Article

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 (MA2) 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.
CI - (c) 2017 Mac Keith Press.

Langue : ANGLAIS