多系統萎縮症における突然死は気管切開では防げない - Neurology 興味を持った「脳神経内科」論文
本邦より多系統萎縮症（multiple system atrophy; MSA）における突然死の頻度や特徴，予防的治療の効果についての検討が報告された．対象はGilman分類におけるprobable MSA患者47名であり，5年間の経過観察が行われた．予防的治療は，①睡眠中の高度の低酸素血症（CT90>10%；検査中にSpO2が90％を下回る割合が10%を超す），②声帯外転麻痺，③繰り返す誤嚥性肺炎を認める場合に行っている．具体的な方法としては，①②に対しては非侵襲的陽圧換気療法（NPPV）を行い，③に対しては気管切開術を行っている．最終的に，上記の基準を満たし，同意を得られた25名に対し予防的治療が行われた． 結果としては，NPPV は適切な圧設定により，いびきや喉頭喘鳴をほぼ消失させ，さらに睡眠中の低酸素血症を改善することができた．死因に関する検討では，5年間の経過観察中の死亡者は10
The Heidenhain variant of Creutzfeldt-Jakob disease: clinical, pathologic, and neuroimaging findings - PubMed
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Neuroimaging findings in human prion disease - PubMed
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Quantitative cerebral blood flow imaging in a patient with the Heidenhain variant of Creutzfeldt-Jakob disease - PubMed
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Sporadic Creutzfeldt-Jakob disease: a comparison of pathological findings and diffusion weighted imaging - PubMed
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