Ben Ghorbel I, Ibnelhadj Z, Zouari M, Nagi S, Khanfir M, Hentati F, Houman M H
Service de Médecine Interne, Hôpital La Rabta, 1007 Tunis, Tunisie.
Rev Neurol (Paris). 2005 Feb;161(2):218-20. doi: 10.1016/s0035-3787(05)85026-x.
The involvement of the peripheral nervous system in Behçet's disease is very rare.
We report a case of a 47-year-old man with a six-year history of Behçet's disease and a two-year history of peripheral nervous system involvement. This patient presented with paraesthesia and weakness of the upper and lower limbs, diarrhea and erectile dysfunction. The electromyogram showed evidence of an axonal sensorimotor neuropathy and the nerve biopsy showed an axonal neuropathy. Routine blood tests were normal, there was no increase of serum creatine kinase, aspartate aminotransfease or lactate dehydrogenase and no signs of hyperthyroidism. Fibroscopy and colonoscopy showed no signs of entero-Behçet. The patient was treated with prednisone, cyclophosphamide and carbamazepine with an improvement of paraesthesia.
The mechanism of the peripheral neuropathy in Behçet's disease is still unknown, it might be due to vasculitis of the vasa nervorum or to the side effects of colchicine. Our report is particular by the association of sensorimotor and autonomic involvement of peripheral neuropathy in a patient with Behçet's disease.
白塞病累及周围神经系统极为罕见。
我们报告一例47岁男性患者,有六年白塞病病史,周围神经系统受累两年。该患者表现为上下肢感觉异常及无力、腹泻和勃起功能障碍。肌电图显示为轴索性感觉运动神经病,神经活检显示为轴索性神经病。常规血液检查正常,血清肌酸激酶、天冬氨酸转氨酶或乳酸脱氢酶无升高,也无甲状腺功能亢进迹象。纤维胃镜和结肠镜检查未显示肠白塞病迹象。患者接受泼尼松、环磷酰胺和卡马西平治疗后感觉异常有所改善。
白塞病周围神经病变的机制尚不清楚,可能是由于营养血管的血管炎或秋水仙碱的副作用所致。我们的报告特别之处在于,一名白塞病患者的周围神经病变同时伴有感觉运动和自主神经受累。