Myopathy PDF

21-Dec-23 MYOPATHY Dr. Mahmoud ElSayed Midan PhD. Lecturer of PT for neurology CU. & SU. 1 MYOPATHY Myopathies: a group of diseases affecting skeletal muscles that are ch...

21-Dec-23 MYOPATHY Dr. Mahmoud ElSayed Midan PhD. Lecturer of PT for neurology CU. & SU. 1 MYOPATHY Myopathies: a group of diseases affecting skeletal muscles that are characterized by gradual progressive weakness and destruction of skeletal muscles. Progressive muscular dystrophies: a genetically determined myopathies, a group of genetically determined diseases that lead to progressive muscle fiber destruction with progressive muscle weakness. 2 1  21-Dec-23 Causes of myopathies: A. Inherited myopathies: B. Acquired myopathies: caused by genetic disturbance. occurred secondary to disturbance elsewhere in the body. Muscular dystrophy (1st,2nd , 3rd decade) Cushing’s syndrome As in Duchenne, Becker, limb girdle, facioscapulohumeral myopathies Thyrotoxicosis Paraneoplastic Inherited mitochondrial myopathies Systemic inflammatory disease (SLE) Metabolic: K+ disturbances (periodic paralysis syndrome) Congenital myopathy (at birth) Toxic: excessive alcohol consumption Iatrogenic: corticosteroids (cortisone) 3 Clinical picture: Muscle fiber form the end part of motor unit. 1. Muscle fiber affection is LMNL nature 2. Distribution: Hypotonia weakness distributed symmetrical bilateral. Hyporeflexia Affecting proximal girdle muscle more than Weakness distal (shoulder girdle, pelvic girdle, or both) Wasting (some cases pseudohypertrophy) Affecting skeletal muscles rare types may affect primarily distal muscles and others may be combined with: cardio myopathy, pharyngeal, or facial muscles. 4 2  21-Dec-23 3. Clinical features: due to weakness and distribution, some feature affecting posture, mobility, and gait. ❑Gower’s sign: it is a characteristic way of getting up from the floor in which the patient climb himself up ❑Winging of the scapula due to weakness of serratus anterior. ❑Belly abdomen due to abdominal muscles weakness. ❑Hyper lordosis in lumber curve in standing and walking (lurching gait) due to weakness of trunk muscles and gluteal muscles in attempt to move the LOG backward. ❑Wide BOS and Waddling gait to substitute for hip abductors weakness. ❑Deformities including scoliosis, kyphosis, equines. 5 6 3  21-Dec-23 4. Pseudo hypertrophy: infiltration of fats between muscle fibers causing the muscle to appear bulky but with unmatched power (weakness). 5. There is a selectivity of the affected muscle ex: It affects sternal head only of pectoralis major. Also, usually lower abdominal muscles are more severely affected than upper so patient when trying to curl up the umbilicus seemed to be pulled up due to this imbalance. 6. As it is a skeletal muscle disease sensory and autonomic functions are normal unless affected by a comorbidity. 7 Muscular dystrophies Duchenne and Becker represent most common genetically determined myopathies. Several characteristics may assist in differentiation. Duchenne Becker Onset 1st decade 2nd or 3rd decade Progression Progressive Slowly progressive combined deformities +ve ex, scoliosis -ve Cardiomyopathy (ECG) Affected Not affected 8 4  21-Dec-23 Course of the disease Course of the disease depend mainly on its type. ❑In inherited muscular dystrophy the course is progressive and sever Duchenne type affect life span. ❑In congenital myopathy it appears at birth but many may develop some weakness others may sever weakness that cannot develop motor skills and usually affect on life span. ❑Acquired myopathy may reverse course if cause treated. Common causes of death: Respiratory muscles affection Chest infections Cardiomyopathy 9 Medical management: ❑Medical treatment depends mainly on supportive medicine according to the affection. ❑Some medication may increase resistance of the muscle fibers. ❑In secondary myopathies treatment of the cause may reverse the course. 10 5  21-Dec-23 Assessment of patients with myopathy 11 Assessment of patients with myopathy ❖ History: In addition to familiarity with the patient and medical recording history assist in determining the cause by age of onset, special habits, medications and combined symptoms. ❖ Cranial nerves examination: Some types (facio-scapulo-humeral) may affect facial muscles in which there is a bilateral symmetrical weakness of facial muscles. Other rare types may include pharyngeal muscles causing true bulbar palsy symptoms. ❖ Speech: some patients may develop dysarthria due to weakness of facial & pharyngeal muscles ❖ Sensory examination: normal sensory function in myopathy distinct it from some types of neuropathies in which there is sensory affection. 12 6  21-Dec-23 ❖ Motor examination: Inspection: deferent features in posture and gait and deformities appears as described. In addition, patients may develop wasting or pseudohypertrophy. Tone assessment: In addition to passive movement other common methods may be used as: o shaking for distal part o Gower’s method for shoulder girdle: the patient head appeared to sink in between the shoulders (normally shoulders up to ear level only). Reflexes assessment: myopathy characterized by hyporeflexia or lost all deep reflexes and lost superficial reflexes. 13 ❖ Motor examination: Muscle power: weakness is the main feature in myopathy. In mild cases we may use individual manual muscle testing. In moderate cases we may use group muscle test to decrease fatigue. In advanced cases we use functional muscle test. ROM assessment: Muscle shortening is one of complication which commonly occurs in myopathy Increase with advancement of the disease due to lack of active mobility. 14 7  21-Dec-23 ❖ Coordination assessment: weakness disturb non-equilibrium coordination particularly in loss of proximal stability needed for accurate fine delicate movement. In addition, weakness of proximal girdle muscles lead to affection of equilibrium coordination (balance) against gravity. Patient characterized by wide BOS and imbalance. ❖ Gait assessment: Patient usually walk with clumsy wide BOS pattern. Patients use compensatory lurching and side to side trunk movement (waddling) due to weakness of gluteus maximus and medius muscles. Patient may develop equines deformity which affect pattern of gait by altering initial heal contact and may develop genu-recurvatum. 