Myopathies & Neuropathies Flashcards
Muscular dystrophy Rx
- Avoid strenuous exercise & low rep max strengthening
- Monitor O2 & deep breathing, diaphragmatic exercise, encourage cough
- Positioning, ROM
- Pool tx for endurance
- home environment and equipment needs
Muscular dystrophy
- Hereditary
- Symmetrical mm wasting without sensory or neural deficits
- lack of dystrophin causing: mm necrosis with excess eccentrics, decreased protein synthesis, increased Ca+ in mm cell destroying
- diagnosed with presentation, hereditary, EMG, serum enzymes, mm biopsy
Duchenne’s Muscular Dystrophy
(Pseudohypertrophic mm dystrophy)
- most common
- genetic X-linked trait (men get, women carry)
- onset 5-10 y/o - death by 20 primarily from cardiac weak and pulmonary infections
- weakness in pelvis or thighs -> shoulder girdle within 3-5 yrs
- big calve compensation
- Gower’s Maneuver
Gower’s maneuver
Getting up from floor by turning prone and pushing up to arms and walking hands up thighs to standing
Dystrophin
Protein that links mm surface membrane (sarcolemma) with contractile mm protein (actin)
Becker’s muscular dystrophy
- similar to duchennes but slower progression with later life expectancy (40 y/o)
- death 2’ to resp dysfunction or heart failure
- proximal mm more affected than distal
- calf hypertrophy
Fascioscapulohumeral muscular dystrophy
- more benign, starts adolescence, slow progression, normal lifespan
- mostly affecting shoulder girdle, then facial mm
- expressionless face, inability to close eyes
- Popeyes appearance 2’ forearm mm sparing
Limb girdle muscular dystrophy
- mild, normal lifespan
- primary deltoid, biceps, and pelvic mm
- onset late adolescence to early adulthood
- NO calf hypertrophy
Myotonic muscular dystrophy
- genetic, 50% if parent carries
- onset 15-25 y/o
- Myotonia, affecting hand & forearm mm
- prominent neck weakness,
- slow progression
Myotonia
Inability to relax mm promptly after nrml contraction
Polymyositis
- inflammation and degeneration of mm fibers
- idiopathic
- 1* is mm weakness
- starts proximal spreads rapidly distaly and CN’s
- spontaneous remissions and exacerbations possible
Myasthenia gravis
- weakness and easily fatiguing mm
- neuromuscular junction and motor end plate disease from autoimmune disorder (fewer ACh receptors)
- 3:2 women:men
- onset women 20-30y/o; men 50-60y/o
- diagnose detect ACh receptor antibodies, Tensilon drug increases strength in 30-90” for 1-2’, EMG
Myasthenia gravis Rx
- immuno suppression, anticholinesterase, removal of thymus
- deep breathing & coughing, sit up to eat, cervical flexion to sallow
- watch for myasthenia crisis
- energy conservation edu & pacing
- avoid strenuous exercise, stress, sun or cold weather
Myasthenia crisis
Life threatening weakness of respiratory mm which will require a ventilator
Peripheral neuropathy
- involvement of peripheral nerves with neurologic deficits along the distribution of one or more peripheral nerves
- from trauma, nutrition, cancer, infection, drugs, diabetes
Mononeuropathy
- Involves only one peripheral nerve
- usually motor and sensory aspects
Polyneuropathy
- all major nerves entering an anatomical area are affected
- usually distal branches (stocking glove distribution)
Rediculoneuropathy
Involvement of nerve root as it emerges from spinal column
Polyradiculitis
Several nerve roots involved
-occurs from inflammatory response to infection
