of0
Export
NINDS CDE Notice of Copyright
International Pediatric Mitochondrial Disease Score (IPMDS)
Availability
Freely available as soon as the instrument has been validated
Classification
Exploratory for pediatric Mitochondrial Disease
Short Description of Instrument
The instrument was designed to measure general disease severity in the whole spectrum of children (? – 18 years) with mitochondrial disease. The IPMDS consists of 3 domains:
 
  1. Complaints and symptoms (23 questions)
  2. Physical examination (21 items)
  3. Functional abilities (13 items = 6 years of age; 10 < 6 years of age)
 
Comments/Special Instructions
Scoring: The score is calculated by dividing the patient’s score by the maximal score for that patient (some items may be left out for several reasons).
 
In order to familiarize examiners with the utilization of the IPMDS and its scoring principles, a training session, in which the items are practiced in a in a roleplay at least twice, is recommended. There is also an instructional video.
 
In order to familiarize examiners with the utilization of the IPMDS and its scoring principles, a training session, in which the items are practiced in a in a roleplay at least twice, is recommended. There is also an instructional video.
 
Administration: about 30 minutes, by a trained physician (history taking, physical examination and functional abilities)
Rationale/Justification
Strengths/Weaknesses:
Limitations for use in mitochondrial disease patients: Time consuming, although the exercises are fun to perform it may be a burden to the patient, dependent on patient cooperation (functional abilities)
 
Advantages for use in mitochondrial disease patients: Generally applicable, measures patient important symptoms and functions, may be sensitive to change.
 
Validations studies testing feasibility, inter- and intrarater reliability, test-retest reliability and validity are currently running.
 
This instrument has not yet been used, but it has been tested in children aged 0- 18 years.
References
Koene S, Hendriks JCM, Dirks I, de Boer L, de Vries MC, Janssen MCH, Smuts I, Fung CW, Wong VCN, de Coo IRFM, Vill K, Stendel C, Klopstock T, Falk MJ, McCormick EM, McFarland R, de Groot IJM, Smeitink JAM. International Paediatric Mitochondrial Disease Scale. J Inherit Metab Dis. 2016 Sep;39(5):705-712. doi: 10.1007/s10545-016-9948-7.
Page 1 of 1