In patients with recurrent oro-genital ulceration and unexplained brainstem or diencephalic neurological findings, Neuro-Behçet disease should be considered after careful exclusion of other disorders, and early diagnosis and immunosuppressive treatment can lead to substantial clinical and radiological improvement.
Key Findings
Results
Two patients with long histories of recurrent oral and genital ulceration were diagnosed with parenchymal Neuro-Behçet disease (P-NBD) but presented very differently.
Patient 1 was a 26-year-old man with a two-week progressive headache, fever, vomiting, and blurred vision that resembled meningitis.
Patient 2 was a 41-year-old woman with a two-year history of gait imbalance and dysarthria who presented with three days of dizziness and new neurological signs.
The authors note there is limited published data on NBD from Palestine regarding presentation, diagnosis, imaging and management.
Results
The first patient's presentation was a meningitis-like febrile syndrome with central neurological signs and pathological laughter.
Examination showed central facial weakness, gait imbalance and pathological laughter.
The presentation mimicked central nervous system infection, which complicates early diagnosis.
Laboratory testing and brain MRI were performed to exclude other differential diagnoses.
Results
The second patient had an acute-on-chronic brainstem syndrome.
Over three days she developed dizziness, horizontal nystagmus, central facial weakness, right lower-limb weakness, and a positive bilateral extensor plantar response.
These acute findings were superimposed on a two-year history of gait imbalance and dysarthria.
Laboratory testing and brain MRI were performed to exclude other differential diagnoses.
Results
The first patient improved clinically and radiologically after corticosteroids and cyclophosphamide induction.
Treatment was intravenous methylprednisolone followed by induction cyclophosphamide.
Clinical improvement was maintained at follow-up.
Results
The second patient improved significantly on imaging after infliximab maintenance therapy but retained chronic neurological symptoms.
Treatment was intravenous methylprednisolone followed by infliximab maintenance.
She also improved clinically, but chronic gait imbalance and dysarthria persisted.
Conclusions
NBD should be considered in patients with recurrent oro-genital ulceration and unexplained brainstem or diencephalic findings, after excluding other disorders.
NBD's heterogeneous presentation can mimic CNS infection, cerebrovascular disease, inflammatory demyelination or intracranial tumors.
Early diagnosis and immunosuppressive treatment can lead to substantial clinical and radiological improvement.
This is a two-patient case report, so findings are descriptive.
What This Means
This report describes two people from Palestine with Behçet disease, a condition that causes recurrent mouth and genital ulcers, who developed a rare brain complication called parenchymal Neuro-Behçet disease. The first was a young man whose fever, headache, vomiting and blurred vision looked like meningitis. He also had facial weakness, balance problems and episodes of pathological laughter. The second was a woman with two years of balance and speech problems who suddenly developed dizziness, abnormal eye movements and weakness on one side.
Both were tested to rule out other causes, including infection and tumors, and both had brain MRI. Both received high-dose intravenous steroids followed by an immune-suppressing drug. The man received cyclophosphamide and improved clinically and on imaging. The woman received infliximab and showed marked improvement on imaging and some clinical improvement, though she still had long-standing balance and speech problems.
This research suggests that Neuro-Behçet disease can look very different from one person to another and can be mistaken for other brain conditions. Doctors may need to consider it in people with a history of recurrent mouth and genital ulcers who develop unexplained brain symptoms. As a report of only two patients, it cannot show how well the treatments work in general, but it illustrates that early recognition and immune-suppressing treatment were linked to improvement in these cases.
Zaro A, Alatrash Y, Amro R, Nairoukh M, Itmaiza N, Qaysia I. (2026). Parenchymal Neuro-Behçet disease with heterogeneous clinical presentations: a comparative case report.. Frontiers in immunology. https://doi.org/10.3389/fimmu.2026.1965989