Exercise programme intervention for persons with motor ataxia Programa de ejercicios de intervención para personas con ataxia motora

Introduction: Ataxia affects the nervous system by decreasing balance and coordination most mostly in the trunk, arms and legs. Physical activity is used to help lowering the symptoms of this disease. Objective: The study objective aims to determine the effects of an exercise program intervention focused on quality of life of this person in terms of body composition, hemodynamic parameters and functional capacity. Methods: A Longitudinal study-case with the exercise sessions performed in a gymnasium with a specialized Personal Trainer. A 43 years old male individual with motor ataxia as participated with a pre and post-test assessments with an exercise program comprised sessions twice a week of 30 minutes each, for 6 months, focused on cardio-fitness, strength and body stabilization. The Scale for the Assessment and Rating of Ataxia was applied to evaluate Gait, Stance, Sitting, Finger Chase, Nose-finger Test, Fast alternating hand movements and Heel-chin slide, and the program SPSS (v20) to present descriptive statistics to express the participant’s improvements. Results: This study indicates that rehabilitation can improve health, well-being and life quality improvements in individuals with ataxia, however, a larger study is required to have analyse if these variables would suffer significant changes in all participants. Besides the improvements in body composition and blood pressure, benefits in all variables of SARA scale were observed, except in «Sitting» were the value remained unchanged. Conclusions: This type of exercise programme intervention can promote capacity and health-related quality of life. This study provides evidence for maintaining physical activity programs in patients with ataxia.


Introduction
Ataxia can be defined as a change in the accurate movement coordination and can be a symptom of many diseases processes in general (Thakkar, Maricich & Alper, 2016). Ataxia can be described by different ways, such as, weakness, dizziness, stroke, falls or other non-specific major complaint. Classically, ataxia is associated with cerebellar dysfunction, but may occur due to different problems with a change in sensitivity that may cause sensorial ataxia. Huff (2016) reported that motor coordination performance is altered, even if motor systems and the cerebellum are intact. Sensory ataxias can be compensated by visual sensory information. However, loss of visual information leads to the observation that sensory ataxias often worsen in poor lighting conditions and may be exhibited during the examination. Motor ataxia is based on unstable and irregular steps, and 2020, Retos, 38, 602-605 © Copyright: Federación Española de Asociaciones de Docentes de Educación Física (FEADEF) ISSN: Edición impresa: 1579-1726. Edición Web: 1988-2041 the lack of compensation for barriers in the environment may be insufficient. The gait of sensory ataxia resulting from the loss of proprioception is notable for the abnormal movement of the legs and the impact of the feet at each step (Bastian & Keller, 2014). Improvements were found in a study of Ilg, Synofzik, Brotz, Burkard & Giese (2009) where it was observed locomotor performance in people with cerebellar ataxia after a six-week home balance exercise program. A total of 14 patients with cerebellar ataxia participated in a 6-week individualized home-based balance exercise program and attended 5 testing sessions using the Scale for the Assessment and Rating of Ataxia (SARA). In the pre-training, post-training, and follow-up testing, assessments of balance and walking were included. Participants revelled in posthoc comparisons significant rehabilitative improvements over the 6-week training period, but TUG (Timed-Up and Go) was retained 1 month later. There were no changes across other measures for the group. This study indicated that improvements in walking speed were affected by the level of balance challenge, but not by age, ataxia severity, proprioception, or duration of exercise.
Fecha recepción: 23-01-19. Fecha de aceptación: 02-04-20 Samuel Honório samuelhonorio@hotmail.com Changes resulting from motor ataxia include problems of balance and motor coordination involving the trunk and limbs. Balance difficulties and inadequate posture generate difficulties in the execution of daily activities directly affect the functional capacity and quality of life of the patient (Armutlu & Karabudak, 2001;Castillo, Gómez-Carmona, Reche, Gil & Ortega, 2018).
According to these studies, an exercise program should include: 1) balance exercises to improve or maintain balance and stability during activities, such as, sitting, standing, walking and moving, and motor coordination exercises incorporated into functional activities of daily living (Abellán & Sáez-Gallego, 2015); 2) muscle strengthening carefully supervised to avoid fatigue. The number of repetitions, the amount of weight used and rest periods should be individually prescribed and adapted periodically. The patient's own weight should be used for global strengthening; strengthening the proximal musculature of the shoulders and hip is important to maintain functional use of upper and lower limbs; 3) physical resistance through cardiovascular exercises, such as walking on the treadmill and or bike exercises. Water exercises offer less limitation and can be beneficial; 4) a specific strategy for each individual, i.e., for patients with walking capacity and loss of proprioception, it is important to encourage compensation with visual feedback, such as looking at the feet while walking. The use of weight at the extremities may be indicated to increase sensory information; 5) Train independent functions, always considering ways to reduce energy effort and monitoring «sitting» and «standing» posture. Patients who do not wander in the community should be encouraged to maintain maximum mobility at home and to stand with support for weight bearing on lower limbs; 6) Evaluate the need, indicate and train the use of auxiliary means with the purpose of giving security to the transfers and locomotion. Assistance equipment with grab bars, crutches and wheelchairs may be important to compensate for the loss of coordination and strength required in different activities; 7) Maintain joint range of motion through postures and stretching exercises to prevent deformities (Robles-Rodríguez, Abad-Robles, Fuentes-Guerra & Benito-Peinado, 2017; Gomez-Marcos & Sanchez-Sanchez, 2019); 8) Monitor respiratory function by associating specific exercises as needed. Relaxation techniques may also be indicated. During balance training, the patient must be exposed to a movement or sequence of movements during which he is stimulated to maintain stability, (Gregorio, Pérez & Moro, 2019). This activity should be done slowly, in order to maintain a proper posture and to the maximum degree where the balance is required to «stop» and «balance» for a few seconds (Armutlu & Karabudak, 2001). Other study Ilg, Brotz, Murkard, Giese, Schols & Synofzik (2010) evaluated a rehabilitation programme with 26 patients with ataxic multiple sclerosis. It comprised sessions of coordination exercises, balance and walking over the ground three times a week for four weeks. In the end of this programme, balance, gait parameters and the Expanded Disability Status Scale (EDSS) scores were improved. Weyer, Abele, Schmitz-Hubsch, Schoch, Frings & Timmann (2007) evaluated a rehabilitation programme comprising static and dynamic balance exercises and coordination exercises (with three one-hour sessions a week for four weeks) with 16 patients. Improvements in SARA scale was observed and the effect lasted for up to a year at least, Weyer et al. (2007). Therefore, this study aims to determine the effects of an exercise programme intervention focused on well-being, including body composition, hemodynamic parameters and functional capacity developed for an adult suffering from motor ataxia.

