This study examined the association between Gait Deviation Index (GDI) and the five-times-sit-to-stand test (FTSST) or gait speed results, which represent mobility and muscle strength of the lower extremities in ambulatory children with Gross Motor Function Classification System (GMFCS) level I and II spastic cerebral palsy. In this cross-sectional, observational study, three-dimensional gait analysis data were obtained during gait trials to evaluate the GDI in 35 children (age 5-16 years) with spastic palsy. Motor function was evaluated using FTSST and gait speed. Gross motor function was evaluated using GMFCS. Children with GMFCS level II spastic cerebral palsy demonstrated lower GDI (p < 0.001) and poorer FTSST (p = 0.031) than those with GMFCS level I spastic cerebral palsy. Correlation analysis showed that FTSST results were significantly correlated with GDI (r = −0.624; p < 0.001). Motor function may be important for the maintenance of gait quality in patients with GMFCS level I and II spastic cerebral palsy and should not be ignored. In conclusion, reduction in gait impairment may affect the values of FTSST and GDI in patients with spastic cerebral palsy who can ambulate without an assistive device.

Achondroplasia (ACH) is the most common short-limbed skeletal dysplasia caused by activating mutations in the fibroblast growth factor receptor 3 (FGFR3) gene. We identified that meclizine hydrochloride inhibited FGFR3 signaling in various chondrocytic cells and promoted longitudinal bone growth in mouse model of ACH. Meclizine has safely been used for more than 50 years, but it lacks the safety data for repeated administration and pharmacokinetics (PK) when administered to children. We performed a phase Ia study to evaluate the PK and safety of meclizine administered orally to ACH children. Twelve ACH children aged from 5 to younger than 11 years were recruited, and the first 6 subjects received once a day of meclizine in the fasted condition, subsequent 6 subjects received twice a day of meclizine in the fed condition. Meclizine was well tolerated in ACH children with no serious adverse events. The mean C max , T max , AUC 0-24h , t1/2 during 24 hours in the fasted condition were 130 ng/mL, 1.7 hours, 761 ng�h/mL, and 8.5 hours respectively. The simulation of repeated administration of meclizine for 14 days demonstrated that plasma concentration apparently reached steady state around 10 days after the first dose both at once a day and twice a day administration. The AUC 0-10h of the fasting and fed condition were 504 ng�h/mL and 813 ng�h/mL, respectively, indicating exposure of meclizine increased with the diet. Although higher drug exposure was confirmed in ACH children compared to adults, a single administration of meclizine seemed to be well tolerated.

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[Purpose] This study aimed to identify a simple and useful muscle parameter for use with the Gait Deviation Index in assessment of ambulatory children with unilateral and bilateral spastic cerebral palsy. [Participants and Methods] Twenty-eight patients (aged 6 to 18 years; 16 females and 12 males) participated in this cross-sectional study. Outcome measurements included the Gait Deviation Index, grip strength, 5-repetition chair stand test, upper limb skeletal muscle mass index, and lower limb skeletal muscle mass index. [Results] By multiple regression analysis, significant independent correlations were observed between the Gait Deviation Index and 5-repetition chair stand test and the Gait Deviation Index and lower limb skeletal muscle mass index, but not between the Gait Deviation Index and grip strength or upper limb skeletal muscle mass index. [Conclusion] The Gait Deviation Index was correlated with lower limb muscle mass in children with spastic cerebral palsy. Determination of lower limb muscle mass may be useful gait evaluation.

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