Bogdanova D, Milanov I, Georgiev D
University Hospital for Neurology and Psychiatry, Sofia, III Neurological Clinic, Bulgaria.
Can J Neurol Sci. 1998 Feb;25(1):82-5. doi: 10.1017/s0317167100033552.
The central nervous system is often involved in Behcet's disease. Most common are meningoencephalitic and brain stem syndromes. Although basal ganglia involvement is not an uncommon finding on necropsy, there are only single reports on extrapyramidal syndromes-dyskinesia, chorea and Parkinsonism in patients with Behcet's disease.
We report a patient fulfilling the criteria of the International Study Group for Behcet's disease. He had recurrent oral ulcerations, bilateral posterior uveitis and retinal vasculitis, skin papules and pustules, and recurrent monoarthritis. Neurologic examination revealed pseudobulbar palsy, slight and asymmetric bilateral pyramidal syndrome, muscle rigidity involving the four limbs, bradykinesia, masked face, and impaired postural reflexes. There was postural tremor in the extremities and myoclonic jerks involving the tongue and face muscles. Magnetic resonance imaging demonstrated small bilateral multifocal hyperintense lesions, with right predilection, involving the periventricular white matter, brain stem and basal ganglia.
The Parkinsonian syndrome found in our patient might be due to involvement of both substantia nigra and basal ganglia. This case further emphasizes the wide spectrum of the neurological manifestations of Behcet's disease.
中枢神经系统常受累于白塞病。最常见的是脑膜脑炎和脑干综合征。尽管在尸检中基底节受累并非罕见发现,但关于白塞病患者锥体外系综合征——运动障碍、舞蹈症和帕金森综合征的报道仅有个别病例。
我们报告一例符合白塞病国际研究组标准的患者。他有复发性口腔溃疡、双侧后葡萄膜炎和视网膜血管炎、皮肤丘疹和脓疱以及复发性单关节炎。神经系统检查发现假性延髓麻痹、轻度不对称双侧锥体综合征、四肢肌肉僵硬、运动迟缓、面具脸和姿势反射受损。四肢有姿势性震颤,舌肌和面部肌肉有肌阵挛性抽搐。磁共振成像显示双侧多发小的高强度病灶,右侧更明显,累及脑室周围白质、脑干和基底节。
我们患者中发现的帕金森综合征可能是由于黑质和基底节均受累。该病例进一步强调了白塞病神经表现的多样性。