[Manifestation of neuro-Behçet disease in cyclosporin A therapy]

Behçet's disease is a chronic inflammatory disorder characterized by the triad of oral and genital ulcers and ocular lesions. One of the most life-threatening manifestations results from involvement of the central nervous system, presenting as necrotising meningo-encephalitis, most typically affecting the brain stem, internal capsula and basal brain ganglia. We report on a young Caucasian mate with Behçet's disease (HLA B 51+) and recurrent uveitis, who presented with acute neurologic involvement under CyA therapy 5 years after first diagnosis. At the time of admission MRI showed two high intensity lesions in the brain stem on T1 weighted images enhanced with Gd-DTPA, reflecting active inflammation. Shortly after admission the CyA treatment was stopped and a therapy with high dose steroids and chlorambucil, starting with a dose of 2 mg daily was initiated. This led to improvement of neurologic symptoms, also documented by brain stem evoked potentials and investigations of cerebrospinal fluid, as well as of ophtalmologic symptoms within few days of treatment. Steroids were reduced to a maintenance dose of 12 mg Prednyliden daily. The brain MRI taken 8 weeks after onset of chlorambucil treatment showed the same lesions in the brain stem, with low intensity in the T1 weighted images an no longer enhanced Gd-DTPA uptake. Chlorambucil dose was reduced to 2 mg every second day after 8 months. There was no exacerbation in the follow-up of 12 months. We conclude that a 6-week Chlorambucil therapy consisting of 2 mg/p.o./d led to remission of neurologic involvement firstly evolving under CyA-medication which suggests superiority of chlorambucil as a treatment modality in neurologic as well as ophtalmologic features of the disease.