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

Abstract : 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.
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Submitted on : Friday, February 22, 2013 - 6:22:05 PM
Last modification on : Friday, August 5, 2022 - 3:39:48 AM

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Nicolas Noel, Marie Hutié, Bertrand Wechsler, Stéphane Vignes, Du Le Thi Huong-Boutin, et al.. Pseudotumoural presentation of neuro-Behcet's disease: case series and review of literature.. Rheumatology, Oxford University Press (OUP), 2012, 51 (7), pp.1216-1225. ⟨10.1093/rheumatology/ker449⟩. ⟨hal-00793700⟩



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