Martínez-Yélamos A, Vidaller A, Mitjavila F, Pujol R, Jato M, Rubio F
Servicio de Neurología, CSU de Bellvitge, L'Hospitalet de LI, Barcelona, España.
Rev Neurol. 1998 Aug;27(156):223-5.
Behçet disease is a systemic form of vasculitis which presents with neurological symptoms with a frequency varying between 16 and 40%. Involvement of the parenchyma has been found to worsen the prognosis in patients with neuroBehçet (NB).
To review the clinical features and course of patients with NB involving the parenchyma of the central nervous system (CNS).
Seven patients with Behçet disease and neurological localizing signs were seen in our hospital between 1989 and 1996. The initial diagnosis was of ischemic ictus in five of the seven patients. Both neuroimaging studies and investigation of the cerebrospinal fluid were always pathological in all cases. Vascular studies (arteriography and echo-Doppler of the supra-aortic trunks) were normal. One patient died. Four patients had serious sequelae following treatment.
NB should be included in the different diagnosis of ictus. Involvement of the parenchyma of the CNS was accompanied by lymphocytic meningitis, perhaps also leading to a worse functional prognosis.
白塞病是一种系统性血管炎,出现神经症状的频率在16%至40%之间。已发现脑实质受累会使神经白塞病(NB)患者的预后恶化。
回顾累及中枢神经系统(CNS)脑实质的NB患者的临床特征及病程。
1989年至1996年间,我院诊治了7例患有白塞病且有神经定位体征的患者。7例患者中有5例最初诊断为缺血性卒中。所有病例的神经影像学检查和脑脊液检查结果均呈病理性。血管检查(主动脉弓上干动脉造影和超声多普勒检查)均正常。1例患者死亡。4例患者治疗后有严重后遗症。
NB应纳入卒中的鉴别诊断。CNS脑实质受累伴有淋巴细胞性脑膜炎,这可能也会导致更差的功能预后。