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Pseudotumoural presentation of neuro-Behcet's disease: case series and review of literature.
MedLine Citation:
PMID:  22337940     Owner:  NLM     Status:  Publisher    
Abstract/OtherAbstract:
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.
Authors:
Nicolas Noel; Marie Hutié; Bertrand Wechsler; Stéphane Vignes; Du Le Thi Huong-Boutin; Zahir Amoura; Didier Dormont; Véronique Delcey; Marc Polivka; Patrice Cacoub; David Saadoun
Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2012-2-15
Journal Detail:
Title:  Rheumatology (Oxford, England)     Volume:  -     ISSN:  1462-0332     ISO Abbreviation:  -     Publication Date:  2012 Feb 
Date Detail:
Created Date:  2012-2-16     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  100883501     Medline TA:  Rheumatology (Oxford)     Country:  -    
Other Details:
Languages:  ENG     Pagination:  -     Citation Subset:  -    
Affiliation:
Department of Internal Medicine, Department of Neuroradiology, Assistance Publique-Hôpitaux de Paris (AP-HP), Groupe Hospitalier Pitié-Salpétrière, Université Pierre et Marie Curie, Department of Internal Medicine and Department of Pathology, AP-HP, Hopital Lariboisière, Paris, France.
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