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

Rheumatology (Oxford). 2012 Jul;51(7):1216-25. doi: 10.1093/rheumatology/ker449. Epub 2012 Feb 15.

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.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Behcet Syndrome / complications*
  • Behcet Syndrome / diagnosis
  • Biopsy
  • Brain / diagnostic imaging
  • Brain / pathology*
  • Diagnosis, Differential
  • Female
  • Follow-Up Studies
  • Humans
  • Magnetic Resonance Imaging
  • Male
  • Middle Aged
  • Nervous System Diseases / complications*
  • Nervous System Diseases / diagnosis
  • Pseudotumor Cerebri / diagnosis
  • Pseudotumor Cerebri / etiology*
  • Retrospective Studies
  • Tomography, X-Ray Computed