%0 Case Reports
%T Pseudotumoral neuro-behcet's disease: case series and review of literature.
%A Hadj Taieb MA
%A Slimane H
%A Mhiri M
%A Ben Dhia R
%A Daoussi N
%A Frih-Ayed M
%J Acta Neurol Belg
%V 124
%N 2
%D 2024 Apr 23
%M 38396190
%F 2.471
%R 10.1007/s13760-024-02477-1
%X <B>BACKGROUND: </B>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.<BR><B>METHODS: </B>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.<BR><B>RESULTS: </B>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.<BR><B>CONCLUSIONS: </B>PT NBD is a rare but life-threatening condition.