PMID- 28543714 OWN - NLM STAT- MEDLINE DCOM- 20170719 LR - 20171209 IS - 1469-8749 (Electronic) IS - 0012-1622 (Linking) VI - 59 IP - 8 DP - 2017 Aug TI - Motor function domains in alternating hemiplegia of childhood. PG - 822-828 LID - 10.1111/dmcn.13443 [doi] AB - 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. FAU - Masoud, Melanie AU - Masoud M AD - Duke University Health System, Duke University School of Medicine, Durham, NC, USA. FAU - Gordon, Kelly AU - Gordon K AD - Department of Speech Pathology and Audiology, Duke University Health System, Durham, NC, USA. FAU - Hall, Amanda AU - Hall A AD - Department of Physical and Occupational Therapy, Duke University Health System, Durham, NC, USA. FAU - Jasien, Joan AU - Jasien J AD - Division of Pediatric Neurology, Duke University Health System, Durham, NC, USA. FAU - Lardinois, Kara AU - Lardinois K AD - Department of Physical and Occupational Therapy, Duke University Health System, Durham, NC, USA. FAU - Uchitel, Julie AU - Uchitel J AUID- ORCID: 0000-0003-4568-5421 AD - Division of Pediatric Neurology, Duke University Health System, Durham, NC, USA. FAU - Mclean, Melissa AU - Mclean M AD - Division of Pediatric Neurology, Duke University Health System, Durham, NC, USA. FAU - Prange, Lyndsey AU - Prange L AD - Division of Pediatric Neurology, Duke University Health System, Durham, NC, USA. FAU - Wuchich, Jeffrey AU - Wuchich J AD - Cure AHC, Inc., Rolesville, NC, USA. FAU - Mikati, Mohamad A AU - Mikati MA AD - Division of Pediatric Neurology, Duke University Health System, Durham, NC, USA. LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20170525 PL - England TA - Dev Med Child Neurol JT - Developmental medicine and child neurology JID - 0006761 RN - Alternating hemiplegia of childhood SB - IM CIN - Dev Med Child Neurol. 2017 Aug;59(8):778-779. PMID: 28556907 MH - Adolescent MH - Adult MH - Child MH - Child, Preschool MH - Deglutition Disorders/diagnosis/etiology/physiopathology MH - Female MH - *Hemiplegia/complications/diagnosis/physiopathology MH - Humans MH - Infant MH - Male MH - *Movement Disorders/diagnosis/etiology/physiopathology MH - *Severity of Illness Index MH - *Speech Disorders/diagnosis/etiology/physiopathology MH - Young Adult EDAT- 2017/05/26 06:00 MHDA- 2017/07/20 06:00 CRDT- 2017/05/26 06:00 PHST- 2017/03/03 00:00 [accepted] PHST- 2017/05/26 06:00 [pubmed] PHST- 2017/07/20 06:00 [medline] PHST- 2017/05/26 06:00 [entrez] AID - 10.1111/dmcn.13443 [doi] PST - ppublish SO - Dev Med Child Neurol. 2017 Aug;59(8):822-828. doi: 10.1111/dmcn.13443. Epub 2017 May 25.