視力障害と嚥下障害を併発した肥厚性硬膜炎の1例

Optic neuropathy and dysphagia as the initial manifestation in a case of hypertrophic pachymeningitis

要約　目的：視力障害と嚥下障害を初発症状とした肥厚性硬膜炎の症例の報告。症例：77歳男性が複視を主訴として受診した。矯正視力は左右眼とも1.2で，左眼の外斜視があった。そのほかに眼球運動を含めて異常所見はなく，頭部のMRIでも病的所見はなかった。その後左眼視力が徐々に低下し，2か月後には光覚弁になった。その翌日から嚥下障害と嗄声が出現した。頭部の造影MRIで大脳左半球の肥厚性硬膜炎が疑われ，ステロイドパルス療法を行った。嚥下障害は改善し，左眼視力は0.4になった。その後プレドニゾロンで後療法を行ったが，6か月後に感染性脳症が発症し，不帰の転帰をとった。結論：肥厚性硬膜炎が視力と嚥下障害を初発症状として発症することがある。本例では頭部の造影MRIが診断に有用であった。

Abstract.　Purpose：To report a case of hypertrophic pachymeningitis with optic neuropathy and dysphagia as the initial manifestation. Case：A 77-year-old male presented with diplopia as the chief complaint. Corrected visual acuity was 1.2 in either eye. Exotropia was present in the left eye. Ocular motility was apparently normal. Magnetic resonance imaging（MRI）of the brain showed normal findings. Visual acuity in the left eye deteriorated gradually and became light perception 2 months later. He developed dysphagia and hoarseness the following day. Contrast-enhanced MRI showed high-intensity area in the dura mater of left cerebral hemisphere, suggesting hypertrophic pachymeningitis. Steroid pulse therapy was followed by improvement of dysphagia with visual acuity of 0.4. Following maintenance therapy with peroral prednisolone, the patient died due to infectious encephalopathy 6 months later. Conclusion：The present case illustrates that optic neuropathy and dysphagia may develop as the initial manifestation of hypertrophic pachymeningitis and that contrast-enhanced MRI may facilitate the diagnosis.