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Among a variety of central nervous system (CNS) manifestations of Behçet's disease, the most serious one is progressive dementia. However, there has been no effective treatment to prevent the progression of dementia. The present studies have examined the efficacy of low-dose weekly methotrexate therapy for the progressive dementia in Behçet's disease. Six patients with the progressive type of neuro-Behçet's disease were treated with low-dose weekly methotrexate (7.5-15 mg/week). Neurological findings, psychological findings, brain MRI and IL-6 in cerebrospinal fluid were evaluated at the start and one year after the initiation of the therapy. All the 6 patients were refractory to the conventional therapy for neuro-Behçet's disease. The progression of neuropsychological abnormalities such as dementia was not recognized after one year from the initiation of methotrexate therapy. Consistently, IL-6 levels in cerebrospinal fluid were significantly decreased after one year from the initiation of therapy. Six months after the discontinuation of low-dose weekly methotrexate, progression of neuropsychological abnormalities such as dementia was recognized, and IL-6 levels in cerebrospinal fluid were significantly increased. These findings may suggest the suppression of the ongoing inflammatory process in the intracranial lesions by methotrexate. These results suggest that low-dose weekly methotrexate therapy may be a new promising method for the treatment of the progressive type of neuro-Behçet's disease.

Low-dose weekly methotrexate therapy for progressive neuro-Bechcet's disease.