Motor problems in Prader-Willi syndrome: A systematic review on body composition and neuromuscular functioning

NEUROSCIENCE AND BIOBEHAVIORAL REVIEWS

卷:35

Review

Reus, Linda¹  Zwarts, Machiel²  van Vlimmeren, Leo A.¹  Willemsen, Michel A.³  Otten, Barto J.⁴  Nijhuis-van der Sanden, Maria W. G.⁵ 

[1] Radboud Univ Nijmegen, Med Ctr, Sci Inst Qual Hlth Care, Dept Pediat 818, NL-6500 HB Nijmegen, Netherlands

[2] Radboud Univ Nijmegen, Med Ctr, Dept Clin Neurophysiol 920, NL-6500 HB Nijmegen, Netherlands

[3] Radboud Univ Nijmegen, Med Ctr, Dept Pediat Neurol 818, NL-6500 HB Nijmegen, Netherlands

[4] Radboud Univ Nijmegen, Med Ctr, Dept Pediat Endocrinol 842, NL-6500 HB Nijmegen, Netherlands

[5] Radboud Univ Nijmegen, Med Ctr, Sci Inst Qual Hlth Care, Dept Pediat, NL-6500 HB Nijmegen, Netherlands

关键词: Prader-Willi syndrome;    Body composition;    Growth hormone;    Muscle mass;    Systematic review;   

DOI  :  10.1016/j.neubiorev.2010.10.015

来源: Elsevier

【 摘 要 】

Motor problems in Prader-Willi syndrome (PWS) are presumably related to abnormal body composition and certain neuromuscular abnormalities. The authors reviewed the literature to evaluate the extent to which body composition is affected and gathered all findings on neuromuscular functioning in PWS. A systematic review was conducted in four databases (1956-2010). The methodological quality of each included article was evaluated. Thirty-eight papers were included: body composition (9 studies), neuromuscular functioning (7) and growth hormone (GH) effect studies (23). Increased fat mass and decreased lean body mass are characteristics of PWS. As a result, muscle mass is decreased by 25-37%, which might explain partly the weakness and hypotonia. However, there are also structural and functional muscle abnormalities, and cortical motor areas are hypo-excitable in PWS patients. Moreover, disuse as result of decreased activity in PWS could also contribute. GH treatment positively influences body composition, but does not normalize it. Training could prevent disuse and improves body composition. Therefore GH treatment and training will probably enhance one another. (C) 2010 Elsevier Ltd. All rights reserved.

【 授权许可】

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