Respiratory Function Changes as Early Signs of Amyotrophic Lateral Sclerosis.
Respiratory Function Changes as Early Signs of Amyotrophic Lateral Sclerosis.
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呼吸功能变化是肌萎缩侧索硬化症的早期症状。
DOI：
10.1159/000533870
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发表时间：
2023
期刊：
影响因子：
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通讯作者：
Polverino,Francesca
中科院分区：
文献类型：
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作者：
Polverino,Mario;Sampaolo,Simone;Capuozzo,Antonio;Fasolino,Marco;Aliberti,Michele;Satta,Ersilia;Santoriello,Carlo;Orengo,JamesPeter;Polverino,Francesca
背景目前肌萎缩侧索硬化症 (ALS) 的诊断标准在疾病早期可能数月或数年仍不能令人满意。肺部评估从未被认为对 ALS 的早期诊断有用，并且缺乏针对此类患者的肺功能研究。目的本研究的目的是评估具有非特异性 ALS 症状的受试者的肺功能，这些受试者根据当前可用的指南无法达到 ALS 诊断。方法我们进行了肺功能测试、动脉气体分析、最大吸气 (MIP) 和 对 35 名在就诊时有非特异性神经系统症状的患者进行呼气 (MEP) 压力和呼吸动力 (P < 0.1) 评估，这些患者在初次就诊 2 年后被诊断患有 ALS（“ALS 前期”）；我们将这些患者与 29 名已确诊 ALS 的患者以及 28 名对照受试者进行了比较。结果三组之间的肺活量参数没有差异。然而，与对照组相比，已确诊的 ALS 患者和预 ALS 患者的 MIP 均显着降低，P 0.1 显着升高（比值 P 0.1/MIP 显着更高），而已确诊的 ALS 和预 ALS 之间的 MIP 和 P 0.1 相似。 结论 当肺量测定和神经诊断测试仍处于临床前阶段时，MIP、P 0.1 和 P 0.1/MIP 比值的变化高度提示临床前 ALS。 尚无定论。 MIP 和 P 0.1 是无创测量，可以在门诊环境中轻松评估。未来需要对更大的队列进行研究，以验证这些参数在 ALS 以及其他神经肌肉疾病的临床前诊断中的使用。
BackgroundThe current diagnostic criteria for amyotrophic lateral sclerosis (ALS) may remain unsatisfactory for months or years in the early disease. Pulmonary assessment has never been considered useful in the early diagnosis of ALS, and studies of pulmonary function in this patient category are lacking.ObjectivesThe objective of this study was to assess the pulmonary function in subjects with unspecific symptoms of ALS in whom an ALS diagnosis cannot be reached based on the current available guidelines.MethodsWe performed pulmonary function tests, arterial gas analysis, maximal inspiratory (MIP) and expiratory (MEP) pressure, and respiratory drive (P 0.1) assessment in 35 patients with unspecific neurological symptoms at the time of the visit and those were subsequently diagnosed with ALS 2 years after the initial visit (“pre-ALS”); we compared these patients with 29 patients with established ALS and with 28 control subjects.ResultsSpirometric parameters were not different between the three groups. However, MIP was significantly lower and P 0.1 was significantly increased (with the ratio P 0.1/MIP significantly higher) in both established and pre-ALS patients compared to controls, while both MIP and P 0.1 were similar between established ALS and pre-ALS.ConclusionsChanges in MIP, P 0.1, and P 0.1/MIP ratio are highly suggestive of preclinical ALS when the spirometry and neurodiagnostic tests are still inconclusive. MIP and P 0.1 are noninvasive measurements that can be easily assessed in an ambulatory setting. Future studies on larger cohorts are needed to validate the use of these parameters in the preclinical diagnosis of ALS as well as in other neuromuscular diseases.