15 16 8  21-Dec-23 ❖ ADL assessment: ❖Problem list: ADL scales as Functional Independence Measure (FIM) 1. Respiratory problems it is important for follow up the progression and 2. Muscle weakness selecting targeted treatment goals. 3. Limitation of ROM 4. Deformities 5. Gait and Balance ❖ Fatigue assessment: 6. Fatigue important point to be considered in assessment and 7. Facial treatment is fatigue as it affects patient quality of life. 8. Pharyngeal muscles affection 17 Physiotherapy interventions: Respiratory problems: Deep diaphragmatic breathing exercise and Forced inspiration (incentive spirometer) to maintain lung extensibility and inspiratory Deconditioning due to lack of activity. muscles activation. Weak inspiration and decrease lung aeration Maintain low level aerobic exercises to due to prevent deconditioning and improve o weakness of intercostal muscles cardiopulmonary endurance. o in some types, diaphragm affected Forced expiration exercises, effective cough, o excessive dependence on accessory and self-assisted cough training. inspiratory muscles. Bilateral symmetrical PNF with inspiration Accumulation of secretion due to weak can be used. expiration and un effective cough o weak intercostal Managing of postural problems and try delay its presentation or decreasing of its severity. o abdominal muscles Use of postural drainage, percussion, Spinal deformities, in addition, add a vibration, assisted cough to improve extraction limitation for lung expansion. of secretions. 18 9  21-Dec-23 Muscle weakness: Causes: Primary muscle weakness mainly of proximal muscles with destruction of muscle fibers. Rehab: Muscle fibers are more fragile so high intensity resisted exercises have adverse effect. In early mild affection mild resisted training may be used to: o just maintain muscle strength o Improve activation (neural component of quality of muscle contraction). In moderate stage active free exercises and active assisted may be used usually in form of functional training 19 Limitation of ROM: Causes: Lack of mobility bad posture structural changes specially that passing over two joints. Rehab: Excessive forceful stretching cause rupture of fragile muscle fibers. Depend mainly on active free or assisted mobility in wide ranges. Mild stretching may be used but with precaution to not using excessive force. Consider that the offered muscle resistance will be weak. 20 10  21-Dec-23 Gait and Balance: Causes: Weakness affects balance ability Physiotherapy approach include both: presented in: Remedial corrective approach o wide BOS Adaptive approaches. o Dependence on upper limb support. As the disease progress we depend more Weakness and imbalance force patient to on adaptive approach due to the fact of use different compensatory strategies as: irreversibility of the disease. o Backward lurch o Side lurch o wide clumsy steps In addition, deformities mainly equines affect posture and stability during gait. 21 Early or mild affection: aiming to to prevent or delay its effect ❑Use mild resistive training ❑Active free. ❑Limitation of the compensatory strategies forcing normal activation. Later with obvious muscle affection, o active free or assisted may be used. o In this stage usually substitution cannot be prevented o Some compensatory strategies must be accepted as wide BOS and or mild lurching. o Using of assistive tools may be introduce as use of walker which may be wheeled. o Some types progress to non-ambulant stage that need use of wheal chair. 22 11  21-Dec-23 Fatigue: ❑Causes of easy fatigability of patient with myopathy Muscle weakness Resultant compensation Poor respiratory function Impaired cardiopulmonary endurance ❑It affect the patient’s quality of life. ❑This problem must be considered during: Assessment treatment putting goals describing assistive tool or device. ❑Rehab: Aerobic training improves cardiopulmonary endurance and decrease fatigue. 23 Facial and Pharyngeal muscles affection: ❑Facioscapulohumeral type affect facial muscles affect patient facial expression may affect speech feeding in severe cases. ❑In rare cases affection of pharyngeal muscles lead to dysphagia and frequent aspirations. ❑Rehab: Working on oromotor control good posturing improve dysphagia problem. Speech therapy may be needed. 24 12  21-Dec-23 Hydrotherapy for myopathy: Hydrotherapy offers a great treatment option for patient with myopathy. Bouncy of the water give considerable assistance and support. Viscosity offering good adequate resistance and in functional form. Hydro static pressure o give some resistance to respiratory muscles o improving cardiopulmonary endurance. It is also recreational in nature o improving motivation and self-steam by maximizing ability. 25 Orthosis for myopathy: ❑Orthosis can benefit patient with myopathy in different ways. It can provide support, assistance It may serve in stretching of tight muscles protect susceptible muscles. ❑Different form of orthosis including soft, flexible, and hard orthosis. ❑The main concern is to be light weight to prevent fatigue. Weight control: Weight control is very important factor improve patient mobility decrease fatigue lengthen ambulatory period. 26 13  21-Dec-23 In conclusion: rehabilitation can be divided into three stages. Independent stage: treatment include: Ambulatory stage; treatment include: Non ambulatory stage: some patients Mild resistive training Active free and active assisted according to type reach to non- Active free exercises Aerobic training ambulatory stage in which patient is confound to wheal chair. Aerobic training Stretching Active assisted exercises Stretching Deep breathing and coughing Stretching training Respiratory muscles strengthening Deep breathing and airway clearance Hydrotherapy modalities PNF Permit compensations Hydrotherapy Corrective gait training Use of orthosis and walking assistive Orthosis as splinting tools Hydrotherapy Stress on weight control due o Weight control measure decreased activity 27 14