scholarly journals BONE HEALTH AND GROWTH IN SPINAL MUSCULAR ATROPHY TYPE 2 AND 3

2022 ◽  
Vol 0 (0) ◽  
pp. 0-0
Author(s):  
Osman Kipoğlu ◽  
Esin Karakılıç Özturan ◽  
Orhan Coşkun ◽  
Ayşe Öztürk ◽  
Edibe Pembegül Yıldız ◽  
...  
Children ◽  
2021 ◽  
Vol 8 (11) ◽  
pp. 1039
Author(s):  
Minsu Gu ◽  
Hyun-Ho Kong

Although nusinersen has been demonstrated to improve motor function in patients with spinal muscular atrophy (SMA), no studies have investigated its effect on fine manual dexterity. The present study aimed to investigate the ability of nusinersen to improve fine manual dexterity in patients with SMA type 2. A total of five patients with SMA type 2 were included. The Hammersmith Functional Motor Scale (expanded version) (HFMSE) and Purdue Pegboard (PP) tests were used to evaluate gross motor function and fine manual dexterity, respectively, until 18 months after nusinersen administration. HFMSE scores improved by 3–10 points (+13–53%) in all patients following nusinersen administration. PP scores also improved in all patients, from 4 to 9 points (+80–225%) in the preferred hand and from 3 to 7 points (+60–500%) in the non-preferred hand. These results suggest that nusinersen treatment improved both gross motor function and fine manual dexterity in children with SMA type 2. Addition of the PP test may aid in evaluating the fine manual dexterity essential for activities of daily living in these patients.


2021 ◽  
Author(s):  
Ryuta Tanaka ◽  
Fujiko Fukushima ◽  
Keiichi Motoyama ◽  
Chie Kobayashi ◽  
Isho Izumi

2019 ◽  
Vol 29 ◽  
pp. S132
Author(s):  
J. Exposito ◽  
D. Natera-de Benito ◽  
L. Carrera ◽  
A. Frongia ◽  
M. Alarcón ◽  
...  

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