Participant
We have studied one individual of male gender with 43 years old. This is a study-case with pre, post-test and followup assessments with an exercise program with sessions twice a week of 30 minutes each, for 6 months, focused on cardiofitness, strength, body stabilization and neurologic evaluations in an indoor gymnasium. The follow-up test were made one month after this programme. The individual has this diagnostic confirmed by medical report, after neurologic evaluations and loss of coordination that lead to this conclusion.

Instruments and Procedures
We have used the Scale for Assessment and Rating Ataxia (SARA) to evaluate Gait, Stance, Sitting, Finger Chase, Nosefinger Test, Fast Alternating Hand Movements and Heel-Chin Slide. This scale is reliable and has been validated (Weyer, Abele, Schmitz-Hubsch, Schoch, Frings & Timmann, D. (2007). A specialized physical therapist trained the SARA instrument one month before the first assessment and was responsible for the measurements.

Ethical procedures
The individual was recruited by the investigators from a group of individuals with special needs, that perform physical activities in the gym. The study was approved by the Scientific Board of the Polytechnic Institute and was given full informed consent in writing by the individual.

Statistical Procedures
Program SPSS (v20) was applied to present descriptive statistics to express variables improvements.

Results
According to our findings, the participant made improvements in all variables under study, except for sitting and finger chase that remain equal. Improvements in gait and stance (two aspects directly related to body and posture control) were observed. The improvements in gait, stance and posture after the six-month exercise program are similar in magnitude to those seen after an intensive program with direct intervention previously conducted, Weyer et al. (2007).

Discussion
It is valuable that an assisted training program can be very effective. Determining the appropriate amount of intervention is an important step, because according to literature, rehabilitation will not prevent progression of cerebellar degeneration, and future longitudinal training studies should optimize functions throughout and monitor this disease. Additionally, our program, through SARA evaluation, included sitting and standing balance exercises. For other individuals who are no longer able to walk, the use of this type of exercises is recommendable such as the exercises used by other authors (Ilg et al., 2009). A larger mobility in later stages of cerebellar degeneration will improve patients' quality of life (Kim, Lim, Lee & Koh, 2010) Also, trunk weighting has been recommended (Cernak, Stevens, Price & Shumway-Cook, 2008) and was studied in a randomized trial in 36 patients with ataxia. Trunk weighting of 1.5% (less than ours) improves balance and gait and involves an increase in contributions to movement control (including postural control) and a mechanical reduction in coordination disorders (Freund & Stetts, 2010;Castillo et al. 2018). We provided in our study an exercise on a treadmill that can be an important tool as well. Twenty minutes of treadmill training three times a week improved gait parameter in two patients with cerebellar syndrome (Cernak et al., 2008;Freund & Stetts, 2010). After the exercise program, less assistance with gait was required and the walking distance had increased. These case studies can be confirmed and supported by ours due to the intensive and repeated training, has it was found in Milne, Corben, Georgiou-Karistianis, Delatycki & Yiu (2017) that exercise interventions included coordination and balance training, balance exercises, respiratory muscle training, and treadmill training improved the quality of life of the participants.
In addition to these studies, Marques, Ferreira, Silva, Bezerra & Rabay (2018) evaluated also a study case of an individual with 20 years of age with the main objective of analyse the benefits of strength training. The benefits can be seen in aspects such as walking that previously was not possible without the help of people, walkers and even from a wheelchair, today you do not need any assistance for short walks. Through the stabilization of the body segments, by the balance of muscular strength and dynamic postural balance, which can achieve a more functional gait. The sitting and standing which also needed help, was are able to sit and stand up alone. Thus, proving the effectiveness of strength training to improve motor coordination of limbs and trunks, Table 1 Evaluation criteria of classification and methodology of the variables analysed.
Variable Methodology Classification

