Neuro-Behcet is a great masquerader of chronic meningitis and demyelination diseases.

This case is unique because of extensive spinal cord involvement which is extremely rare in BD, along with multiple ring-enhancing lesions in Neuro-Behcet.

A 14-year-old boy with progressive spastic quadriparesis, dysphagia, dysarthria, decreased sensation below C2 spinal segment, increased urinary frequency, urgency and constipation for 10 months. History revealed recurrent episodes of fever, mouth ulcers and non-migratory multiple large joint pain, recurrent episodes of painful watery eyes for the past 7 years for which he was never evaluated.

The patient was evaluated two years back due to fever and weakness and based on magnetic resonance imaging (MRI brain and spine) findings and Cerebrospinal fluid (CSF) analysis (high protein and low sugar but negative for all infective etiology including tuberculosis) the patient was started anti-tubercular treatment (ATT). The patient keeps on deteriorating despite strict compliance with drugs.

      On admission patients’ routine blood investigations were normal. MRI brain showing multiple rings enhancing lesions with holo-cord myelitis. CSF examination showed cell-180 (neutrophils 66%), sugar-38 mg/dl, protein- 70 mg/dl, RBS-104 mg/dl with negative for all infection. Oligoclonal bands were not detected. Anti-aquaporin-4, Myelin oligodendrocyte antibody, and auto-immune panel were negative. Human Leukocyte Antigen (HLA B-5/51) testing was positive. Given recurrent oral ulcers, genital scarring, anterior uveitis, non-erosive arthritis, typical brain and spinal cord involvement, CSF/MRI findings and positive HLA B51, a final diagnosis of Neuro-behçet’s disease was made.

The patient was started on pulse methylprednisolone (1 gm/day) and cyclophosphamide injection (750 mg/m²) every two weeks. The patient deteriorated rapidly due to brain stem involvement and succumbed to sepsis.