title: 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.
  
  
  CONCLUSIONS
  The pseudotumoural form of NBD is a rare and life-threatening condition.
authors.affiliation: Service de Médecine Interne, AP-HP, Hôpital Pitié-Salpêtrière, Université Pierre et Marie Curie, Paris F-75013, France.
authors.forename: Nicolas
authors.initials: N
authors.lastname: Noel
authors.forename: Marie
authors.initials: M
authors.lastname: Hutié
authors.forename: Bertrand
authors.initials: B
authors.lastname: Wechsler
authors.forename: Stéphane
authors.initials: S
authors.lastname: Vignes
authors.forename: Du
authors.initials: D
authors.lastname: Le Thi Huong-Boutin
authors.forename: Zahir
authors.initials: Z
authors.lastname: Amoura
authors.forename: Didier
authors.initials: D
authors.lastname: Dormont
authors.forename: Véronique
authors.initials: V
authors.lastname: Delcey
authors.forename: Marc
authors.initials: M
authors.lastname: Polivka
authors.forename: Patrice
authors.initials: P
authors.lastname: Cacoub
authors.forename: David
authors.initials: D
authors.lastname: Saadoun
mesh_terms: D000328:Adult; D001528:Behcet Syndrome / Q000150:complications* / Q000175:diagnosis; D001706:Biopsy; D001921:Brain / Q000000981:diagnostic imaging / Q000473:pathology*; D003937:Diagnosis, Differential; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D009422:Nervous System Diseases / Q000150:complications* / Q000175:diagnosis; D011559:Pseudotumor Cerebri / Q000175:diagnosis / Q000209:etiology*; D012189:Retrospective Studies; D014057:Tomography, X-Ray Computed