베체트병의 중추신경 침범양상에 대한 임상적, 영상적 고찰

Abstract

Background and Objectives: Behcet's disease (BD) may develop variable neuro logic manifestations. We herein report patients with neuroBD with special reference to clinical, cerebrospinal fluid (CSF), and radiological findings. Methods: Twenty-six patients, who fulfilled international BD criteria, were enrolled. Twenty-four of them fulfilled the criteriae at the time of, and another two at a three and four month follow up after presenting neurological manifestations. Twenty-two patients had CSF examination. All but three patients had imaging studies (CT scan in 7 and MRI in 16). Results: Most patients with neuroBD presented with focal neurologic deficits, followed by meningitic symptoms and behavioral changes or cognitive dysfunctions. The common initial neurological symptoms or signs were hypereflexia, headache, dysarthria, and disturbances of eye movement in order of frequency. One or more parameters of CSF examinations were abnormal in all but two patients. Mean WBC count was 165±279/㎣ (9-1320). Nine of them were neutrophilic (polys >50%). Protein was mildly ele vated up to 155 mg/dI. Neuroimaging studies showed abnormal findings in 18 patients (75 %). Increased signal intensity on T2 weighted image was the most common finding. Basal ganglia, midbrain, pons and thalamus were the common sites of involvement in that of frequency. Although most patients had a lesion in the brain parenchyme or meninges, one patient had venous sinus thrombosis and another one had myelitis. Clinical outcomes were good in that all but three patients improved. However, 10 of them (38 %) recurred during their follow-up period of average 36 months. Conclusions: Our findings suggest that neuroBD may present with variable neurologic manifestations and show characteristic neutrophilic CSF findings and multifocal brainstem or diencephalon involvements. The frequent recurrence, despite their good clinical outcomes, points up the need for careful long-term neuologic observations.