Gait
(1) to walk at a safe distance parallel to a wall including a half-turn (turn around to face the opposite direction of gait) and; (2) to walk in tandem (heels to toes) without support.
Stance to stand (1) in natural position, (2) with feet together in parallel (big toes touching each other) and (3) in tandem (both feet on one line, no space between heel and toe).
For each condition, three trials were allowed. Best trial was rated, variating between 0 ("Normal", able to stand in tandem for > 10 s) and 6 ("Unable to stand" for >10 s even with constant support of one arm).
Sitting the proband had to sit on an examination bed without support of feet, eyes open and arms outstretched to the front, 0 ("Normal", no difficulties sitting >10 sec) and 4 ("Unable to sit" for >10 s without continuous support).

Finger Chase
Rated separately for each side), the proband had to seat comfortably. If necessary, the support of feet and trunk was allowed. The examiner seated in front of the proband and performed 5 consecutive sudden and fast pointing movements in unpredictable directions in a frontal plane at about 50 % of the proband's reach.
Movements had an amplitude of 30 cm and a frequency of 1 movement every 2 seconds. Proband was asked to follow the movements with his index finger, as fast and precisely as possible.
Average performance of last 3 movements was rated variating between 0 (No dysmetria) and 4 (Unable to perform 5 pointing movements) Nose-Finger Test (rated separately for each side), the proband had to seat comfortably. If necessary, the support of feet and trunk was allowed. The proband was then asked to point repeatedly with his index finger from his nose to the examiner's finger, which was in front of the proband at about 90 % of proband's reach. Movements were performed at moderate speed.
Average performance of movements were rated according to the amplitude of the kinetic tremor. This item variated between 0 (No tremor) to 4 (Unable to perform 5 pointing movements).

Fast Alternating Hand Movements
(Rated separately for each side), the proband seated comfortably. If necessary, the support of feet and trunk is allowed. The proband was asked to perform 10 cycles of repetitive alternation of pro-and supinations of the hand on his/her thigh as fast and as precise as possible. This movement was demonstrated by the examiner at a speed of approximately 10 cycles within 7seconds. Exact times for movement execution had to be taken.
Heel-Chin Slide (Rated separately for each side), the proband lied on the examination bed without sight of his legs. He was asked to lift one leg, point with the heel to the opposite knee, slide down along the shin to the ankle, and lay the leg back on the examination bed. The task was performed 3 times. Slide-down movements should be performed within 1s.
The task was performed 3 times. Slide-down movements should be performed within 1 s. If proband slides down without contact to shin in all three trials, rate 4. It variates from 0 (Normal) and 4 (Unable to perform the task).  (2019) investigated the effects of different exercise protocols for 42 ataxia patients with multiple sclerosis. Participants were divided into three different groups: a balance training (BT) group, a lumbar stabilization (LS) group and a task-oriented training (TT) group. All groups have received balance training, and additionally, the LS group also received specific lumbar stabilization exercises. The TT group received task-oriented training. All these groups had 18 training sessions. The results showed significant improvements, however balance training alone is not enough for the rehabilitation of these patients. A combination of lumbar stabilization exercises or task-oriented training favours the values of balance rehabilitation.

Conclusions
This study showed improvement in all measures of mobility and body composition in the patient studied after a six-month training program. Individualizing the training program was not critical, as our results suggest that were important for improving these characteristics with an increasing exercise frequency. In table 2 its observable that all body composition variables have improved in the second evaluation. Continued training and progression of the program may be necessary to see optimal retention of results. Although, techniques such as treadmill exercises and others mentioned, with supported body weight and trunk exercises, appear to be valuable. This literature is limited by the small sample sizes, however this will be the challenge for incoming studies and we sure made another contribution towards a better life quality of patients suffering from ataxia.

Study limitations
This investigation, being a case study, has some limitations, such as: evaluating only one participant, which does not allow extrapolating the results for a better comparison between more generic results. The follow-up period could be longer to check if the results obtained during the program are maintained for a longer period. In addition to assessments at the level of motor capacity and body composition, it would be interesting in future studies to evaluate the individual in terms of personal satisfaction, in addition to the improvements presented, to analyse whether in terms of his personal status combined with these improvements, what is his personal and psychological perception regarding these improvements.