Rehabilitation of Ataxia




© Springer International Publishing Switzerland 2017
Hsin Fen Chien and Orlando Graziani Povoas Barsottini (eds.)Movement Disorders Rehabilitation10.1007/978-3-319-46062-8_5


5. Rehabilitation of Ataxia



Marise Bueno Zonta1, Giovana Diaferia , José Luiz Pedroso  and Hélio A. G. Teive1


(1)
Universidade Federal do Paraná, Curitiba, PR, Brazil

(2)
Department of Neurology, Universidade Federal de São Paulo, São Paulo, Brazil

 



 

Giovana Diaferia



 

José Luiz Pedroso (Corresponding author)



Keywords
RehabilitationPhysical therapyMotor coordinationSpeech and voice therapyAtaxia



Introduction


Cerebellar ataxias represent an extensive group of heterogeneous diseases, characterized by gait impairment and loss of balance, used related to cerebellar involvement. There are several forms of cerebellar ataxias, including acute causes (for instance, cerebellar stroke) or neurodegenerative disorders (such as hereditary ataxias). There is no effective treatment for the hereditary ataxias, and management remains supportive and symptomatic. For acute cerebellar syndrome, rehabilitation is mandatory. Rehabilitation process may lead to improvement in cerebellar symptoms and daily life functions, and must be included as a crucial treatment option for the cerebellar ataxias.


Motor Rehabilitation


Rehabilitation is essential for all patients with ataxia. Any approach that includes physical therapy can help to improve quality of life. Physical therapy should start early at the diagnosis of ataxia, even in people with mild symptoms, such as sporadic imbalance. Therapy on the ground should consist of balance training and therapists may suggest other practices that are complementary to the treatment program. When people present with the early signs and symptoms of ataxia, they are likely to engage in unsupervised activities in an attempt to minimize their symptoms. Guidance of a skilled professional is key at the beginning of any program to incorporate activities that are suitable for these patients. In general, patients with ataxia should be encouraged to exercise as part of health promotion, and as long as risk factors and safety considerations have been assessed. Exercise should be tailored individually and may involve exploring several different options for building motivation, but patients with ataxia should be fully aware of the importance of motor coordination and balance training.

Physical therapy should not be discontinued in advanced stages of disease in dependent, bedridden people because passive/assisted mobilization and correct positioning are essential at these stages to maintain the same level of residual activity, prevent pain and bedsores, and improve well-being. Over the course of the disease, management targets should be adjusted based on the patient’s health status and needs taking into account individual characteristics, including age, interests, and financial resources, among others.

Rehabilitation of patients with ataxia involves a thorough assessment to establish the patient’s current level of functioning and to set up treatment goals and strategies. Regular reassessments are also necessary to assess the effectiveness of the current management and to identify any changes to the treatment plan that may be required [1].

Patients with ataxia have specific needs depending on their symptoms. Individually tailored physical therapy strategies should be planned and incorporate specific objectives and goals toward the highest possible level of functioning, with continuous adjustments over time to minimize loss of function. Besides motor coordination and balance training, other goals can be set, including increased transfer and locomotion independence; muscle strengthening; increased physical resilience; “safe fall” strategy; learning to use mobility aids (e.g., a walker); learning how to reduce the body’s energy expenditure; developing specific breathing patterns; monitoring posture while sitting and standing; preventing and minimizing deformities and pain in people confined to a bed and/or wheelchair; and learning muscle relaxation exercises, among others [2]. Patient assessment can provide objective information to guide the use of these therapy modalities or a combination of different modalities.

In their review, Cassidy et al. [3] make recommendations on physical therapy interventions, including dynamic task practices that challenge stability and explore stability limits; combined strength and flexibility training; a compensatory approach including orthotics and devices; teaching practical, everyday strategies for those with severe upper extremity tremor; and careful documentation of patient outcomes.

A physical therapy plan should be prepared from the interpretation of the assessment results and can vary depending on the type and characteristics of ataxia [1]. Recovery of motor function depends on the cause and the site of the cerebellar injury [4]. Although approaches that improve proprioception and incorporate visual aids are used more commonly in people with sensory ataxia, stabilization training is more important to reduce truncal and extremity ataxia in people with cerebellar ataxia. People with vestibular ataxia should be given habituation exercises to reduce vertigo, and vestibulo-ocular and vestibulospinal reflexes should be stimulated to improve balance. In some challenging cases, such as mixed ataxia, a number of physical therapy interventions are required. When preparing a treatment plan, it should be kept in mind that the proprioceptive, vestibular, and visual systems and the cerebellum are closely related, and that balance and coordination result from this relationship [1]. These authors argue that the methods applied for the rehabilitation of ataxia cannot be classified as approaches directed merely toward proprioception or balance, as all of these interact with each other.

