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Top 3 Tests for Pediatric Neuromuscular Diseases

Note that this article is part of a series of articles on assessment. If you have not done so, first, read this articles:


 

Q: What are the best tests for…Pediatric Neuromuscular Diseases?

 

 

Pediatric Neuromuscular Diseases include many diagnoses where problems with the nerves and muscles impact the way the children’s muscles work, such as Duchenne and Becker’s Muscular Dystrophy, Spinal Muscular Atrophy, Charcot-Marie Tooth Disease, congenital myopathies, and many others. Because each of these diagnoses has very specific presentations. it is critical that we use the right tests to capture their current function, and in some cases, predict their future needs.

 

Here are our 3 favorite tests, which are all downloadable for free:

 

1. North Star Ambulatory Assessment (NSAA)

 

 

The NSAA is a 17-tem scale that rates the performance of different functional skills. 

      • Best for: Duchenne Muscular Dystrophy
      • Test instruction and form: Download for free  
      • Why we like this test: 
        • For monitoring disease progression: it can help predict loss of ambulation, and therefore, future need for wheeled mobility so the family can order early
        • For measuring effect of new treatment approaches 
      • What to note: Use the Revised NSAA for children less than 5 years old. “At 3 years (±3months) there were only 8 items that were age appropriate, at 3 years and 6 months there were 13 items while by the age of 4 years all 17 items were appropriate.” (Mercuri et al, 2016)

 

 

2. Expanded Hammersmith Functional Motor Scale (HFMSE)

 

 

A fusion of the Hammersmith Functional Motor Scale (HFMSE) and some items from the Gross Motor Function Measure (GMFM).

 

3. Motor Function Measure-32 (MFM-32)

 

 

A 32-item test for monitoring  severity and progression of motor function in most neuromuscular diseases (Source: ePROVIDE).

 

If you would like to learn more about assessment and interventions for PND, take…

 

Pediatric Neuromuscular Diseases

 

Or these on-demand webinars…

 

 

 

References:

 

Mercuri, E., Coratti, G., Messina, S., Ricotti, V., Baranello, G., D’Amico, A., … & Pane, M. (2016). Revised North Star Ambulatory Assessment for young boys with Duchenne muscular dystrophy. PLoS One, 11(8), e0160195.

 

Pierzchlewicz, K., Kępa, I., Podogrodzki, J., & Kotulska, K. (2021). Spinal muscular atrophy: the use of functional motor scales in the era of disease-modifying treatment. Child neurology open, 8, 2329048X211008725.

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