Oral functions in adult persons with spinal muscular atrophy compared to a healthy control group: a prospective cross-sectional study with a multimodal approach.
Teresa Kruse, Diana Leflerovà, Annette Cap, Sara Portegys, Brunhilde Wirth, Raoul Heller, Svenja Brakemeier, Tim Hagenacker, Bert Braumann, Gilbert Wunderlich
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引用次数: 0
Abstract
Background: Oral function tests have been shown to reliably detect impaired bulbar function in adults with spinal muscular atrophy (SMA). Although not routinely recorded, it is known that persons with SMA are affected to varying degrees. Detecting differences in bite and tongue force, endurance, and maximum mouth opening has become particularly promising since the introduction of causal therapy for SMA. This study aimed to compare oral function among adult persons with SMA with different SMA types, walking abilities, and treatment status to a healthy control group.
Methods: Data from oral function tests conducted on 58 persons with SMA and 45 healthy individuals were analyzed. Differences in oral function between SMA subgroups were pairwise tested and compared to the healthy control group using Wilcoxon rank sum tests.
Results: In an overall comparison, three out of five oral function tests revealed lower values for the SMA group compared to the control group. Subgroup analyses indicated lower scores for most oral function tests in non-ambulatory, untreated patients with SMA type 2 compared to controls. Ambulatory, treated patients with SMA type 3 achieved strength and endurance values comparable to those of healthy individuals.
Conclusions: The impairment of oral function varies across persons with SMA. Routine measurement of oral function is warranted to determine individual bulbar involvement stages. Further evaluation should be scheduled if indicators such as restricted maximum mouth opening arise. Trial registration DRKS, DRKS00015842. Registered 30 July 2019, https://drks.de/register/de/trial/DRKS00015842/preview .
背景：口腔功能测试已被证明能可靠地检测出患有脊髓性肌萎缩症（SMA）的成人的球部功能受损情况。虽然没有常规记录，但已知 SMA 患者受到不同程度的影响。自从对 SMA 采用因果疗法以来，检测咬合力、舌力、耐力和最大张口度的差异变得尤为重要。本研究旨在比较不同 SMA 类型、行走能力和治疗状况的成年 SMA 患者与健康对照组的口腔功能：分析了对 58 名 SMA 患者和 45 名健康人进行的口腔功能测试数据。采用 Wilcoxon 秩和检验对 SMA 亚组之间的口腔功能差异进行配对检验，并与健康对照组进行比较：在总体比较中，五项口腔功能测试中有三项显示 SMA 组的数值低于对照组。分组分析表明，与对照组相比，不活动、未接受治疗的 2 型 SMA 患者的大多数口腔功能测试得分较低。接受过治疗的 3 型 SMA 患者的力量和耐力值与健康人相当：结论：SMA 患者的口腔功能受损情况各不相同。有必要对口腔功能进行常规测量，以确定每个人的球部受累阶段。如果出现最大张口受限等指标，应安排进一步评估。试验注册 DRKS，DRKS00015842。2019年7月30日注册，https://drks.de/register/de/trial/DRKS00015842/preview 。
期刊介绍：
Orphanet Journal of Rare Diseases is an open access, peer-reviewed journal that encompasses all aspects of rare diseases and orphan drugs. The journal publishes high-quality reviews on specific rare diseases. In addition, the journal may consider articles on clinical trial outcome reports, either positive or negative, and articles on public health issues in the field of rare diseases and orphan drugs. The journal does not accept case reports.
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