The pathological presentations of neuro-Behçet disease: a case report and review of the literature.

OBJECTIVES

To report the neuropathologic features of a patient with neuro-Behçet disease and to review the literature.

METHODS

Herein, we describe a 35-year-old male who fulfills the International Study Group criteria for Behçet disease.

RESULTS

The patient developed headache and hemiparesis in a subacute course. His neurologic examination revealed an altered level of consciousness and unilateral corticospinal signs. The brain magnetic resonance imaging (MRI) showed areas with increased signal intensity in the basal ganglia and cerebral peduncles in T2-weighted and fluid-attenuated inversion recovery (FLAIR) images. Cerebrospinal fluid (CSF) analysis showed mononuclear pleocytosis but normal protein and glucose levels. After his death, the patient's autopsy revealed a perivascular infiltration of mononuclear inflammatory cells, most prominently in basal ganglia, midbrain, and thalamic regions and the subarachnoid space, consistent with presentations of neuro-Behçet disease.

CONCLUSION

Pathologic findings of central nervous system involvement in Behçet disease mainly include perivascular infiltration with lymphocytes, neutrophils and rarely, eosinophils, with or without signs of necrosis. The most common sites of brain involvement include the brainstem and basal ganglia. Nonetheless, other areas can also be involved.