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International Pediatric Mitochondrial Disease Score (IPMDS)
Please visit this website for more information about the instrument: International Paediatric Mitochondrial Disease Scale
Supplemental: Mitochondrial Disease (Mito)
Short Description of Instrument
The instrument was designed to measure general disease severity in the whole spectrum of children (0 - 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
Administration: about 30 minutes, by a trained physician (history taking, physical examination and functional abilities)
Scoring and Psychometric Properties
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).
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.
Psychometric Properties: Good construct validity was found using hypothesis testing. The overall interrater reliability was good [median intraclass correlation coefficient for agreement between raters (ICC agreement: 0.85; range 0.23-0.99). (Koene et al, 2016)
Validations studies testing feasibility, inter- and intra-rater reliability, test-retest reliability and validity are currently running.
Strengths: Generally applicable, measures patient important symptoms and functions, may be sensitive to change.
Weaknesses: Time consuming, although the exercises are fun to perform it may be a burden to the patient, dependent on patient cooperation (functional abilities)
Key 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.

Koene S. Erratum to: International Paediatric Mitochondrial Disease Scale. J Inherit Metab Dis. 2017 May;40(3):463. doi: 10.1007/s10545-017-0024-8. Erratum for: J Inherit Metab Dis. 2016 Sep;39(5):705-12.

Additional Reference:
van de Loo KFE, Custers JAE, de Boer L, van Lieshout M, de Vries MC, Janssen MCH, Verhaak CM. Cognitive functioning and mental health in children with a primary mitochondrial disease. Orphanet J Rare Dis. 2022 Oct 1;17(1):368.
Document last updated March 2024