Journal of neuroimmunology · 2026
Neuro-Behçet's disease: Clinical spectrum, management, and outcomes from an Indian cohort.
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Mohapatra Prachi, Garg Divyani, Garg Ajay, Radhakrishnan Divya M, Pandit Awadh K, Gaikwad Shailesh B, Srivastava Achal K, Agarwal Ayush
Аннотация
BACKGROUND: Neuro-Behçet's disease (NBD) is a severe neurological manifestation of Behçet's disease. Data on NBD from South Asia remains limited. We describe the clinical spectrum, neuroimaging features, treatment, and outcomes of NBD in an Indian cohort.
METHODS: We conducted a retrospective chart and neuroimaging review of NBD patients evaluated at a tertiary care referral centre in India between January 2021 and October 2025. Behçet's disease was classified using the International Criteria for Behçet's Disease, and NBD was categorized as probable or definite according to international consensus recommendations. Clinical features, neuroimaging findings, laboratory data, treatments, and functional outcomes (mRS and EDSS) were extracted. Movement disorders were systematically characterized.
RESULTS: Twelve patients were included (all males; median age at neurological onset 29.0 years [IQR 25.0-39.8]). Neurological-onset disease occurred in 9 patients (75.0%). Ten patients (83.3%) had probable NBD, and two (16.7%) had definite NBD. Parenchymal disease predominated (9, 75%), with brainstem involvement in 9 patients (75%). Movement disorders were present in eight patients (66.7%), most commonly cerebellar ataxia, and were significantly associated with brainstem involvement (100% vs 25%; p = 0.018). Optic nerve involvement occurred in 7 patients (58.3%) [optic neuritis in 4 (33.3%), secondary optic neuropathy in 3 (25.0%), and one overlap]. Cerebrospinal fluid (CSF) showed inflammatory changes, with CSF-restricted oligoclonal bands detected in one patient (8.3%). During a median follow-up of 15 months (IQR 5-40), relapses occurred in 7 patients (58.3%), while clinical improvement was observed in 10 (83.3%). Intravenous methylprednisolone and plasma exchange were used in 10 (83.3%) and 4 (33.3%) patients, respectively. Steroid-sparing immunosuppression (most commonly azathioprine, 75%) and biologic therapy (most commonly rituximab, 33.3%) were frequently required, with 7 patients (58.3%) requiring ≥1 agent during follow-up.
CONCLUSIONS: NBD in this Indian cohort closely mirrored global disease patterns. Movement disorders, particularly ataxia, are frequent and strongly associated with brainstem involvement, highlighting an under-recognized dimension of parenchymal NBD.
METHODS: We conducted a retrospective chart and neuroimaging review of NBD patients evaluated at a tertiary care referral centre in India between January 2021 and October 2025. Behçet's disease was classified using the International Criteria for Behçet's Disease, and NBD was categorized as probable or definite according to international consensus recommendations. Clinical features, neuroimaging findings, laboratory data, treatments, and functional outcomes (mRS and EDSS) were extracted. Movement disorders were systematically characterized.
RESULTS: Twelve patients were included (all males; median age at neurological onset 29.0 years [IQR 25.0-39.8]). Neurological-onset disease occurred in 9 patients (75.0%). Ten patients (83.3%) had probable NBD, and two (16.7%) had definite NBD. Parenchymal disease predominated (9, 75%), with brainstem involvement in 9 patients (75%). Movement disorders were present in eight patients (66.7%), most commonly cerebellar ataxia, and were significantly associated with brainstem involvement (100% vs 25%; p = 0.018). Optic nerve involvement occurred in 7 patients (58.3%) [optic neuritis in 4 (33.3%), secondary optic neuropathy in 3 (25.0%), and one overlap]. Cerebrospinal fluid (CSF) showed inflammatory changes, with CSF-restricted oligoclonal bands detected in one patient (8.3%). During a median follow-up of 15 months (IQR 5-40), relapses occurred in 7 patients (58.3%), while clinical improvement was observed in 10 (83.3%). Intravenous methylprednisolone and plasma exchange were used in 10 (83.3%) and 4 (33.3%) patients, respectively. Steroid-sparing immunosuppression (most commonly azathioprine, 75%) and biologic therapy (most commonly rituximab, 33.3%) were frequently required, with 7 patients (58.3%) requiring ≥1 agent during follow-up.
CONCLUSIONS: NBD in this Indian cohort closely mirrored global disease patterns. Movement disorders, particularly ataxia, are frequent and strongly associated with brainstem involvement, highlighting an under-recognized dimension of parenchymal NBD.