scholarly journals Initial Development of Mechanical Arm Support for Spinal Muscular Atrophy Patient

Author(s):  
Dedy H.B. Wicaksono ◽  
Daniel J. Engel ◽  
Leticia A. Genilar ◽  
Samantha T. Wijaya ◽  
Samuel A. Setiawan ◽  
...  

Spinal Muscular Atrophy or SMA disorder is generally thought to have affected as many as 1 in 40 in country like USA. SMA type 2 and 3 are commonly found in Indonesia. Children who suffer from SMA disease cannot move their hands with flexion - extension and abduction - adduction orientation maximally, because the muscles in the patient cannot support the movements of the hand. In this project, an initial joint effort was conducted by SGU and Bioteknik Design, to develop an active mechanical arm support with muscular feedback. Hence, the hand movements of the patient can be supported externally through detecting the muscle tension produced by moving the patient's hand. The tension is detected using Carbon Nanotube (CNT)-coated thread Mechanomyography (MMG) sensor. The sensor signal is then fed into Arduino microcontroller, to give appropriate control signal to the universal power window motor. As an early proof of concept, wood was used as the main structural material for the arm support. The project, however, did not go as expected due to the lack of torque from the motor and missed feedback connection from the sensor. A counter balance mechanism like spring may be attached for future improvement.

BMC Neurology ◽  
2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Tina Duong ◽  
Jessica Braid ◽  
Hannah Staunton ◽  
Aurelie Barriere ◽  
Fani Petridis ◽  
...  

Abstract Background The 32-item Motor Function Measure (MFM32) is a clinician-reported outcome measure used to assess the functional abilities of individuals with neuromuscular diseases, including those with spinal muscular atrophy (SMA). This two-part study explored the relationship between the functional abilities assessed in the MFM32 and activities of daily living (ADLs) from the perspective of individuals with Type 2 and Type 3 (non-ambulant and ambulant) SMA and their caregivers through qualitative interviews and a quantitative online survey. Methods In-depth, semi-structured, qualitative interviews were conducted with individuals with SMA and caregivers from the US. Subsequently, a quantitative online survey was completed by individuals with SMA or their caregivers from France, Germany, Italy, Poland, Spain, Canada, the United States (US) and the UK. In both parts of the study, participants were asked to describe the ADLs considered to be related to the functional abilities assessed in the MFM32. Results from the qualitative interviews informed the content of the quantitative online survey. Results Qualitative interviews were conducted with 15 adult participants, and 217 participants completed the quantitative online survey. From the qualitative interviews, all of the functional abilities assessed in the patient-friendly MFM32 were deemed as related to one or more ADL. The specific ADLs that participants considered related to the patient-friendly MFM32 items could be grouped into 10 key ADL domains: dressing, mobility/transferring, self-care, self-feeding, reaching, picking up and holding objects, physical activity, writing and technology use, social contact/engagement, toileting and performing work/school activities. These results were confirmed by the quantitative online survey whereby the ADLs reported to be related to each patient-friendly MFM32 item were consistent and could be grouped into the same 10 ADL domains. Conclusion This study provides in-depth evidence from the patient/caregiver perspective supporting the relevance of the patient-friendly MFM32 items to the ADLs of individuals with Type 2 and Type 3 SMA.


Author(s):  
Mélanie Annoussamy ◽  
Andreea M. Seferian ◽  
Aurore Daron ◽  
Yann Péréon ◽  
Claude Cances ◽  
...  

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

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