Longitudinal Course of Long Finger Flexor Shortening in Males with Duchenne Muscular Dystrophy: A Retrospective Review1.

IF 3.2 4区 医学 Q2 CLINICAL NEUROLOGY
Saskia L S Houwen-van Opstal, Menno van der Holst, Michel A A P Willemsen, Erik H Niks, Imelda J M De Groot, Edith H C Cup
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Abstract

Background: Shortening of the long finger flexors (Flexor Digitorum Profundus, FDPs) in Duchenne Muscular Dystrophy (DMD) causes reduced hand function. Until now, longitudinal studies on the natural course of the shortening of the FDPs are lacking, which impedes recommendations on timing and evaluation of preventive measures.

Objective: To investigate the longitudinal course of the FDP length during different disease stages focusing on symmetry, timing, and decline of the FDP length.

Methods: A retrospective, longitudinal multicenter study was conducted in the Radboud university medical center and the Leiden university medical center. The FDP outcome was measured using goniometry and gross motor function was assessed using the Brooke score. Longitudinal mixed model analyses were used to describe the course of the FDP outcome, and to investigate symmetry in both hands.

Results: Data on 534 visits of 197 males (age ranged 4-48 years) showed that in the ambulatory stages the FDP outcome was within a normal range. The mean decline in FDP outcome is 3.5 degrees per year, the biggest decline was seen in Brooke 5 (>15 degrees per year). In Brooke 4, 41% of the FDP outcome was < 40 degrees. No significant differences were found between right and left.

Conclusions: This study supports the consideration of preventive measures to delay shortening of the FDPs in DMD patients transitioning to a Brooke scale of 4 or higher. Besides, natural history of FDP outcome has been established, which provides a base to evaluate (preventive) interventions.

Duchenne肌营养不良男性长指屈肌缩短的纵向过程:回顾性综述†。
背景:Duchenne肌营养不良(DMD)患者长指屈肌(Flexor Digitorum Profundus,FDPs)缩短会导致手部功能下降。到目前为止,缺乏关于缩短FDP的自然过程的纵向研究,这阻碍了关于预防措施的时间安排和评估的建议。目的:从FDP长度的对称性、时间性和下降性等方面探讨不同疾病阶段FDP长度纵向变化规律。方法:在拉德布大学医学中心和莱顿大学医学中心进行回顾性纵向多中心研究。FDP结果采用角度测量法进行测量,总运动功能采用Brooke评分进行评估。纵向混合模型分析用于描述FDP结果的过程,并研究双手的对称性。结果:197名男性(年龄4-48岁)的534次就诊数据显示,在门诊阶段,FDP结果在正常范围内。FDP结果的平均下降幅度为每年3.5度,其中Brooke 5的下降幅度最大(每年>15度)。在Brooke 4中,41%的FDP结果为 <  40度。左右两侧无显著差异。结论:本研究支持考虑采取预防措施,以延迟DMD患者的FDPs缩短,这些患者的Brooke评分为4或更高。此外,建立了FDP结果的自然史,为评估(预防性)干预措施提供了基础。
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来源期刊
Journal of neuromuscular diseases
Journal of neuromuscular diseases Medicine-Neurology (clinical)
CiteScore
5.10
自引率
6.10%
发文量
102
期刊介绍: The Journal of Neuromuscular Diseases aims to facilitate progress in understanding the molecular genetics/correlates, pathogenesis, pharmacology, diagnosis and treatment of acquired and genetic neuromuscular diseases (including muscular dystrophy, myasthenia gravis, spinal muscular atrophy, neuropathies, myopathies, myotonias and myositis). The journal publishes research reports, reviews, short communications, letters-to-the-editor, and will consider research that has negative findings. The journal is dedicated to providing an open forum for original research in basic science, translational and clinical research that will improve our fundamental understanding and lead to effective treatments of neuromuscular diseases.
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