Pseudotumoral neuro-behcet's disease: case series and review of literature.
Acta Neurol Belg
; 124(2): 431-445, 2024 Apr.
Article
en En
| MEDLINE
| ID: mdl-38396190
ABSTRACT
BACKGROUND:
Behcet's disease (BD) is a multisystem autoimmune relapsing vasculitis with an almost unknown etiology involving both large and small vessels. The neurological involvement called neuro-Behcet's disease (NBD) is rare. NBD can be responsible for tumor-like masses mimicking low-grade gliomas in only a few cases.METHODS:
We report here the main characteristics, treatment, and outcome of 43 patients (4 personal cases and 39 patients from the literature) with a pseudotumoral presentation of NBD (PT NBD). We compared our findings with those of the classical form of NBD.RESULTS:
The median age was 35.86 (12-59 years) years, with a male predominance (67.4%). PT NBD was the inaugural of the disease in 51.2% of cases. The neurological manifestations included headache (n = 31), pyramidal syndrome (n = 28), cerebellar syndrome (n = 5), behavioral changes (n = 5), and pseudobulbar signs (n = 2). Ophthalmologic examination revealed papilledema in 3 cases. On cerebral imaging, the most affected regions of the brain were the capsulothalamic region (n = 15, 37.5%) and the brainstem (n = 14, 35). Histological analysis revealed necrotic lesions with perivascular inflammatory infiltrate without signs of tumoral or infectious lesions. Treatment consisted of corticosteroids (n = 40, 93%) and immunosuppressive agents (n = 28, 65.11%), leading to complete clinical and imaging remission in 41.5% of patients.CONCLUSION:
PT NBD is a rare but life-threatening condition.Palabras clave
Texto completo:
1
Colección:
01-internacional
Base de datos:
MEDLINE
Asunto principal:
Enfermedades Cerebelosas
/
Síndrome de Behçet
Límite:
Adolescent
/
Adult
/
Child
/
Female
/
Humans
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Male
/
Middle aged
Idioma:
En
Revista:
Acta Neurol Belg
Año:
2024
Tipo del documento:
Article
País de afiliación:
Túnez
Pais de publicación:
Italia