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Newcastle Mitochondrial Disease Adult Scale (NMDAS)
Availability
Please visit this website for more information about the instrument: The Newcastle Mitochondrial Disease Adult Scale
Classification
Supplemental - Highly Recommended: Mitochondrial Disease (Mito)
Recommendations for use: Indicated for studies measuring progression of mitochondrial disease in patients over 16 years.
Short Description of Instrument
The NMDAS evaluates progression of mitochondrial disease in patients over 16 years. The Newcastle Mitochondrial Disease Paediatric Scale (NMDPS) provides a similar assessment tool for pediatric patients. Repeated administration of the scale provides a quantitative assessment in the longitudinal follow up of patients with mitochondrial disease. The use of the NMDAS will standardize patient assessment and ensure more accurate data collection to aid understanding of the natural history of mitochondrial disease.
 
The rating scale encompasses several domains: Current Function; System Specific Involvement; Current Clinical Assessment and Quality of Life.
 
The assessment can be performed via a caregiver in patients who are not themselves capable of providing information. The tool appears best suited to patients in whom disease affects several systems and progression in one or more can vary.
 
This instrument can be applied to all types of mitochondrial disease. Single organ diseases, such as Leber hereditary optic neuropathy (LHON), may be least suited, but even here the scale covers the essential requirements for following disease progression. Neurological diseases are probably best suited.
 
It has been validated in Mitochondrial Encephalopathy, Lactic Acidosis, and Stroke-like episodes (MELAS), single deletion disorders amongst others.
Comments/Special Instructions
This assessment scale appears to have been created specifically as a tool to follow disease progression. It is broad, possibly too broad, but has been validated and is widely used. It is designed to cover the systemic nature of mitochondrial disease so does not go into the same detail as do some of the more specific tools (e.g., SARA cerebellar scales). It includes quality of life questions and self-reported psychiatric evaluation.
 
There is a detailed manual available online along with video teaching tools with instructions on how to score, and strict adherence to the rules for scoring is required if the tool is to function properly. This scale was developed by and for neurologists and is probably best suited for diseases in which neurological dysfunction is an integral part. It does, however, cover all systems.
 
Administration: The instrument developers recommend that it be administered by clinicians at 6 or 12-month intervals. They further recommend that the administering clinicians be experienced in caring for persons who have mitochondrial disease.
Scoring and Psychometric Properties
Scoring: Items in the NMDAS are scored from 0 to 5. The total score for each of the first 3 sections is the sum of all item scores in the section. Higher scores indicate greater severity.
 
The Quality of Life section is scored separately; details are provided in the SF manual. It is recommended that all section scores be reported individually rather than reporting a single total score for the instrument. At a minimum, the Quality of Life score from the SF-12 v2 should be reported separately from the score of Sections 1 through 3 of this instrument (regardless of whether the latter are reported individually or as a single aggregate score).
 
Psychometric Properties: The Newcastle Mitochondrial Disease Research Group has validated this instrument twice, once by administration to 16 participants and once by administration to 15 participants. In both cases four clinicians administered the instrument. In both cases, between-rater agreement for individual domain scores and for the overall disease score was good to excellent. It must be emphasized that achieving good between-rater agreement depends on close adherence to the instructions by all clinicians. Idiosyncratic evaluations of the items on the instrument will negatively affect data consistency and reliability.
Rationale/Justification
Strengths: Appears robust for following multisystemic diseases that are typical of mitochondrial dysfunction.
 
Weaknesses: The scoring requires normal level of neurological competence to perform some of the clinical evaluations. This instrument is not designed for short term clinical trials as the questionnaires are designed to be administered at 6- and 12-month intervals. The Current Function section assesses the patient or caregiver responses in the previous 4 weeks and the System Specific Involvement section assesses the previous 12 months.
References
Key Reference:
Schaefer AM, Phoenix C, Elson JL, McFarland R, Chinnery PF, Turnbull DM. Mitochondrial disease in adults: a scale to monitor progression and treatment. Neurology. 2006 Jun 27;66(12):1932-4.
 
Additional References:
Bates MG, Hollingsworth KG, Newman JH, Jakovljevic DG, Blamire AM, MacGowan GA, Keavney BD, Chinnery PF, Turnbull DM, Taylor RW, Trenell MI, Gorman GS. Concentric hypertrophic remodelling and subendocardial dysfunction in mitochondrial DNA point mutation carriers. Eur Heart J Cardiovasc Imaging. 2013 Jul;14(7):650-8.
 
de Laat P, Koene S, van den Heuvel LP, Rodenburg RJ, Janssen MC, Smeitink JA. Clinical features and heteroplasmy in blood, urine and saliva in 34 Dutch families carrying the m.3243A > G mutation. J Inherit Metab Dis. 2012 Nov;35(6):1059-69.
 
de Laat P, Smeitink JA, Janssen MC, Keunen JE, Boon CJ. Mitochondrial retinal dystrophy associated with the m.3243A>G mutation. Ophthalmology. 2013 Dec;120(12):2684-96.
 
Grady JP, Campbell G, Ratnaike T, Blakely EL, Falkous G, Nesbitt V, Schaefer AM, McNally RJ, Gorman GS, Taylor RW, Turnbull DM, McFarland R. Disease progression in patients with single, large-scale mitochondrial DNA deletions. Brain. 2014 Feb;137(Pt 2):323-34.
 
Mancuso M, Orsucci D, Ienco EC, Pini E, Choub A, Siciliano G. Psychiatric involvement in adult patients with mitochondrial disease. Neurol Sci. 2013 Jan;34(1):71-4.
 
Nesbitt V, Pitceathly RD, Turnbull DM, Taylor RW, Sweeney MG, Mudanohwo EE, Rahman S, Hanna MG, McFarland R. The UK MRC Mitochondrial Disease Patient Cohort Study: clinical phenotypes associated with the m.3243A>G mutation--implications for diagnosis and management. J Neurol Neurosurg Psychiatry. 2013 Aug;84(8):936-8.
 
Newcastle Mitochondrial Disease Adult Scale Manual. Available from: from https://www.newcastle-mitochondria.com/wp-content/uploads/2016/03/NMDAS-Scale-neurology-2006-261114.pdf. Accessed 26 October 2022
 
Document last updated March 2024