ABSTRACT

Purpose:

Behçet disease (BD) is a vasculitis with mucocutaneous, ocular, arthritic, vascular, and other manifestations. Its neurologic manifestations named as neuro-Behçet disease (NBD).

Methods:

In this study, three hundred thirty four patients who have fulfilled the International Study group criteria for the diagnosis of BD from 1990 to 2008 were retrospectively evaluated. The clinical records, images, cerebro spinal fluid (CSF) analyses of patients with NBD were recorded.

Results:

Twentytwo patients (15 males, 7 females) with NBD were detected (6.6%). Mean age at neurological presentation was 28.3±8.9 years. Thirteen patients had positive pathergy test and 17 patients had positive HLA-B5 test. Parenchymal central nervous system (CNS) pattern was detected in 14 patients, nonparenchymal CNS pattern was detected in 7 patients, mixt pattern was detected in one patient. Parenchymal involvements were included brainstem involvement, hemispheric manifestations, spinal cord lesions, and meningoencephalitic presentations and nonparenchymal involvement were included dural sinus thrombosis, arterial occlusion. Peripheral neuropathy and myopathy were not seen. Pleocytosis and elevated protein levels were detected in CSF analysis. Iso/hypointense lesions in T1-weighted images and hyperintense lesions in T2-weighted images were seen in magnetic resonance imaging. Corticosteroids and adjuvant immunosuppressive therapy were used for parenchymal manifestations, and corticosteroids and anticoagulants were used for treatment of dural sinus thrombosis.

Conclusion:

Neuro-Behçet disease is relatively rare, but it must be carefully investigated due to their serious prognosis and considered in the differential diagnosis of stroke in young adults, multiple sclerosis, movement disorders, intracranial hypertension, intracranial venous occlusive diseases, and other neurologic syndromes.