Peak Jump Power Reflects the Degree of Ambulatory Ability in Patients with Mitochondrial and Other Rare Diseases

  • Christopher Newell
  • Barbara Ramage
  • Alberto Nettel-Aguirre
  • Ion Robu
  • Aneal Khan
Research Report
Part of the JIMD Reports book series (JIMD, volume 33)

Abstract

Metabolic diseases that lead to neuromuscular, bone, and joint involvement can reduce ambulation and quality of life. Using jumping mechanography, we developed a novel assessment, peak jump power (PJP), and related this to ambulatory ability in patients either having a known or suspected underlying rare disease. From adults and children, we recruited 88 healthy controls and 115 patients (61 with mitochondrial disease and 54 with another diagnosis). Patients were categorized as having no complaints of weakness or ambulation (ambulatory competent; AC), weakness but able to ambulate without aids (ambulatory weakness; AW), or not able to ambulate without aids such as a walker, cane, or wheelchair (ambulatory assistance; AA). Subjects were asked to perform five successive jumps from a squat position. Instantaneous power (W; watts) was calculated and the highest result was divided by the body mass (kg) to calculate PJP (W/kg). Between healthy controls and AC patients, there was no difference in mean PJP (20.5 ± 7.0 W/kg vs. 19.0 ± 7.4 W/kg, p = 0.601; mean ± SD). Progressively lower results were found in patients with AW with a mean PJP of 11.7 ± 5.1 W/kg (p < 0.001 versus AC) and further those with AA with a mean PJP of 5.8 ± 3.2 W/kg (p < 0.001 versus AW). A subgroup analysis of subjects showed that those who did not use ambulatory aids all had a PJP above 10 W/kg. Using this threshold, the receiver operating characteristic curve (ROC) analysis showed PJP to be highly sensitive evaluation of ambulatory ability (sensitivity 95.8%, specificity 52.1%).

Keywords

6-Minute walk test Ambulation Disability Jumping force Jumping power Lysosomal Mitochondrial 

Abbreviations

6MWT

Six-minute walk test

ANOVA

Analysis of variance

AUROC

Area under the ROC curve

DCMA

Dilated cardiomyopathy with ataxia

DMD

Duchene muscular dystrophy

F

Force

FSHD

Facioscapulohumeral muscular dystrophy

GSD

Glycogen storage disorder type V or VII

MPS-I or II

Mucopolysaccharide storage disease

MRC

Medical Research Council

MS

Multiple sclerosis

P

Power

PJP

Peak jump power

ROC

Receiver operating characteristic curve

SD

Standard deviation

SSC

Stretch shortening cycle

V

Velocity

W

Watts

Notes

Acknowledgments

This research was supported by the following: Alberta Health Services, Riddell Movement Assessment Centre, and Alberta Children’s Hospital for the use of facilities. Funding support was provided through the Alberta Children’s Hospital Foundation. The authors would also like to thank Shelly Jelinksi, Connie Mohan, and Laurel Ryan for research support and the Metabolic Clinic (Karin Klassen, Sheryl Jackson, Karen Sabo, Patricia Moar, Deanne Durand) for logistical support. Chris Newell was supported by a grant from MitoCanada. Fariha Ahmed helped with recruitment of healthy controls.

Supplementary material

978-3-662-55012-0_583_MOESM1_ESM.docx (13 kb)
Table S1 Prevalence of neuromuscular patient diagnoses (DOCX 13 kb)

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Copyright information

© SSIEM and Springer-Verlag Berlin Heidelberg 2016

Authors and Affiliations

  • Christopher Newell
    • 1
  • Barbara Ramage
    • 2
  • Alberto Nettel-Aguirre
    • 3
  • Ion Robu
    • 2
  • Aneal Khan
    • 4
  1. 1.Department of Medical ScienceCumming School of Medicine, University of CalgaryCalgaryCanada
  2. 2.Department of NeurosciencesCumming School of Medicine, University of CalgaryCalgaryCanada
  3. 3.Departments of Pediatrics and Community Health SciencesCumming School of Medicine, University of CalgaryCalgaryCanada
  4. 4.Department of Medical Genetics and PediatricsCumming School of Medicine, University of Calgary, Alberta Children’s HospitalCalgaryCanada

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