2011 Jun 17.
Although peripheral neuropathy is a common complication of microscopic angiitis, manifestations involving the muscle and the central nervous system have been rarely reported. We describe a 48-year-old man who rapidly developed a clinical picture of mononeuritis multiplex. A month after the appearance of the primary symptoms, he became comatose and had left hemiplegia in relation with a massive cerebral haematoma. Laboratory data revealed signs of inflammation, glomerular dysfunction with microhaematuria, and positive myeloperoxidase-antineutrophil cytoplasmic antibodies. The neuromuscular biopsy disclosed a small-vessel vasculitis, consisting with microscopic angiitis, associated with myositis and extensive axonal loss. The patient had surgical evacuation of the haematoma and received immunosuppressive therapy with good outcome. Thus, microscopic angiitis should be considered as a differential diagnosis in cases of myositis and intracerebral haemorrhage.
尽管周围神经病是显微镜下血管炎的常见并发症,但涉及肌肉和中枢神经系统的表现很少有报道。我们描述了一名 48 岁男性,他迅速出现多发性单神经炎的临床表现。原发性症状出现一个月后,他昏迷不醒,并因大量脑出血导致左侧偏瘫。实验室数据显示炎症迹象、肾小球功能障碍伴镜下血尿和髓过氧化物酶-抗中性粒细胞胞质抗体阳性。神经肌肉活检显示小血管血管炎,符合显微镜下血管炎,伴有肌炎和广泛轴索丧失。患者接受了血肿清除术,并接受了免疫抑制治疗,结果良好。因此,在出现肌炎和脑出血的情况下,应考虑显微镜下血管炎作为鉴别诊断。