Objective:

To describe a rare case of pseudo-tumoral Neuro-Behçet’s disease presenting with atypical MRI findings mimicking a neoplastic process, and to highlight the importance of considering inflammatory etiologies such as Neuro-Behçet’s in the differential diagnosis of demyelinating and tumor-like central nervous system lesions.

Background:

Neuro-Behçet’s disease (NBD) is a rare neurological manifestation of Behçet’s disease that typically involves the brainstem and basal ganglia. In exceptional cases, NBD can present with tumor-like lesions mimicking neoplastic or demyelinating diseases, posing significant diagnostic challenges due to overlapping clinical and radiologic features.

Design/Methods:

A 37-year-old female presented with headache and cognitive disturbances. MRI showed T2/FLAIR hyperintensities in the bilateral frontal lobes, left insula, temporal lobe, caudate nucleus, and corpus callosum. Brain biopsy revealed demyelination. HLA-B51 and pathergy tests were positive, confirming Neuro-Behçet’s disease. She was treated with high-dose corticosteroids followed by azathioprine.

Results:

High-dose corticosteroid therapy led to rapid clinical improvement. Follow-up MRI one week later showed a marked reduction in lesion size and resolution of enhancement on T2/FLAIR imaging. With prednisone taper and azathioprine, the patient remained clinically stable on neurological examination.

Conclusions:

Neuro-Behçet’s disease can present as pseudo-tumoral lesions mimicking neoplasms or demyelinating disease. This case underscores the need to consider NBD in the differential diagnosis of atypical CNS masses and demonstrates rapid clinical and radiologic improvement with high-dose corticosteroids and subsequent immunosuppression.