Parenchymal Neuro-Behçet disease with heterogeneous clinical presentations: a comparative case report

Abstract

Neuro-Behçet disease (NBD) is a rare but potentially disabling manifestation of Behçet disease (BD). Its heterogeneous neurological presentation can mimic central nervous system infection, cerebrovascular disease, inflammatory demyelination or intracranial tumors, making early diagnosis difficult. Importantly, there is still limited published data on NBD from Palestine regarding presentation, diagnosis, imaging and management. We describe two patients with a long history of recurrent oral and genital ulceration who were diagnosed with parenchymal Neuro-Behçet disease (P-NBD). The first patient was a 26-year-old man with a two-week progressive headache, fever, vomiting, and blurred vision, which resembled meningitis, in whom examination showed central facial weakness, gait imbalance and pathological laughter. The second patient was a 41-year-old woman with a two-year history of gait imbalance and dysarthria who presented with three days of dizziness, horizontal nystagmus, central facial weakness, right lower-limb weakness, and a positive bilateral extensor plantar response. Laboratory testing and brain magnetic resonance imaging (MRI) were performed to exclude other differential diagnoses. Both received intravenous methylprednisolone followed by an immunosuppressive agent. The first patient had clinical and radiological improvement after corticosteroids and initiation of induction cyclophosphamide, with clinical improvement on follow-up. The second patient improved significantly on imaging after infliximab maintenance therapy. She also improved clinically, but chronic symptoms of gait imbalance and dysarthria were still present. These two cases illustrate the variable presentation of P-NBD, including a meningitis-like febrile syndrome with pathological laughter and an acute-on-chronic brainstem syndrome. In patients with recurrent oro-genital ulceration and unexplained brainstem or diencephalic neurological findings, NBD should be considered after careful exclusion of other disorders. Early diagnosis and immunosuppressive treatment can lead to substantial clinical and radiological improvement.