Nowack Rainer, Wachtler Paul, Kunz Jürgen, Rasmussen Niels
Nephrology/Dialysis centre Lindau, Friedrichshafener Str. 82, 88131, Lindau, Germany.
J Neurol. 2009 Mar;256(3):299-304. doi: 10.1007/s00415-009-0121-1. Epub 2009 Mar 1.
The problem of diagnosing vasculitic neuropathy is discussed based on case reports of two patients with Wegener's granulomatosis. One patient developed de novo 6(th) nerve palsy as an isolated relapse manifestation and the second patient a sequence of multiple cranial nerve palsies. Brain imaging with CT and MRI and the laboratory provided no clues suggesting active vasculitis. However, in both patients the neuropathies fully recovered in response to standard induction protocols of vasculitis. In the absence of organ-specific proof of vasculitis, these treatment decisions were guided by the overall clinical presentations. Cranial neuropathy may be the first obvious vasculitic manifestation preceding other organ disease, and since single reliable tests for its diagnosis are lacking, a multidisciplinary approach is advocated here to detect vasculitic manifestations in other organs.
基于两例韦格纳肉芽肿病患者的病例报告，对血管炎性神经病的诊断问题进行了讨论。一例患者新发第6颅神经麻痹，作为孤立的复发表现，另一例患者出现一系列多发性颅神经麻痹。CT和MRI脑部成像以及实验室检查均未提供提示活动性血管炎的线索。然而，在这两例患者中，神经病经血管炎标准诱导方案治疗后均完全恢复。在缺乏血管炎器官特异性证据的情况下，这些治疗决策以整体临床表现为指导。颅神经病变可能是先于其他器官疾病出现的首个明显血管炎表现，由于缺乏用于诊断的单一可靠检测方法，本文提倡采用多学科方法来检测其他器官的血管炎表现。
