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両側性孤立性句膜神経障害：呼吸困難の稀な原因
Bilateral Isolated Phrenic Neuropathy: a Rare Cause of Dyspnoea.
PMID: 32670989 PMCID: PMC7080215. DOI: 10.12890/2020_001258.
抄録
両側性孤立性フレニック神経症（BIPN）による横隔膜麻痺は，原因不明の呼吸不全の非常に稀な原因である．症例は65歳で既往歴はなく，mMRC1 dyspnoeaとOrthopnoeaを呈して呼吸器内科を受診した．診察の結果，横隔膜麻痺と診断された．吸引筋トレーニングにより軽度の症状改善がみられ，非侵襲的機械換気（NIV）による治療が開始された．この病態は一般的に慢性化しており、他の句膜神経病変の症例と比較して予後が悪い。この症例では、NIVによりほぼ正常な日常生活機能が回復しました。
Bilateral diaphragm paralysis due to bilateral isolated phrenic neuropathy (BIPN) is a very rare cause of unexplained respiratory failure. We present a 65-year-old patient with no relevant previous medical history who presented in the Pulmonology Clinic with mMRC1 dyspnoea and orthopnoea. After the medical work-up, diaphragmatic paresis was diagnosed. Inspiratory muscle training resulted in mild symptomatic improvement and treatment with noninvasive mechanical ventilation (NIV) was initiated. This condition is generally chronic and has a poorer prognosis, compared to other cases of phrenic nerve involvement. In this case, NIV restored near-normal daily function.
学習ポイント:
非侵襲的な機械的人工呼吸が症状のある患者の治療の主役である。
LEARNING POINTS: Besides being a rare cause of dyspnoea, diaphragmatic dysfunction should be considered in the medical evaluation.Noninvasive mechanical ventilation is the mainstay of treatment for symptomatic patients.Neurologic and electrodiagnostic evaluation is essential, since the differential diagnosis of phrenic nerve dysfunction may imply different treatment strategies.
© EFIM 2020.