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

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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.

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Authors and Affiliations

  1. Neurosurgery department, Fatouma Bourguiba Hospital, Monastir, Tunisia

    Mohamed Amine Hadj Taieb

  2. Neurology department, Fatouma Bourguiba Hospital, Monastir, Tunisia

    Houssem Slimane, Mariem Mhiri, Rihab Ben Dhia, Nizar Daoussi & Mahbouba Frih-Ayed

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  1. Mohamed Amine Hadj Taieb
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Correspondence to Mohamed Amine Hadj Taieb.

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This study was approved by the ethical committee of our hospital Fattouma Bourguiba Monastir, Tunisia.

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Hadj Taieb, M., Slimane, H., Mhiri, M. et al. Pseudotumoral neuro-behcet’s disease: case series and review of literature. Acta Neurol Belg 124, 431–445 (2024). https://doi.org/10.1007/s13760-024-02477-1

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