Examination of Motor Function and Changes Over Time in Patients with Fukuyama Congenital Muscular Dystrophy

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  • ADACHI Michiru
    Department of Rehabilitation, Tokyo Women's Medical University
  • SATO Takatoshi
    Department of Pediatrics, School of Medicine, Tokyo Women's Medical University
  • ADACHI Taku
    Cardiac Rehabilitation Room, Tokyo Women's Medical University, Yachiyo Medical Center
  • GOTO Keisuke
    Department of Rehabilitation, Tokyo Women's Medical University, Medical Center East
  • ZUSHI Masaya
    Department of Rehabilitation, Tokyo Women's Medical University
  • NAKAMURA Kaho
    Department of Rehabilitation, Tokyo Women's Medical University
  • WADA Futoshi
    Department of Rehabilitation Medicine, School of Medicine, Tokyo Women's Medical University
  • WAKABAYASHI Hidetaka
    Department of Rehabilitation Medicine, School of Medicine, Tokyo Women's Medical University
  • ISHIGURO Kumiko
    Department of Pediatrics, School of Medicine, Tokyo Women's Medical University
  • SHICHIJI Minobu
    Department of Pediatrics, School of Medicine, Tokyo Women's Medical University
  • MURAKAMI Terumi
    Department of Pediatrics, School of Medicine, Tokyo Women's Medical University
  • KONDO Izumi
    National Center for Geriatric Medicine and Gerontology
  • NAGATA Satoru
    Department of Pediatrics, School of Medicine, Tokyo Women's Medical University
  • ISHIGAKI Keiko
    Department of Pediatrics, School of Medicine, Tokyo Women's Medical University

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Other Title
  • 福山型先天性筋ジストロフィー患者における運動能力の経時的変化

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Abstract

<p>Objectives: We examined changes in motor function over time in Fukuyama congenital muscular dystrophy (FCMD) patients.</p><p>Methods: The subjects were 57 FCMD patients. Motor functions were evaluated using the gross motor function measure (GMFM). The subjects were grouped according to genotype, and GMFM score transitions were observed for each individual. We determined each GMFM score monthly and performed a regression analysis for all data obtained, using a least-squares method. The age at peak GMFM score (peak age) was also studied based on regression analysis results for all cases and for each clinical severity.</p><p>Results: The monthly course of GMFM scores showed a biphasic pattern comprised of a rapidly increasing phase, followed by a slowly decreasing phase. According to genotypes, the range of GMFM scores was greater in compound heterozygotes than in homozygotes. The peak motor function ages of severe, typical and mild cases, based on an approximated graph for each, were 20, 48 and 68 months, respectively. The peak GMFM score in typical cases was 28 for homozygous and 18 for compound heterozygous cases.</p><p>Conclusion: Changes in FCMD motor function over time showed a rapidly increasing phase, followed by a slowly decreasing phase. The peak ages, in the approximated graphs, differed according to clinical severity. Among typical cases, compound heterozygotes tended to have poorer motor skills than homozygotes.</p>

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