Premium
Motor function domains in alternating hemiplegia of childhood
Author(s) -
Masoud Melanie,
Gordon Kelly,
Hall Amanda,
Jasien Joan,
Lardinois Kara,
Uchitel Julie,
Mclean Melissa,
Prange Lyndsey,
Wuchich Jeffrey,
Mikati Mohamad A
Publication year - 2017
Publication title -
developmental medicine and child neurology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.658
H-Index - 143
eISSN - 1469-8749
pISSN - 0012-1622
DOI - 10.1111/dmcn.13443
Subject(s) - gross motor function classification system , motor function , physical medicine and rehabilitation , psychology , motor skill , physical therapy , medicine , cerebral palsy , developmental psychology
Aim To characterize motor function profiles in alternating hemiplegia of childhood, and to investigate interrelationships between these domains and with age. Method We studied a cohort of 23 patients (9 males, 14 females; mean age 9y 4mo, range 4mo–43y) who underwent standardized tests to assess gross motor, upper extremity motor control, motor speech, and dysphagia functions. Results Gross Motor Function Classification System ( GMFCS ), Gross Motor Function Measure‐88 ( GMFM ‐88), Manual Ability Classification System ( MACS ), and Revised Melbourne Assessment ( MA 2) scales manifested predominantly mild impairments; motor speech, moderate to severe; Modified Dysphagia Outcome and Severity Scale (M‐DOSS), mild‐to moderate deficits. GMFCS correlated with GMFM‐88 scores (Pearson's correlation, p =0.002), MACS ( p =0.038), and MA2 fluency ( p =0.005) and accuracy ( p =0.038) scores. GMFCS did not correlate with motor speech ( p =0.399), MA2 dexterity ( p =0.247), range of motion ( p =0.063), or M‐DOSS ( p =0.856). Motor speech was more severely impaired than the GMFCS ( p <0.013). There was no correlation between any of the assessment tools and age ( p =0.210‐0.798). Interpretation Our data establish a detailed profile of motor function in alternating hemiplegia of childhood, argue against the presence of worse motor function in older patients, identify tools helpful in evaluating this population, and identify oropharyngeal function as the more severely affected domain, suggesting that brain areas controlling this function are more affected than others.