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(P3-1-15) 眼症状と同時期に発症した神経ベーチェット病の1例を経験した。症例は27歳,女性。陰部潰瘍,結節性紅斑,口腔アフタ性潰瘍,虹彩毛様体炎がみられ,完全型ベーチェット病と診断した。経過中,視力右(0.6)と低下し,両虹彩炎の増悪と右眼硝子体出血,左眼底出血,血管炎を生じた。翌日,記銘力と見当識障害を認め,髄液所見で細胞数,蛋白の増加,およびMRIのT2強調画像で右基底核に高信号域を認めた。神経ベーチェット病と診断し,プレドニン100mgから漸減療法を開始した。ステロイド大量全身投与により神経症状とともに眼症状の消退を認めたが,今後、重篤な眼発作を起こす可能性もあり,慎重な経過観察が必要と思われた。
A 27-year-old female presented with bilateral iridocyclitis. She had also genital ulcer, erythema nodosum and aphthous ulcer since 3 months before. We diagnosed her as complete type of Behçet disease. She developed later aggravation of iridocyclitis in both eyes, vitreous hemorrhage in the right eye, and retinal vasculitis with hemorrhage in the left eye. She also developed dementia and amnesia compatible with neuro-Behçet disease. The cerebrospinal fluid showed pleocytosis. Magnetic resonance imaging (MRI) showed high-intensity area in the right basal ganglia. The ocular and neurological symptoms improved following systemic treatment with colchicine 0.5 mg daily and oral prednisolone starting with 100 mg daily. This case illustrates that active ocular lesions of Behçet disease may be associated with neurological involvement.
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