Case Report Cerebral Small-Vessel Disease in Neuro-Behçet Disease Sohei Yoshimura, MD,* Tetsuro Ago, MD,†‡ Masatoshi Koga, MD,x Masahiro Kamouchi, MD,†k and Takanari Kitazono, MD†‡ Background: Involvement of central nervous system is very common in Behçet disease, known as neuro-Behçet disease (NBD). However, there have been few reports which evaluated stroke-like cerebral small-vessel disease associated with Behçet disease. Objective: We evaluated cerebral small-vessel lesions by using magnetic resonance imaging in 2 patients with NBD. Clinical Cases: In a 41-year-old woman case, who developed dysarthria and left hemiparesis, diffusion-weighted images (DWI) showed small high-intensity areas in the bilateral internal capsule. The right lesion had a low apparent diffusion coefficient (ADC) value, whereas the left one had a high value. In a 59-year-old woman case, who developed dysarthria and left hemihypesthesia, DWI showed high-intensity areas in the right side of the pons and in the right peduncle of the midbrain. The lesion in the pons had a low ADC value, whereas the lesion in the midbrain had a high value. ADC map may be useful to identify symptomatic lesions in the brain, because only the lesions with low ADC values were symptomatic. Conclusions: NBD should be considered in those who had not had any risk factors for ischemic stroke but experienced cerebral smallvessel disease. Key Words: Neuro-Behçet disease—lacunar infarction—apparent diffusion coefficient—diffusion-weighted image. Ó 2015 by National Stroke Association Involvement of central nervous system is reported to occur in 10%-25% of patients with Behçet disease, known as neuro-Behçet disease (NBD).1 Pathologically, inflam- From the *Department of Cerebrovascular Medicine, National Cerebral and Cardiovascular Center, Suita; †Department of Nephrology, Hypertension, and Strokology, Kyushu University Hospital, Fukuoka; ‡Department of Medicine and Clinical Science, Graduate School of Medical Sciences, Kyushu University, Fukuoka; xDivision of Cerebrovascular Medicine and Neurology, National Cerebral and Cardiovascular Center, Suita; and kDepartment of Health Care Administration and Management, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan. Received February 5, 2015; revision received May 5, 2015; accepted May 11, 2015. Address correspondence to Sohei Yoshimura, MD, Department of Cerebrovascular Medicine, National Cerebral and Cardiovascular Center, 5-7-1, Fujishirodai, Suita, Osaka 565-8565, Japan. E-mail: sohei-y@ncvc.go.jp. 1052-3057/$ - see front matter Ó 2015 by National Stroke Association http://dx.doi.org/10.1016/j.jstrokecerebrovasdis.2015.05.012 mation, demyelination, and gliosis in the perivascular region at capillary to very small vessels are observed in NBD.2-4 Moreover, ‘‘stroke-type’’ NBD caused by stenosis, occlusion, dissection, and aneurysm of medium to large size cerebral arteries and venous thrombosis have been reported.5-7 However, there have been few reports which evaluated stroke-like cerebral small-vessel disease associated with Behçet disease. We evaluated cerebral small-vessel lesions by using magnetic resonance imaging (MRI) in 2 patients with NBD. Case Report Case 1 A 41-year-old woman was admitted to our hospital because of moderate left hemiparesis and mild dysarthria without sensory disturbance. She was diagnosed with entero-Behçet disease 9 years earlier and kept taking 2.5 mg of prednisolone per day. She had no risk factors for cerebrovascular diseases. She did not have any Journal of Stroke and Cerebrovascular Diseases, Vol. -, No. - (---), 2015: pp e1-e3 e1 S. YOSHIMURA ET AL. e2 Fig 1. Case 1: MRI showed lesions with hyperintense signal on T2WI (A) and DWI (B) in the bilateral posterior limb of the internal capsule. The ADC value in the right posterior limb was lowered, whereas that of the left one was elevated (C). Case 2: MRI showed lesions with hyperintense signal on T2WI in the right side of the midbrain (D) and pons (G). These lesions were visible as hyperintense areas on DWI (E and H). The ADC value was slightly elevated in the midbrain (F) but lowered in the pons (I). Abbreviations: ADC, apparent diffusion coefficient; DWI, diffusion-weighted images; MRI, magnetic resonance imaging; T2WI, T2-weighted image. worsening of the symptoms related to Behçet disease. Laboratory data showed that markers of inflammation, coagulation, and fibrinolysis were within normal limits. HLA-B51 was negative. Cerebrospinal fluid examination showed mild leukocytic pleiocytosis (17/mm3; mononuclear cell, 90%; polynuclear cell, 10%) and slightly increased protein levels (40 mg/dL). MRI showed lesions with hyperintense signal on T2-weighted image (Fig 1, A) and diffusion-weighted images (DWI; Fig 1, B) in the bilateral posterior limb of the internal capsule. The apparent diffusion coefficient (ADC) value of the right lesion was low, whereas that of the left one was high (Fig 1, C). Case 2 A 59-year-old woman noticed nausea, mild left hemiparesis, hemihypesthesia, and dysarthria. She had kept taking oral prednisolone (5 mg per day) against the uveitis related to Behçet disease for 17 years and had steroid-induced diabetes. She had no risk factors for cerebrovascular diseases except steroid-induced diabetes. Worsening of oral and genital ulcers, and erythema nodosum on the bilateral lower thigh were observed. Laboratory data showed that white blood cell count (9,440/mL), C-reactive protein (.7 mg/dL), and erythrocyte sedimentation rate (49 mm/hour) were slightly elevated. Markers of coagulation and fibrinolysis were within normal limits. HLA-B51 was negative. Cerebrospinal fluid examination showed mild leukocytic pleiocytosis (12/ mm3; mononuclear cell, 100%) and slightly increased protein levels (24 mg/dL). MRI showed lesions with hyperintense signal on T2-weighted image (Fig 1, D,G) and DWI (Fig 1, E,H) in the right side of the midbrain and of the pons (Fig 1, D,G). The ADC value was low in the pons (Fig 1, F), whereas slightly high in the midbrain (Fig 1), I. In both cases, magnetic resonance angiography showed no stenotic lesion of the cerebral arteries. After treatment with high dose of prednisolone (50 mg per day), their neurologic symptoms disappeared and the sizes of the lesions decreased in the follow-up MRI. Discussion Small-vessel lesions in the brain were found in the present cases by using MRI. Interestingly, 2 distinct lesions were detected in both cases. Among them, only the lesions with low ADC values were symptomatic. In Behçet disease, inflammation at capillary to very small arteries underlies its pathology, and acute lesions of typical NBD are shown as hyperintense areas on both DWI and ADC map.8,9 In the present 2 cases, such lesions were found in the left internal capsule (Case 1) and in the midbrain (Case 2). Additionally, areas with DWI hyperintensity and ADC hypointensity were also detected in these cases, indicating the presence of ischemic change. Therefore, severely damaged perforating arteries may CEREBRAL SMALL-VESSEL DISEASE IN NEURO-BEHÇET DISEASE have caused an ischemia in the distal portion in addition to inflammatory changes of the arteries. Two distinct lesions, that is, inflammatory and ischemic lesions of cerebral small vessels, coexisted in the present cases with NBD. However, these patients presented neurologic symptoms only due to ADC hypointense areas corresponding to ischemic lesions, whereas inflammatory changes did not cause any symptoms. Therefore, ADC map may be useful to identify symptomatic lesions in the brain. MRI revealed cerebral small-vessel lesions mimicking lacunar infarction in patients with NBD. Behçet disease may be one of the important causes of small-vessel diseases in the brain especially in young patients. 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