二次性ファー症候群における両側大脳石灰化

Andreas Posa1, Stefan Schob1, Walter A Wohlgemuth1

  • 1University Clinic and Outpatient Clinic for Radiology, Martin Luther University Halle-Wittenberg, Halle, Germany, uni-halle.de.

PubMed

ファール症候群は、主に基底核に認められる両側性の脳石灰化を特徴とする、稀で緩徐に進行する神経変性疾患である。これらの脳石灰化はカルシウムとリン酸塩で構成されており、カルシウム・リン交代能(ホメオスタシス)の乱れによって生じる。臨床症状としては、神経学的、神経認知的な症状および精神医学的な症状が含まれる。本論文では、顕著な両側性脳石灰化を呈した3つの稀な症例について報告する。3例すべての患者が、初発のてんかん発作により入院した。全例において、臨床検査で著明な低カルシウム血症が認められ、頭部コンピューター断層撮影(CT)では、脳の様々な領域に顕著な両側性脳石灰化が認められた。カルシウム補充療法および抗てんかん薬による治療後、患者は入院前の状態に回復し、発作のない状態で退院した。本論文の目的は、薬物療法および栄養補充療法を通じて回避可能な神経学的・精神医学的合併症を防ぐため、ハイリスク患者(例:甲状腺切除術後など)における長期的な生化学的検査および頭蓋内画像診断の臨床的重要性を強調することである。

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