Physical therapy techniques may vary depending on the approach used. An individual treatment may consist of modalities on the ground (with or without equipment), and/or in the water (hydrotherapy), and/or on a horse (equine therapy), and/or virtual reality training. In a single exercise practice or therapy session they can work stability, balance, strength, elongation, and motor coordination at the same time as all these skills are interconnected.

Regarding motor recovery, two major issues have deserved attention in the rehabilitation of degenerative cerebellar diseases [5]. The first issue is whether motor sequence learning or relearning of everyday tasks occurs, as the cerebellum plays a crucial role in motor learning. The second one is whether gains in motor function can be maintained over time given the progressive nature of these conditions. Ilg et al. [4] contend that motor rehabilitation in people with cerebellar damage is challenging because the cerebellum plays an important role in motor learning and they may have difficulty recovering function with physical therapy owing to damage to structures critically involved in the relearning of motor skills.

In another review, Ilg et al. [4] looked at studies that assessed physical therapy interventions for patients with cerebellar ataxia . These interventions included progressive stimulation on gait and balance for improved postural stability and less dependence in daily life activities. The authors note that most studies comprised heterogeneous, small samples, making it difficult to compare the methods of intervention. They also cite the clinical studies by Ilg et al. [6] and Miyail et al. [5], both large cohorts that systematically showed the benefits of motor rehabilitation in patients with cerebellar degenerative diseases. Ilg et al. [7] found significant benefits of intensive coordination and balance training that persisted over 12 months, despite a gradual decline in motor performance due to progressive neurodegenerative symptoms.

The finding that patients with ataxia can improve movement performance [6] corroborated that seen in physical therapy practice. Therapists’ current challenge is to find new ways to encourage patients to keep up with continuous training to preserve their gains. Continuous coordination and balance training should be the standard of care; however, raising patient awareness and adjusting their daily routine are required. An exercise program should take into account patient age, clinical features, and prognosis. There are countless exercises available and a good therapist should find new creative ways to keep patients interested and engaged.

The physical therapy approaches listed in a review study by Armutlu [1] are designed for proprioception improvement, balance and stabilization training, and vertigo and dizziness reduction. The author reviewed several studies on approaches to improving proprioception that included techniques to increase proprioception input through mechanical stimulation of articular surfaces, muscles, and tendons, decreasing postural instability by improving body awareness. The techniques include proprioceptive neuromuscular facilitation (PNF), mat activities, resistive exercises, use of Johnstone pressure splints, gait exercises on different surfaces (hard, soft, and inclined surfaces) with eyes open and closed, and vibration techniques. They also included body awareness and balance activities. The authors of the studies reviewed cited exercise disciplines such as Tai Chi and yoga. Proximal stabilization and activities in different positions with movement progress from one position to another were emphasized. These authors concur that the best indicator of dynamic stabilization and balance is gait.

Frenkel exercises were also developed for the stimulation of proprioception. Simon Frenkel (1860–1931) was a pioneer of neurorehabilitation and developed an exercise program with various positions for improving motor coordination, balance, and therefore ataxia. These exercises can be used in combination with PNF techniques [8] and both are effective in mild cases of ataxia.

A variety of physical therapy techniques involves balance and proprioception stimulation. The main principles of therapy are:


  1. 1.


    Keep the best posture for as long as the patient can. For example, people who are able to walk or stand should maintain it for long enough. They should also be encouraged to maintain a “standing position” as home practice (e.g., standing while watching TV for a period every day, even if they need support). The focus is to preserve their level of functioning: if a person is able to walk, she/he should do it as much as possible. The order of exercises does not matter; it is important to exercise as many positions as possible by using different mechanisms and muscles, giving emphasis to the best posture.

     

  2. 2.


    Promote stability by using different positions and while progressing from one position to another. Train both static postural stability and dynamic postural stability for every position. Mechanical stimulation of joint surfaces, muscles, and tendons increases sensory and proprioceptive information on these body areas and helps reduce instability.

     

  3. 3.


    In addition to balance and coordination exercises, muscle strength and stretching exercises should be performed in every position. Some people may require stretching exercises at the beginning of the therapy session, whereas others may gradually change positions and progressively perform stretching exercises.

