6MWT can identify type 3 SMA patients with neuromuscular junction dysfunction

Marika Pane, Serenella Servidei, Eugenio Maria Mercuri, Maria Carmela Pera, Marco Luigetti, Giorgia Coratti, Elena Stacy Mazzone, Concetta Palermo, Domiziana Ranalli, Giuseppe Granata

Research output: Contribution to journalArticle

16 Citations (Scopus)

Abstract

The aim of the study was to establish if the decrease in gait velocity on the 6 minute walk test relates to signs of neuromuscular junction dysfunction in spinal muscular atrophy type 3 patients. 6 minute walk test and low-rate repetitive nerve stimulation test were performed in fifteen ambulant patients with spinal muscular atrophy type 3 of age between 9 and 66 years. The 6 minute walk distance ranged between 66 and 575 m. The difference between the first and the 6th minute ranged between 0 and −69%. The low-rate repetitive nerve stimulation test measured in % of loss ranged between −31.7% to +4.2% to the axillary nerve. The correlation between 6 minute walk test changes and low-rate repetitive nerve stimulation test changes was 0.86. Our data suggest that the 6 minute walk test can identify fatigue in the ambulant type 3 patients who have a concurrent neuromuscular junction dysfunction. The identification of fatigue with a simple clinical test may help to target patients who may benefit from drugs that facilitate neuromuscular transmission.
Original languageEnglish
Pages (from-to)879-882
Number of pages4
JournalNeuromuscular Disorders
Volume27
DOIs
Publication statusPublished - 2017

Keywords

  • 6 minute walk test
  • Fatigue
  • Genetics (clinical)
  • Low-rate nerve stimulation
  • Neurology
  • Neurology (clinical)
  • Neuromuscular disorders
  • Neuromuscular junction
  • Pediatrics, Perinatology and Child Health
  • Spinal muscular atrophy

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