Neuro-Bechet’s disease: a case series from India

Abstract

Abstract Background There are several studies which have studied large cohort of Neuro-Bechet’s disease (NBD) patients worldwide However, there is sparse literature about NBD from India. We aimed to characterize the clinical, radiological characteristics, treatment response and outcome in NBD. Methods The study was a retrospective descriptive analysis of a cohort of patients with NBD evaluated between January 2017 to June 2021, fulfilling the International Consensus Recommendation (ICR) criteria for NBD. Results Twelve patients were diagnosed as NBD during the study period. The mean age of the patient was 34.7 ± 11.1 (range 21–59 years). The mean duration of illness was 1.4 ± 1.2 years. All 12 patients had neuroparenchymal NBD. Systemic symptoms were present in 6 patients (50%). Pyramidal tract involvement (67%) was the most common symptom and sign followed by cranial nerve, spinal cord and visual involvement. Pathergy test was positive in 6 patients (50%). Human leucocyte antigen (HLA) B51 positivity was seen in all patients. Thalamus (100%) was the most common area involved followed by pons (80%). Favourable outcome (modified Rankin Scale scores ≤ 2) was seen in 7 patients, poor outcome in 3 patients and 2 patients were lost to follow-up after first attack. Conclusion NBD is prevalent in India and there is need for clinical suspicion. Brainstem and cerebral syndrome are the most common presentation of NBD and thalamus is the most common site of involvement in NBD.

Keywords

• Neuro-Behcet’s disease
• HLA B51
• Pathergy test