Pseudotumoral neuro-behcet’s disease: case series and review of literature

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...

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Bibliographic Details
Published in:Acta neurologica Belgica 2024-04, Vol.124 (2), p.431-445
Main Authors: Hadj Taieb, Mohamed Amine, Slimane, Houssem, Mhiri, Mariem, Ben Dhia, Rihab, Daoussi, Nizar, Frih-Ayed, Mahbouba
Format: Article
Language:English
Subjects:
Adolescent
Adult
Behcet Syndrome - complications
Behcet Syndrome - diagnosis
Biomedical and Life Sciences
Biomedicine
Brain - diagnostic imaging
Brain - pathology
Cerebellar Diseases
Child
Female
Humans
Immunosuppressive Agents - therapeutic use
Magnetic Resonance Imaging
Male
Medicine/Public Health
Middle Aged
Neoplasm Recurrence, Local
Neurology
Neuroradiology
Neurosciences
Original Article
Young Adult
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Summary: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.
ISSN:0300-9009
2240-2993
DOI:10.1007/s13760-024-02477-1