Kuriwaka Rika, Kunishige Makoto, Nakahira Haruhito, Inoue Hideo, Higashi Tatsuo, Tokumoto Yoshiaki, Mitsui Takao
Department of Internal Medicine, Anan Kyoei Hospital, Tokushima, Japan.
Clin Rheumatol. 2004 Aug;23(4):364-7. doi: 10.1007/s10067-004-0897-4. Epub 2004 Apr 17.
Neuro-Behçet's disease shows various neuropsychiatric symptoms, but chorea has rarely been reported. We report a case of neuro-Behçet's disease in a 67-year-old woman with depression and chorea that occurred 22 years after the onset of intestinal Behçet's disease. Brain magnetic resonance imaging (MRI) using a fluid-attenuated inversion-recovery (FLAIR) sequence demonstrated lesions more clearly than did T2-weighted MRI. Some of the lesions appeared as small ring-like foci, i.e. low-intensity spots rimmed with remarkable hyperintense signals, in the periventricular white matter and basal ganglia. A review of the literature revealed that the onset of chorea in cases of Behçet's disease varied from the time of onset of Behçet's disease to 31 years after onset of the disease. Psychiatric manifestations have often been associated with neuro-Behçet's disease. In the present patient, treatment with prednisolone resolved the chorea, suggesting that the chorea was caused by an autoimmune mechanism. It seems likely that the long-term development of vasculitis in patients with Behçet's disease results in the formation of these particular brain lesions on FLAIR MR images. Chorea should be taken into consideration as one of the manifestations of Behçet's disease, even many years after remission of the disease.
神经白塞病表现出多种神经精神症状，但舞蹈症鲜有报道。我们报告一例67岁女性神经白塞病患者，在肠道白塞病发病22年后出现抑郁和舞蹈症。使用液体衰减反转恢复（FLAIR）序列的脑磁共振成像（MRI）比T2加权MRI更清晰地显示病变。一些病变在脑室周围白质和基底节区表现为小环状病灶，即低强度斑点周围有明显的高信号。文献回顾显示，白塞病患者舞蹈症的发病时间从白塞病发病时到发病后31年不等。精神症状常与神经白塞病相关。在本例患者中，泼尼松龙治疗使舞蹈症得到缓解，提示舞蹈症是由自身免疫机制引起的。白塞病患者血管炎的长期发展似乎会导致FLAIR MR图像上出现这些特定的脑病变。即使在疾病缓解多年后，舞蹈症也应被视为白塞病的表现之一。
