Neuro-Behçet's disease: a report of sixteen patients

Abstract

Background: Neurologic involvement in Behçet's disease (BD), also known as neuro-Behcet's disease (NBD), is one of the most devastating manifestations of the disease. The etiology of BD remains obscure and speculative. NBD usually occurs 1-10 years after the first symptom of BD has occurred, and its incidence is 18% (range of 4%-49%).

Objective: This study was conducted to describe the clinical and prognostic aspects of neurologic involvement in BD among patients attending a multidisciplinary hospital clinic.

Methods: Eighty patients with BD were diagnosed according to the International Study Group Criteria for BD at our hospital. Sixteen patients (20%, including 11 men and five women) had evidence of and fulfilled the diagnostic criteria for NBD. These patients underwent laboratory and imaging investigations, including human leukocyte antigen-typing, lumbar puncture, electroencephalographic studies, and computed tomography scanning.

Results: Fourteen of the 16 patients received high-dose steroids, and four of these 14 patients were treated with a combination of steroids and cytotoxic agents. Relief was observed in 14 of 16 patients (87.5%). The remaining two patients were untreated males who suffered severe brainstem lesions, and later died.

Conclusion: In contrast with previous reports of a poor prognosis with NBD, our study shows that early aggressive intervention with corticosteroids and cytotoxic agents may ameliorate the prognosis in these patients, and can diminish and stabilize the negative effect of neurologic involvement.

Keywords: aphthous ulceration; genital ulceration; iridocyclitis; neuro-Behçet’s disease; neurologic involvement.

Figure 1

Triple symptom complex commonly observed in Behcet’s disease: Aphthous ulceration, iridocyclitis, and genital ulceration. Aphthous ulceration often appears as the first symptom, ie, a painful, oval or round, shallow or deep, 1–20 mm lesion with central whitish or yellowish necrotic base and red halo. Almost always present during flares, and may precede other features by years. Iridocyclitis and ocular disease is usually more bilateral than unilateral, with anterior uveitis and hypopyon commonly observed. Retinal involvement may also be present, ie, retinal vasculitis/optic neuritis or atrophy and genital ulceration, which are similar in appearance and usually present as large ulcers which are deep and often cause scarring.