Paisansinsup Tawatchai, Manno Edward M, Moder Kevin G
Internal Medicine/Rheumatology, Park Nicollet Medical Center, St. Louis Park, Minnesota, USA.
J Clin Rheumatol. 2004 Oct;10(5):265-8. doi: 10.1097/01.rhu.0000141508.17269.8e.
Primary angiitis of the central nervous system (PACNS) is a form of vasculitis affecting the central nervous system (CNS) that is not associated with systemic disease or vasculitis outside the CNS. It is a rare disease with significant morbidity and mortality. Delay in diagnosis and treatment is common due to its nonspecific clinical symptoms and lack of efficient noninvasive diagnostic tests. The finding of vasculitis in the leptomeningeal and cortical biopsy sample has remained the gold standard for making the diagnosis of PACNS. We describe a patient whose clinical symptoms of cauda equina syndrome led to a nerve root biopsy that was consistent with PACNS despite prior extensive investigations including a brain biopsy. This report highlights the cauda equina syndrome as a clinical presentation of PACNS and emphasizes the need for awareness of this rare condition.
中枢神经系统原发性血管炎(PACNS)是一种影响中枢神经系统(CNS)的血管炎形式,与全身性疾病或CNS以外的血管炎无关。它是一种罕见疾病,具有较高的发病率和死亡率。由于其非特异性临床症状以及缺乏有效的非侵入性诊断测试,诊断和治疗延迟很常见。在软脑膜和皮质活检样本中发现血管炎仍然是诊断PACNS的金标准。我们描述了一名患者,其马尾综合征的临床症状导致进行神经根活检,尽管之前进行了包括脑活检在内的广泛检查,但该活检结果与PACNS一致。本报告强调了马尾综合征作为PACNS的一种临床表现,并强调了认识这种罕见疾病的必要性。