Profile of cognitive abilities in spinal muscular atrophy type II and III: what is the role of motor impairment?

Bianca Buchignani, Gianpaolo Cicala, Federica Moriconi, Martina Ricci, Anna Capasso, Giorgia Coratti, Jacopo Casiraghi, Jacopo Luca Casiraghi, Emilio Albamonte, Paola Cristofani, Costanza Cutrona, Maria Carmela Pera, Laura Antonaci, Camilla Roncoroni, Daniela Pia Rosaria Chieffo, Valeria A. Sansone, Roberta Battini, Marika Pane, Eugenio Maria Mercuri

Research output: Contribution to journalArticle

Abstract

There has recently been some concern on possible cognitive impairment in patients with Spinal Muscular Atrophy (SMA). The aim of this study was to assess cognitive profiles in type II and III SMA with a focus on individual indexes and possible correlations with motor function. 57 type II and III individuals, aged 3.5–17 years, were consecutively enrolled in a prospective, multicentric study. Cognitive function was assessed using age-appropriate Weschler Scales. Motor function was concomitantly assessed using disease-specific functional scales. Only 2 individuals (3%) had a intellectual disability of mild degree while the others were within normal range, with no significant difference in relation to SMA type, gender or functional status. While the overall quotients were mostly within normal range, some indexes showed wider variability. A significant positive medium correlation was found between Processing Speed Index and motor functional scores. Working memory had lower scores in type III patients compared to type II. Intellectual disability is uncommon in type II and III SMA. Motor functional abilities may play a role in some of the items contributing to the overall cognitive profile.
Original languageEnglish
Pages (from-to)711-717
Number of pages7
JournalNeuromuscular Disorders
Volume33
DOIs
Publication statusPublished - 2023

Keywords

  • Cognition
  • Spinal muscular atrophy
  • Motor function
  • Executive function

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