[Neuro-Behçet disease and neuro-Sweet disease]

Behçet disease and Sweet disease are multisystem inflammatory disorders involving mucocutaneous tissue as well as nervous system (neuro-Behçet disease and neuro-Sweet disease). Pathological findings in the encephalitis are chiefly perivascular cuffing of small venules by neutrophils, T lymphocytes, and macrphages. Destruction of the brain substrates is mild in neuro-Sweet disease compared with that of neuro-Behçet disease, especially that of chronic progressive subtype. HLA (human leukocyte antigen)-B51 is frequently positive in neuro-Behçet disease, and the frequencies of HLA-B54 and Cw1 in neuro-Sweet disease are significantly higher than not only those in Japanese normal controls but also those in patients with these diseases without nervous complications. These HLA types are considered as risk factors which are directly associated with the etiology of these diseases. Prednisolone is usually used for the treatment of acute phase of both diseases. Methotrexate and infliximab are administered to patients with the chronic progressive type of neuro-Behçet disease. Colchicine and dapsone are prescribed to prednisolone-dependent recurrent cases of neuro-Sweet disease.