| Summary: | Behçet’s disease is a multisystem vasculitis which can involve the nervous system known as Neuro-Behçet’s disease. Neurological involvement is uncommon in Behçet’s disease, and has a highly variable prevalence. An atypical rare form with pseudotumoral lesions has been described in literature and only a few cases have been reported. We report herein a case of three patients with pseudotumoral neuro-Behçet’s disease aged between 30 and 45 years old, with a history of recurrent orogenital ulceration. All patients presented with neurological symptoms associated with at least one large space-occupying lesion on cerebral imaging. Behçet’s disease was diagnosed according to the International Study Group criteria. All cases received corticosteroids and immunosuppressant agents with a spectacular clinical and radiological improvement. Keywords: Behçet’s disease, Neuro-Behçet’s disease, Atypical form, Pseudotumoral form
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