[P1-74] Motor Function Profile in Patients with Alternating Hemiplegia of Childhood
[Objective] To characterize motor function in Alternating Hemiplegia of Childhood (AHC) and to investigate relationships motor functions have with each other and with age. [Methods] We studied a cohort of 23 AHC patients with standardized gross motor, upper extremity motor control, motor speech, and dysphagia function tests. [Results] Gross Motor Function Classification System (GMFCS), Gross Motor Function Measure (GMFM-88), Manual Ability Classification System (MACS), and Melbourne Assessment-2 (MA2) scales manifested predominantly mild impairments; motor speech, moderate-severe; and Modified Dysphagia Outcome and Severity Scale (M-DOSS), mild-moderate deficits. GMFCS scores were 34.7% level I (normal), 43.5% level II, 8.7% level III, 8.7% level IV, and 4.3% level V (requiring a wheelchair). GMFM-88 scores showed patients had most difficulty with Dimension E (walking, running, jumping), but even in that dimension the total test percent score mean was 54.15%, and higher in other dimensions with a mean of 72.32%. MACS were 20% level I (normal), 30% level II, 20% level III and 20% level IV. There was a correlation between GMFCS and GMFM-88 (p<0.01), GMFCS and MACS (p=0.031), and GMFCS and MA2 scores (p=0.02-0.05), but not between GMFCS and motor speech or M-DOSS (p>0.34), with motor speech more severely impaired than GMFCS (p<0.013). There was no correlation between any test and age (p=0.289-0.907). [Conclusion] Our findings establish a detailed profile of motor function in AHC patients, argue against regression of skills with age, identify helpful evaluation tools and recognize oropharyngeal function as a severely impacted domain suggesting involvement of brain areas controlling this function.