Diagnostic dilemma in CNS Behcet's disease

Area of Science:

• Neurology
• Immunology
• Pathology

Background:

• Central nervous system (CNS) involvement in Behçet's disease is rare but can present with diverse neurological deficits.
• Differentiating CNS Behçet's disease from CNS lymphoma is critical due to vastly different treatment protocols and prognoses.

Observation:

• A young adult male presented with acute neurological dysfunction and painful mucocutaneous ulcers.
• Cerebral magnetic resonance imaging (MRI) revealed a large, enhancing mass-like lesion in the brainstem and surrounding tissues.
• Biopsies of oral and genital lesions showed non-specific inflammatory changes.

Findings:

• The clinical presentation, MRI findings, and biopsy results were consistent with either CNS lymphoma or CNS Behçet's disease.
• CNS Behçet's disease was considered the most likely diagnosis given the overall clinical picture.
• The lesion was not amenable to biopsy, necessitating a presumptive diagnosis.

Implications:

• This case highlights the importance of considering CNS Behçet's disease in the differential diagnosis of CNS mass lesions, especially in the presence of characteristic mucocutaneous findings.
• High-dose immunosuppressive therapy can lead to significant neurological recovery in CNS Behçet's disease.
• Further research is needed to improve diagnostic accuracy and therapeutic strategies for CNS Behçet's disease.