経過中に顕性化した特発性頭蓋内圧亢進症による乳頭腫脹

Acute onset of papilledema in a case of idiopathic intracranial hypertension

要約　目的：乳頭腫脹が顕性化する前から経過を観察し，薬物治療後にその所見が軽快した特発性頭蓋内圧亢進症の1例の報告。

症例：15歳女性が頭痛の精査のため脳神経内科から紹介受診した。2年前から左眼に中心暗点があり，視神経炎と診断された。拍動性頭痛が生じ，慢性化した状態にあった。父と姉に頭痛，妹に頭痛とアトピー性皮膚炎があった。

所見と経過：矯正視力は右0.2，左0.3で，乳頭腫脹はなかった。髄液の成分と圧は正常範囲にあった。MRIを含む全身検査で異常はなかった。21歳になってから両眼の乳頭が急激に腫脹し，左右眼とも視力が0.1以下になった。髄液圧の亢進があり，特発性頭蓋内圧亢進症と診断された。肥満に対する食事療法とアセタゾラミド内服を行い，乳頭腫脹は軽快し，右0.07，左0.15の矯正視力を得た。

結論：本症例では，原因不明の頭痛が発症してから8年後に乳頭腫脹が顕性化し，特発性頭蓋内圧亢進症と診断された。

Abstract　Purpose: To report a case of idiopathic intracranial hypertension who manifested papilledema during the course of observation for seven years.

Case: A 15-year-old female was referred to us for headache. She had had central scotoma in the left eye with pulsating headache since 2 years before. Her father and two sisters had been suffering from headache.

Findings and Clinical Course: Corrected visual acuity was 0.2 right and 0.3 left. Fundus findings were apparently normal. Findings of cerebrospinal fluid were within normal range regarding its component and pressure. Findings by magnetic resonance imaging were uneventful. Both eyes started to show manifest papilledema at the age of 21 years. Visual acuity decreased below 0.1 in either eye. The cerebrospinal fluid showed elevated pressure, leading to the diagnosis of idiopathic intracranial hypertension. Papilledema subsided following diet for obesity and peroral acetazolamide, with visual acuity of 0.07 right and 0.15 left.

Conclusion: Papilledema became manifest 8 years after headache as initial manifestation of idiopathic intracranial hypertension in the present patient.