The influence of somatosensory and muscular deficits on postural stabilization: Insights from an instrumented analysis of subjects affected by different types of Charcot-Marie-Tooth disease

Tiziana Lencioni, Giuseppe Piscosquito, Marco Rabuffetti, Gabriele Bovi, Daniela Calabrese, Alessia Aiello, Enrica Di Sipio, Luca Padua, Manuela Diverio, Davide Pareyson, Maurizio Ferrarin

Research output: Contribution to journalArticle

Abstract

Charcot-Marie-Tooth (CMT) disease is the most common hereditary neuromuscular disorder. CMT1 is primarily demyelinating, CMT2 is primarily axonal, and CMTX1 is characterized by both axonal and demyelinating abnormalities. We investigated the role of somatosensory and muscular deficits on quiet standing and postural stabilization in patients affected by different forms of CMT, comparing their performances with those of healthy subjects. Seventy-six CMT subjects (CMT1A, CMT2 and CMTX1) and 41 healthy controls were evaluated during a sit-to-stand transition and the subsequent quiet upright posture by means of a dynamometric platform. All CMT patients showed altered balance and postural stabilization compared to controls. Multivariate analysis showed that in CMT patients worsening of postural stabilization was related to vibration sense deficit and to dorsi-flexor's weakness, while quiet standing instability was related to the reduction of pinprick sensibility and to plantar-flexor's weakness. Our results show that specific sensory and muscular deficits play different roles in balance impairment of CMT patients, both during postural stabilization and in static posture. An accurate evaluation of residual sensory and muscular functions is therefore necessary to plan for the appropriate balance rehabilitation treatment for each patient, besides the CMT type.

Original languageEnglish
Pages (from-to)640-645
Number of pages6
JournalNeuromuscular Disorders
Volume25
Issue number8
DOIs
Publication statusPublished - Aug 1 2015

Keywords

  • Balance impairment
  • Charcot-Marie-Tooth disease
  • Large and small sensory fibers
  • Muscle weakness
  • Rehabilitation

ASJC Scopus subject areas

  • Clinical Neurology
  • Pediatrics, Perinatology, and Child Health
  • Genetics(clinical)
  • Neurology

Fingerprint Dive into the research topics of 'The influence of somatosensory and muscular deficits on postural stabilization: Insights from an instrumented analysis of subjects affected by different types of Charcot-Marie-Tooth disease'. Together they form a unique fingerprint.

  • Cite this