AB0049 NEURO-BEHCET’S DISEASE: EPIDEMIOLOGICAL, CLINICAL, PARACLINICAL AND THERAPEUTIC CHARACTERISTICS
Authors: B. Mayssa, H. Tounsi, W. Garbouj, S. Wafa, R. Amri
Journal: Annals of the Rheumatic Diseases (2024)

<h3>Background:</h3> Behçet's disease (BD) is a systemic vasculitis that can affect all vessels. Neurological involvement in this chronic pathology varies, depending on the series, from 4% to 49% of cases. <h3>Objectives:</h3> The aim of this study was to investigate the epidemiological, clinical and therapeutic features of neurological involvement in Behçet's disease. <h3>Methods:</h3> A Retrospective and descriptive study, was conducted within the internal medicine department of Taher Maamouri Hospital, between 2015 and 2023. All patients meeting the new MB classification criteria (International Criteria for Behçet's Disease: ICBD) and with neurological involvement were included. <h3>Results:</h3> In a cohort of 113 patients diagnosed with Behçet's disease, fifteen had neurological involvement (13,4%). The male to female ratio was 2. The mean age at diagnosis of MB was 33 years. The mean age at onset of neurological manifestations was 36. It was inaugural in 6 cases. The mean delay between the onset of neurological involvement and the diagnosis of MB was 8.5 years. Fourteen patients had central involvement and one patient had peripheral nervous system involvement. The most common symptoms were headache (n=10), generalized tonic-clonic seizure (n=2) and behavioral disturbance (n=2). Clinical examination revealed pyramidal signs with or without focal neurologic deficit (n=7), cerebellar syndrome (n=2), confusion (n=2) and ocular motor nerve palsy (n=2). Central involvement included meningoencephalitis (n=1), brain stem involvement (n=4), pseudotumor cerebri (1 case), recurrent ischemic stroke (3 cases), cerebral venous thrombosis (3 cases) and psychiatric involvement (n=3). Lumbar puncture was performed in 4 patients. CSF was abnormal in one case, with a predominantly lymphocytic pleocytosis. Cerebral CT scans were performed in 5 cases and were normal in one case. Brain magnetic resonance imaging (MRI), performed for all patients, showed multiple brainstem lesions in 4 cases, cerebellar involvement in 2 cases, demyelinating lesions in periventricular in 7 cases, ischemic stroke in 3 cases, signs of cerebral venous thrombosis in 3 cases, cerebellar atrophy in one case and a pseudotumoral appearance in one case. MRI was normal in 2 cases. Other extra-neurological involvement included mucocutaneous manifestations in all patients, articular manifestations (n=5), ocular manifestations (n=7) and vascular manifestations (n=4). All patients with neurologic involvement were treated with corticosteroids. Immunosuppressive therapy was associated in 12 cases. Curative anticoagulation was prescribed in 3 cases. Antipsychotic treatment was prescribed in 3 cases. Among fifteen patients, nine achieved prolonged remission, six relapsing-remitting and four had a persistently active disease. The Average follow-up was 9 years. <h3>Conclusion:</h3> The neurological involvement in Behcet's disease is potentially serious in the absence of early and specific management. <h3>REFERENCES:</h3> <b>NIL.</b> <h3>Acknowledgements:</h3> <b>NIL.</b> <h3>Disclosure of Interests:</h3> <b>None declared.</b>