IEEE trans: Evaluation of upper limb function in Friedreich's ataxia patients through self-feeding activities

Friedreich type ataxia (FRDA) is the most common idiopathic degenerative disease of cerebellar ataxia.
It was first reported by Friedreich (1863).
This disease has unique clinical features, such as childhood onset and progressive ataxia.
Disorders, with pyramidal tract signs, dysphonia, deep paresthesia, scoliosis, arched feet and heart damage
.
Usually onset at 4-15 years old, occasionally infants and those after 50 years of age, both men and women can be affected.

The first symptoms are progressive gait ataxia, staggering gait, shaking left and right, easy to fall; double upper limbs appear within 2 years Ataxia, showing clumsy movements and intentional tremor; at this early stage, knee tendon reflexes and ankle reflexes disappear, and cerebellar dysarthria or fulminant speech appear.
Both upper limb reflexes and bilateral knee tendon reflexes in some patients can be preserved.
Both lower limbs The sense of joint position and vibration is impaired, light touch, pain and temperature are usually not involved, and the weakness of the lower limbs occurs later, which can be upper or lower motor neuron damage, or both
.
The upper limb function of FRDA is evaluated by clinical scales of disease severity, such as the Friedreich Ataxia Rating Scale (FARS), the nine-hole column test (9HPT), and the block test (BBT)
.
Accurate measurement of meaningful upper extremity function is essential for clinical trials of treatments that alter disease progression
.
Self-feeding is an important function for individuals with FRDA to maintain an independent life
.
This article proposes a system to comprehensively evaluate the factors that lead to the decline of upper limb function in FRDA patients
.
This is the first study to use the spoon as an objective assessment of ataxia in a clinical trial
.
As a device-oriented system, the proposed method not only provides a relevant and user-friendly evaluation tool, but also enables disease monitoring in clinical and community-based rehabilitation
.
As a device-oriented system, the proposed method not only provides a relevant and user-friendly evaluation tool, but also enables disease monitoring in clinical and community-based rehabilitation
.
A spoon equipped with inertial sensing, called Ataxia Instrument Measuring Spoon (AIM-S), is designed and tested when evaluating upper limb function while eating with a spoon
.
Due to their sensitivity to eating speed and pleasure, people tend to use a spoon instead of a fork
.
Using Fused Deposition Modeling (FDM) technology and Acrylonitrile Butadiene Styrene (ABS) material, the 3D printed spoon cover of AIM-S was manufactured in the Network Sensing and Control Laboratory (NSC, Australia, Deakin University)
.
The spoon is similar to a typical dessert spoon (10ml in volume) and weighs 84 grams.
People with FRDA can use it
.
A kind of high computing capacity microcontroller unit (MCU, ARM)Ⓡ Cortex-M3 32-bit, IMU (Invensense MPU9250), Wi-Fi module (GS2011), power management circuit and rechargeable battery are installed on the spoon
.
AIM-S captures the acceleration's inertial change (±8g) and angular velocity (±2000°/s) and transmits the kinematics data to the smartphone via Wi-Fi transmission in accordance with the IEEE 802.
11b/g/n standard
.
Through a customized smartphone application, clinicians can monitor signal dynamics in real time and apply time stamps to indicate the beginning and end of each performance cycle in real time
.
Data is automatically uploaded to a cloud-based computing platform for storage and analysis
.
AIM-S hardware spoon and experimental process AIM-S hardware spoon and experimental process participants sit about 30 cm away from the bowl to ensure that AIM-S can be reached easily
.
This task requires the subjects to grasp AIM-S with their dominant hands, scoop the oats from the bowl into the pot of AIM-S, transfer the oats from the bowl to the mouth (but not to the mouth), and transfer the oats from the bowl to the mouth (but not to the mouth).
Put the spoon (and oats) back into the bowl, and then put the spoon back to its original position on the table
.
Participants came from the FRDA Clinic of the Monash Medical Center and the Murdoch Children's Institute
.
Thirty-five FRDA patients (mean age 34.
2 years) ±, 17 women, 18 men) participated in this trial
.
All participants underwent two routine upper limb function tests, namely 9HPT and BBT
.
According to the three detectable movement modes (table, bowl, mouth) represented by different positions (table, bowl, mouth), the overall movement is divided into four parts, namely, table to bowl (TB), bowl to mouth (BM), Mouth to bowl (MB) and bowl to table (BT), and consider the existence of ataxia respectively
.
The angle span between the maximum and minimum inclination angles of FRDA patients was significantly higher than that of the control group (p<0.
001)
.
When food was collected and returned, the average range of the control group's upward and downward rotation was 63.
9 degrees, and the standard deviation was relatively small (5.
7 degrees)
.
This value was significantly higher in patients with FRDA (77.
1%) ± 15.
3 degrees, p<0.
001), although the horizontal range of motion (ROMH) was similar (p>0.
05), indicating that horizontal plane rotation disorders were less manifested
.
DTW distance (Ddtw) and mobility index (Dmi) are both designed to capture the quality of movement
.
FRDA patients have higher Ddtw (p<0.
001) and greater variability (p<0.
001)
.
Except for Dmi, the detection indicators all showed a significant linear relationship (p<0.
05), and a significant linear relationship with BBT, mFARS, and ADL (p<0.
01)
.
Individual differences in grabbing spoons did affect some kinematics, but the prolonged time required for grabbing spoons (TB) (p<0.
001; GI=0.
83) in FRDA patients had nothing to do with these changes
.
Without increasing the length of the trajectory, the increase in time may reflect a strategy to reduce errors or to stabilize the spoon by increasing grip strength
.
AIM-S provides an objective measurement of upper limb movement using an instrument that is essential for basic daily activities
.
Further research is needed to evaluate the performance of AIM-S in a large number of FRDA patients
.
AIM-S provides an objective measurement of upper limb movement using an instrument that is essential for basic daily activities
.
Further research is needed to evaluate the performance of AIM-S in a large number of FRDA patients
.
KD Nguyen, LA Corben, PN Pathirana, MK Horne, MB Delatycki and DJ Szmulewicz, " The Assessment of Upper Limb Functionality in Friedreich Ataxia via Self-Feeding Activity ," in  IEEE Transactions on Neural Systems and Rehabilitation Engineering KD Nguyen, LA Corben, PN Pathirana, MK Horne, MB Delatycki and DJ Szmulewicz, "Assessment of Upper Limb Functionality at The Friedreich Ataxia in Via Self-Feeding Activity , "in  at The Upper Limb Assessment of Functionality in Friedreich Ataxia Self-Feeding Activity Via the IEEE Transactions ON Neural Systems and Rehabilitation Engineering in this message