motor neurone diseases for all students.pptx

motor neurone diseases for all students.pptx

1. 31.3.2017 Dr Haymar Han Kyi Motor Neurone Disease II

2. Supportive care is just as important as medications in treating patients with motor neuron disease . An interdisciplinary team that includes neurologists, physiatrists, physical and occupational therapists, speech pathologists, orthotists , dieticians , social workers, psychologists , and others can work effectively to provide the needed care for patients and their families . GENERAL PRINCIPLES OF REHABILITATION

3. The most important goal of treatment in physical medicine and rehabilitation for patients with motor neuron disease is to maximize functional independence. It is critical to educate patients and caregivers on the expected disease progression, so that they can prepare themselves for the future.

4. Although each motor neuron disease has a different presentation and clinical progression, it can be helpful to use Sinaki and Mulder's description of the six stages of ALS as a guide in formulating therapies and treatment. During stage I , the patient is independent in mobility and activities of daily living. Therapy for this stage focuses on patient and caregiver education, psychologic support, home safety evaluation, and energy conservation techniques . Therapies at each stage of motor neuron disease

5. Normal physical activities should be continued, and active range of motion exercises, submaximal aerobic strengthening, and general conditioning exercises should be prescribed. Patients should be advised to avoid overuse and fatigue , and to pace themselves appropriately.

6. During stage 2 , the patient with ALS will have moderate generalized weakness , resulting in slightly decreased independence in mobility and activities of daily living . The patient might benefit from bracing, especially with ankle-foot orthoses , mobile arm supports, and wrist supports . Occupational therapists can be particularly helpful in fabricating upper limb orthoses , which can help facilitate fine motor coordination of the upper limbs. Passive range of motion of upper and lower extremities should be taught to patients and caregivers, and incorporated into a daily routine . Depending on the level of disability, it might still be possible for some patients to perform muscle strengthening and endurance exercises with appropriate instruction. A stationary bicycle is often a safe mode of aerobic exercise for patients during this stage.

7. Patients in stage 3 of ALS are still ambulatory, but typically have marked weakness of certain muscle groups . They usually require assistance for transfers and activities of daily living. Patients often need further bracing in this stage, and should also be assessed for a wheelchair . A lightweight manual wheelchair might be useful initially, but patients with ALS almost always require a power wheelchair to maintain their independence. Neck extensor weakness often starts to occur in this stage , and patients report fatigue and difficulty holding their head up . A cervical collar should be considered for this situation. Home modifications, including bathroom revisions, ramp installations, and grab bars, should be done if it is clinically apparent that the patient will need them .

8. Stage 4 is characterized by severe lower extremity and mild upper extremity weakness . Patients will be exclusively wheelchair users during this time . Some can perform activities of daily living at a wheelchair level . Bed with a pressure relieving mattress can help with bed mobility and reduce skin breakdown. Lifting aids can be very helpful for patients and caregivers . Active and passive range of motion exercises should be continued during this stage.

9. Patients in stage 5 have sufficient weakness that they will require assistance with transfers, activities of daily living, and all types of mobility . Pain arising from spasticity or musculoskeletal conditions might need to be the focus of the therapies. Family training on safe transfers, lifting, and positioning should be completed at this point . The caregiver should continue to administer passive range of motion exercises.

10. Stage 6 is the final stage of ALS, at which time the patients are usually in bed on a full-time basis and dependent for all activities. Cardiopulmonary physical therapy techniques can be important in optimizing ventilatory perfusion matching, to mobilize secretions, and to provide cough assistance. The administration of passive range of motion continues, and hospice care is often instituted at this time to help meet the other needs of the patient and caregiver . Although the stages described above are specific to ALS, physicians treating patients with other motor neuron diseases can follow a similar protocol depending on the specific disease and resulting level of disability.

11. Speech and swallowing problems during the course of motor neuron disease can be a major cause of frustration, anxiety, and depression for both patients and caregivers. Patients should be monitored closely by family members and healthcare professionals for progressive dysphagia . A speech-language pathologist can perform a bedside swallowing evaluation or a more involved imaging study to evaluate the degree of dysphagia . The information obtained can then be used to guide treatment and recommendations on diet. Speech and swallowing therapy

12. Mild dysphagia can often be managed with adjustments of head position and/or by diet modifications , such as adding thickening agents to liquids. As dysphagia progresses, supplemental tube feedings might be needed. Neuromuscular electrical stimulation is a fairly new form of therapy that can be tried for patients with dysphagia as well. It is a non-invasive treatment that consists of transcutaneous electrical stimulation applied through electrodes placed on the neck to stimulate inactive swallowing muscles.

13. Sometimes tube feedings are indicated for patients who can still safely swallow, but are unable to fulfill their nutritional needs because of excessive fatigue of the swallowing muscles. In other cases, patients might need to rely completely on tube feedings if they are at severe risk of aspirating with any oral nutrition.

14. There are various types of tubes available for nutritional supplementation. Nasogastric tubes are an option, but in most cases a more permanent option is indicated due to the nature of the disease. A percutaneous endoscopic gastrostomy can be safely and conveniently placed under local anesthesia, and can avoid the gastrointestinal symptoms often associated with a jejunostomy . Patients and caregivers should be educated on the proper care of the feeding tube to maintain its functionality. Education should also be provided on body positioning to decrease the risk of aspiration.

15. Bulbar involvement can lead to dysarthria . In motor neuron disease, dysarthria will usually be one of three types: flaccid, spastic, or mixed. Speech-language pathologists can assist in evaluating the type of dysarthria , and advising on the appropriate treatment and compensatory strategies. In the initial stages of treatment, the focus is usually to maximize intelligibility strategies and introduce energy conservation techniques. With worsening dysarthria and/or anarthria , the incorporation of augmentative communication devices plays an important role. Augmentative communication devices should be customized to meet the needs of the patient. For example, if the upper extremities are too weak to point to letters on a communication board or to type words on a keyboard, a device that utilizes a visual scanning mechanism can be helpful.

