Parsonage-Turner综合征：肺科医生对遗忘膈肌的指南

文献信息

中文摘要

目的回顾： Parsonage-Turner综合征（PTS），即神经痛性肌萎缩，被框定为神经科拥有的肩部疾病，而这种框定使肺科医生失去了患者。大约每13人中就有1人发生膈神经病，许多因不明原因呼吸困难、端坐呼吸或快速眼动（REM）为主睡眠呼吸障碍而被转诊的患者，患有未诊断、可治疗的炎性神经病。本综述为肺科医生重新框定PTS。
近期发现： 三项进展重塑了实践。高分辨率神经肌肉超声和MR神经成像现在可在床旁识别PTS的沙漏样狭窄和束状缠绕，包括在膈神经中。COVID-19感染和SARS-CoV-2疫苗接种产生了全球聚集，将表型扩展为双侧和呼吸为主疾病。大型荷兰队列数据用结构取代了轶事：大约60%的膈肌受累患者在2年内有意义的恢复，而其余患者对早期无创通气反应最佳，难治性病例可行膈肌折叠术。
总结： 每位评估不明原因呼吸困难、不成比例端坐呼吸或REM为主低通气的肺科医生都应问一个问题：在呼吸改变前几周是否有严重的肩部或颈部疼痛？如果有，用直立和仰卧肺量计及膈肌超声筛查。诊断可在一次就诊中达到，免疫调节窗口很短，漏诊的代价是数年可避免的残疾。

英文摘要

PURPOSE OF REVIEW: Parsonage-Turner syndrome (PTS), or neuralgic amyotrophy, is framed as a shoulder disorder owned by neurology, and that framing has cost pulmonologists patients. Roughly 1 in 13 develops phrenic neuropathy, and many patients referred for unexplained dyspnea, orthopnea, or rapid eye movement (REM)-predominant sleep-disordered breathing have an undiagnosed, treatable inflammatory neuropathy. This review reframes PTS for pulmonologists.
RECENT FINDINGS: Three developments have reshaped practice. High-resolution neuromuscular ultrasound and MR neurography now identify the hourglass constrictions and fascicular entwinement of PTS at the bedside, including in the phrenic nerve. COVID-19 infection and SARS-CoV-2 vaccination produced a global cluster, expanding the phenotype toward bilateral and respiratory-predominant disease. Large Dutch cohort data have replaced anecdote with structure: roughly 60% of patients with phrenic involvement recover meaningfully within 2 years, while the remainder respond best to early noninvasive ventilation and, in refractory cases, diaphragmatic plication.
SUMMARY: Every pulmonologist evaluating unexplained dyspnea, disproportionate orthopnea, or REM-predominant hypopneas should ask one question: was there severe shoulder or neck pain in the weeks before the breathing changed? If yes, screen with upright and supine spirometry and diaphragm ultrasound. The diagnosis is reachable in a single visit, the immunomodulation window is short, and the cost of missing it is years of avoidable disability.