Pseudotumoural presentation of neuro-Behcet's disease: case series and review of literature.

OBJECTIVE: To describe the pseudotumoural presentation of neuro-Behçet's disease (NBD). METHODS: We report here the main characteristics, treatment and outcome of 23 patients (5 personal cases and 18 patients from the literature) with a pseudotumoural presentation of NBD. Pseudotumoural NBD patients were compared with 69 consecutive patients, with a classical form of NBD. RESULTS: The median age was 39 (range 27-48 years) years, with a male predominance (65.2%). Clinical features of the pseudotumoural NBD included hemi- or tetra-pyramidal symptoms (n = 20), headache (n = 17), cerebellar syndrome (n = 3), sphincter impotence (n = 3) and pseudobulbar signs (n = 2). CNS imaging showed pseudotumoural lesions mainly in the capsulo-thalamic area (69.6 vs 11.6% for classical NBD; P < 0.01). Histological analysis revealed necrotic lesions with perivascular inflammatory infiltrate without signs of tumoural or infectious lesions. Patients with pseudotumoural NBD had more severe initial disability status (Rankin's score ≥3 in 65.2 vs 24.7%; P < 0.01) and had a 3 years' longer duration between neurological signs and BD diagnosis (P = 0.01) compared with patients with classical NBD. Treatment consisted of CSs (n = 21, 95.5%) and immunosuppressive agents (n = 10, 35.7%) that led to complete clinical and imaging remission in 60.9% of patients. Two (8.7%) of the 23 patients with pseudotumoural NBD died of bedridden state complications. CONCLUSION: The pseudotumoural form of NBD is a rare and life-threatening condition.

Cluster analysis of arterial involvement in Takayasu arteritis reveals symmetric extension of the lesions in paired arterial beds.

OBJECTIVE: The determinants of vessel targeting are largely unknown in vasculitides. This study was undertaken to identify patterns of vascular involvement in Takayasu arteritis (TA), using objective classification of vascular beds. We postulated that cluster analysis could unveil preferential associations between vascular beds commonly affected by TA. METHODS: Peripheral vascular Doppler, computed tomography angiography, and angio-magnetic resonance imaging data from 82 patients with TA (according to the American College of Rheumatology criteria) were studied between January 1995 and May 2006. Cross-relationships of involvement between 24 main arteries were assessed using the phi correlation coefficient. Identification of patterns of vascular involvement was performed using agglomerative hierarchical cluster analysis. RESULTS: Data were obtained from 82 patients (68 women [82.9%] and 14 men [17.1%]). The median duration of followup was 5.1 years (range 1 month to 30 years). For 16 (80%) of 20 paired arteries, the highest correlation of involvement was observed with the contralateral artery. Conversely, disease extension was contiguous in the aorta. Cluster analysis further confirmed that all paired arterial beds, except for the internal and external carotid arteries, clustered with their contralateral counterpart and that the aortic arch, the descending thoracic aorta, and the abdominal aorta clustered together. CONCLUSION: Our findings reveal that TA lesions mostly develop in a symmetric manner in paired vascular territories and that disease extension is contiguous in the aorta. This may prove useful for improving the radiologic followup of patients with TA and for providing a pattern for further investigations focusing on the mechanisms of vessel specificity in vasculitides.

Long-term outcome of neuro-Behçet's disease.

OBJECTIVE: To report the long-term outcome of neurologic involvement in patients with Behçet's disease (BD). METHODS: We performed a retrospective analysis of 115 patients who fulfilled the international criteria for BD (57% male; median age 37 years [interquartile range (IQR) 30-46 years]) and had neuro-BD (NBD) after exclusion of cerebral venous thrombosis. Factors associated with relapse of NBD, inability to perform activities of daily living, and mortality were assessed. RESULTS: Seventy-eight patients (68%) presented with acute NBD and 37 (32%) presented with a progressive course. The HLA-B51 allele was carried by 49% of the patients. Overall, 46 of 115 patients (40%) had severe disability at baseline, represented by a Rankin score of ≥3. The 5- and 7-year event-free survival rates were 65% and 53%, respectively. In multivariate analysis, a positive HLA-B51 status was independently associated with the risk of NBD relapse, with an odds ratio (OR) of 3.6 (95% confidence interval [95% CI] 1.5-9.1). After a median followup of 73 months (IQR 59-102 months), 29 patients (25.2%) became dependent (were unable to perform activities of daily living) or died. Factors independently associated with poor outcome were paresis at onset (OR 6.47 [95% CI 1.73-24.23]) and location of inflammatory lesions at the brainstem on magnetic resonance imaging (OR 8.41 [1.03-68.43]). All 115 patients were treated with corticosteroids; 53 (46.1%) also took cyclophosphamide and 40 (34.8%) also took azathioprine. A trend toward longer event-free survival was observed in patients with severe NBD (i.e., with a Rankin score of ≥3 at onset) receiving intravenous cyclophosphamide compared with those receiving azathioprine (P = 0.06). CONCLUSION: Our findings indicate that NBD is a severe condition in which patients with the HLA-B51 allele appear to experience a worse prognosis.