The presentation of headache in neuro-Behçet's disease: a case-series

Abstract

Introduction: Behçet's disease (BD) is a chronic, relapsing, multisystemic, inflammatory disorder with unknown etiology. Neurological involvement is observed in about 5% of the patients with BD and headache is a frequently reported symptom with or without neurological involvement. In this case-series, we aim to demonstrate the secondary headaches associated with neuro-Behçet's disease in consecutive BD patients who had been referred for neurologic evaluation.

Methods: The case-series included 17 patients (11 males [65%]), mean age 41 +/- 11.4, who met the criteria for BD established by the International Study Group for BD and developed headache during the disease course.

Results: Seventeen BD patients were identified, the neurologic presentations included in parenchymal involvement 12 (70%), extraparenchymal involvement/venous thrombosis in 5 (30%), seizure disorder in 2 (12%), and psychiatric problems/depression or anxiety in 5 (30%) patients, respectively. The characteristics of the headache according to the patients' reports included subacute, moderate to severe headache with unilateral localization and throbbing quality accompanied by nausea, vomiting, and aggravation upon awakening.

Conclusion: Because neurological involvement is one of the most devastating aspects of BD, the need to treat neurological involvement as early as possible, the possible role of headache onset as an early indicator for neurological involvement in this relatively uncommon disease should be kept in mind.