臨床神経学

<シンポジウム(3)―3―3>中枢神経を侵す難治性炎症性疾患の治療法の選択と最適化:Q&A

神経ベーチェット病と神経スウィート病

久永 欣哉

国立病院機構宮城病院神経内科〔〒989―2202 宮城県亘理郡山元町高瀬字合戦原100〕

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.
Full Text of this Article in Japanese PDF (242K)

(臨床神経, 52:1234−1236, 2012)
key words:神経ベーチェット病,神経スウィート病,ヒト白血球型抗原,細胞障害性Tリンパ球

(受付日:2012年5月25日)