Department of Neurology, Charles Nicolle Hospital, Tunis, Tunisia.
Clin Rheumatol. 2012 Apr;31(4):733-8. doi: 10.1007/s10067-011-1923-y. Epub 2012 Jan 11.
Neurological involvement may be seen in 5-30% of the patients with Behcet's disease (BD). Occasionally, parenchymal neurological involvement in BD can present as a spinal cord syndrome. However, motor neuron disease-like presentation is extremely uncommon. Here we are reporting five patients (all male; median age, 38) fulfilling both International Study Group criteria for BD and El Escorial criteria for amyotrophic lateral sclerosis (ALS). These patients were identified by a questionnaire sent to the members of the Neuro-Behcet Study Group of the International Study Group for BD. Three out of five patients had only motor presentations. In two patients, sensory and urinary manifestations were present as well. Spinal cord MRIs were normal in all, and brain MRIs were normal in four patients; one patient had nonspecific white matter changes. Two patients passed away 1-3 years after diagnosis of ALS, and two patients were lost to follow-up 3 and 11 years after admission; one patient is still alive 3 years after onset. The patients that are presented here might represent a rare form of neurological involvement in BD as well as sole coincidence. Larger prospective series are needed to further answer this issue.
神经系统受累可发生在 5-30%的白塞病（BD）患者中。偶尔，BD 的实质神经系统受累可表现为脊髓综合征。然而，运动神经元病样表现极为罕见。我们在此报告了 5 名患者（均为男性；中位年龄 38 岁），均符合 BD 的国际研究组标准和肌萎缩侧索硬化症（ALS）的埃尔埃斯克里尔标准。这些患者是通过向国际 BD 研究组神经白塞研究小组成员发送问卷确定的。5 名患者中有 3 名仅表现为运动障碍。2 名患者还存在感觉和泌尿系统表现。所有患者的脊髓 MRI 均正常，4 名患者的脑部 MRI 正常；1 名患者有非特异性脑白质改变。2 名患者在诊断为 ALS 后 1-3 年内死亡，2 名患者在入院后 3 年和 11 年后失访；1 名患者在发病后 3 年内仍存活。这里介绍的患者可能代表了 BD 中一种罕见的神经系统受累形式，也可能只是巧合。需要更大规模的前瞻性研究来进一步解答这个问题。