     

  4. 4.


    Balance training should be functional, i.e., people should practice functions in the performance of daily tasks that require balance and proper posture. Improving posture control will promote a maximum level of functional capacity; training may help to reduce postural sway during movement.

     

  5. 5.


    As patients have to maintain an unbalanced position to train balance reactions, these exercises should be performed safely and progress depending on their symptoms. The degree of difficulty of an exercise should gradually be increased using obstacles or provoking balance reactions to challenge them in a safe exercise routine.

     

  6. 6.


    Strength training should use body weight exercises. Building muscle strength is crucial, but care should be taken not to fatigue the muscles. It is important to strengthen proximal shoulder and hip muscles to maintain the function of the upper and lower extremities.

     

  7. 7.


    Exercises should be practiced consciously at first, and in later stages should be followed by automatic exercise activities. Exercises should progress from simple to complex within people’s functional level bearing in mind symptom progression. Some exercises should be practiced first with the eyes open and later with the eyes closed.

     

  8. 8.


    A home practice program should incorporate other physical activities such as sport activities to train components of basic skills in patients with ataxia. They should be provided with support materials with pictures/drawings to guide their home exercise routine [9].

     


Use of Virtual Reality Applications


Intensive balance and motor coordination training can make people weary and be a complex practice for children with ataxia especially those lacking motivation. A major advantage of virtual reality is to motivate patients. The literature also reports additional benefits including improved balance and posture, locomotion and gait, and upper and lower extremity function [10, 11].

Many researchers point out the potential of virtual reality games as an effective approach to improve motor skills and as a tool for assessing treatment effectiveness. They have investigated a broad variety of therapeutic applications, especially for motor and locomotor function rehabilitation in patients with neurological conditions, and in patients with cognitive impairment. Ilg et al. [10] assessed the effectiveness of using three X-Box® videogame sets in children with ataxia. The children underwent an 8-week training program (2 weeks at the study site and 6 weeks in their homes) specifically designed to improve motor coordination and balance. The authors found that videogames reduced symptoms of ataxia, improved the children’s ability to maintain good balance, and made the step-to-step transition while walking safer and more effective.


Vestibular Rehabilitation Exercises


People with secondary, nongenetic, and hereditary degenerative ataxia have vestibular complaints in daily clinical practice that require treatment. Different vestibular rehabilitation protocols are available and each has distinctive characteristics. The Cawthorne–Cooksey exercise protocol (1946) was designed to treat vestibular ataxia. This exercise program is aimed at reducing vestibular disturbances, including vertigo, vision changes during head motions, and balance and motor problems. It consists of a series of head movements, body movements, and head–eye coordination and balance exercises performed in various positions where patients are instructed to perform the exercises slowly at first, and then increasing speed. These exercises are intended to improve the patient’s well-being while performing daily activities. In his review [1], Armutlu cites some studies that show the use of many components of this exercise program by physical therapists and occupational therapists.

Many protocols [12] are available to treat people with vestibular ataxia, in particular those using virtual reality applications. Virtual reality-based vestibular rehabilitation consists of interactive graphics creating a virtual reality experience [13]. The benefits of this treatment are reported in the literature and include balance and posture alignment, improved mobility and upper and lower extremity function, and increased motivation to exercise.

Vestibular rehabilitation exercises, either conventional or virtual reality-based protocols, have the same goal of seeking to restore body balance, stimulating and accelerating the natural mechanisms of vestibular compensation, and thus enhancing neuroplasticity in people with peripheral and/or central vestibular disorders. The sole difference is the equipment used to achieve vestibular compensation.


Adding Extra Weight to Exercises


Weighted anklets attached to lower extremities, vests to be worn on the trunk, belts around the waist, cuffs, and adapted insoles to be placed in shoes can be used to add extra weight to exercises. Better movement control is expected. A study by Dias et al. [14] showed the effectiveness of adding extra weight to exercise to improve static postural balance and dynamic postural balance, gait coordination, and functional independence. This finding is corroborated by that reported by providers and patients training with extra weights and suggests that this practice should be considered on an individual basis.

Only gold members can continue reading. Log In or Register to continue

Stay updated, free articles. Join our Telegram channel

Oct 7, 2017 | Posted by in NEUROLOGY | Comments Off on Rehabilitation of Ataxia

Full access? Get Clinical Tree

Get Clinical Tree app for offline access