Pathological gait in Rett syndrome: Quantitative evaluation using three-dimensional gait analysis. (January 2023)

• Record Type: Journal Article
• Authors: Suzuki, Takeshi; Ito, Yuji; Ito, Tadashi; Kidokoro, Hiroyuki; Noritake, Koji; Tsujimura, Keita; Saitoh, Shinji; Yamamoto, Hiroyuki; Ochi, Nobuhiko; Ishihara, Naoko; Yasui, Izumi; Sugiura, Hideshi; Nakata, Tomohiko; Natsume, Jun
• Is Part Of: European journal of paediatric neurology. Volume 42(2023)
• Page Start: 15
• Page End: 21
• Publication Date: 2023-01
• Subjects: Rett syndrome -- Three-dimensional gait analysis -- Ataxic-rigid gait -- Dystonia; 3DGA three-dimensional gait analysis -- GDI Gait Deviation Index -- GPS Gait Profile Score -- GVS Gait Variable Score -- MECP2 methyl-CpG-binding protein 2 -- MRI magnetic resonance imaging -- RTT Rett syndrome; Pediatric neurology -- Periodicals; Nervous System Diseases -- Periodicals; Child -- Periodicals; Infant -- Periodicals; Neurologie pédiatrique -- Périodiques; Pediatric neurology; Electronic journals; Periodicals; Electronic journals; 618.928
• DOI: 10.1016/j.ejpn.2022.11.010
• Languages: English
• ISSNs: 1090-3798

Abstract

Abstract: Objectives: Ataxic-rigid gait is a characteristic gait pathology in patients with Rett syndrome (RTT). In the present study, we aimed to quantitatively evaluate gait pathology in patients with RTT using three-dimensional gait analysis (3DGA). Methods: We performed 3DGA in 11 patients with RTT ranging from 5 to 18 years (median age, 9 years) and in 33 age-matched healthy female controls. We compared the results of 3DGA, including spatiotemporal gait parameters and comprehensive indices of gait kinematics, such as the Gait Deviation Index (GDI) and Gait Profile Score (GPS), between the two groups. The GPS consists of nine sub-indices called Gait Variable Scores (GVSs). Decline in GDI or elevation of GPS and GVS indicated greater abnormal gait pathology. Results: The patients demonstrated significantly slower walking speed, lower step length/length of the lower extremities, lower cadence, wider step width, and higher coefficient of variation of step length than the controls. Moreover, the patients had a lower GDI and higher GPS than the controls. The patients also exhibited higher GVSs for eight out of nine gait kinematics, particularly the sagittal plane in the pelvis, hip, knee, and ankle joint; coronal plane in the pelvis and hip joint; and horizontal plane in the pelvis than the controls. Conclusions: Quantitative evaluation of gait pathology in patients with RTT is possible using 3DGA. We found that in addition to ataxic-rigid gait, abnormalities in the coronal plane of the pelvis and hip joint and the horizontal plane of the pelvis were prominent. Highlights: We performed three-dimensional gait analysis on patients with Rett syndrome. Patients showed decreased walking speed, step length, and cadence than controls. Patients demonstrated increased step width and gait variability than controls. Patients showed reduced gait quality related to ataxic-rigid gait in sagittal plane. Prominent abnormalities in the coronal and the horizontal planes were also observed.