16. Most patients with motor neuron disease develop respiratory compromise. Patients with ALS have at least 84% of the mortality from pulmonary complications and respiratory failure. Pulmonary complications can result from bulbar or respiratory muscle weakness, or a combination of these. Respiratory management

17. Management can initially include chest physiotherapy and postural drainage , especially if the patient has difficulty clearing secretions from the chest. Preventing pulmonary infections is a primary goal in respiratory management, and pneumococcal and influenza vaccines should be administered as well. Respiratory muscle exercise can be instituted. In many cases, a training program can delay the onset of ventilatory failure in ALS. A suction machine is a very helpful device for patients who are unable to clear their secretions.

18. As respiratory compromise worsens, various non-invasive devices can be considered. Non-invasive ventilation can postpone initiation of tracheostomy with mechanical ventilation by several months, prolong life, and improve comfort in the patient who refuses total mechanical ventilation.

19. Traditionally, motor neuron disease is thought of as a disease process that affects the body but spares the mind. Dementia due to involvement of the frontotemporal area of the brain can occur, and can occasionally even be the presenting feature of the disease. Patients with motor neuron disease commonly experience a wide range of emotions, including anxiety, denial, anger, and depression. In addition to the psychologic impact of motor neuron disease on patients, caregivers may also be affected. Psychologic management

20. Psychologists and social workers can be helpful in providing individual and family therapy. In addition to psychologic supportive therapy, medications such as antidepressants and anxiolytics should be used as needed.

21. Equipment needs vary depending on the disease process and the patient's level of disability. Orthoses can be simple in design, but can offer patients an opportunity to improve their comfort and/or functional independence. Cervical collars can provide much needed support for patients with neck extensor weakness. If the neck extensor weakness is mild, a soft foam collar may be adequate. Bracing and equipment

22. An ankle-foot orthosis can allow safer ambulation for patients with lower limb weakness. An analysis of gait and specific muscle testing of muscles providing inversion, eversion , dorsiflexion , plantar flexion, and knee extension provides the most valuable input on the type of brace to be fabricated. Consideration should also be given to the progression of the disease, and the prescribed brace should be designed to permit later modifications.

23. In addition to aiding ambulation, an ankle-foot orthosis can also function to passively stretch the ankle plantar flexors. Tightness and contractures that limit ankle dorsiflexion can occur rapidly. Patients and caregivers should be educated on the importance of monitoring the skin for increased pressure areas or breakdown, and adjustments should be made as needed. Lightweight material should be used to fabricate the orthosis when possible, because a heavy brace can prove too difficult to use.

24. Occupational therapists can provide valuable assistance in fabricating or recommending orthoses that help maximize positioning and upper extremity function. Some of these include wrist splints, dynamic finger extension splints. A mobile arm support or a balanced forearm orthosis can be beneficial for patients who have proximal upper extremity weakness. It supports the weight of the arm and forearm against gravity. This type of orthosis can allow for self-feeding by enabling the user to flex the elbow and bring the hand to the mouth.

25. Utensils with a lightweight built-up grip or an angled shape, plate guards, rocker knives, and lightweight mugs can also be useful for feeding. Occupational therapists can also provide excellent advice on dressing techniques and adaptive equipment, such as button hooks, Velcro straps instead of laces, and long-handled shoehorns that can make the task of dressing easier and less energy-consuming

26. An assistive device for ambulation can be helpful in some patients. These are usually prescribed progressively, beginning with straight or quad canes , then a walker. Rolling walkers can be beneficial to those with upper limb weakness who are unable to lift a standard walker.

27. A wheelchair should be considered for patients who can ambulate for only a short distance or who are non-ambulatory. Appropriate seating in any wheelchair is necessary to provide adequate support. A solid back improves trunk alignment and helps prevent shoulder and back dysfunction more than a sling type back. When a patient can no longer propel a wheelchair, a powered wheelchair might be needed. Before prescribing a power wheelchair, however, a brief cognitive assessment of the patient's ability to manage such a wheelchair should be performed.

28. Lifts are often essential for lifting and transferring patients with motor neuron disease. Fully electric hospital beds can help make position changes easier and provide pressure relief. Patients and caregivers should be instructed on the importance of turning every 2 h to decrease the risk of skin breakdown. A mattress overlay might also be indicated to alleviate pressure on susceptible areas and prevent pressure ulcers.

29. Toileting is an area that often becomes very difficult for patients with motor neuron diseases, despite the fact that bowel and bladder function are often preserved. A variety of equipment is available to assist in toileting, but should only be prescribed after obtaining an understanding of the patient's level of function, available assistance, and home environment. Equipment such as a raised toilet seat with armrests, bedside commode, or urinal for men can help the patient be as independent as possible with toileting activities.

30. In the later stages of motor neuron disease, protective undergarments and/or indwelling or condom catheters might need to be utilized. Toileting can sometimes be a very stressful activity for patients with impaired mobility, because the entire process involves many skills and is energy-consuming. It is not unusual to discover that some patients limit their oral intake in the hope of requiring fewer trips to the commode. These patients should be monitored for signs of dehydration and nutritional deficits.

31. Bathing is also quite challenging in many patients, especially in the later stages of the disease. Bathroom modifications, specialized equipment, or a combination of these can be helpful. A tub bench and/or handheld shower can be useful if the patient is still able to safely bathe in the bathroom. As mobility declines, sponge baths by a caregiver are often the safest option, minimizing risk of injury to either the patient or the caregiver.

32. Thank You