Myopathies & Neuropathies

Question 0

Muscular dystrophy Rx

Answer 0

• 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

Question 1

Muscular dystrophy

Answer 1

• 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

Question 2

Duchenne’s Muscular Dystrophy

Answer 2

(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

Question 3

Gower’s maneuver

Answer 3

Getting up from floor by turning prone and pushing up to arms and walking hands up thighs to standing

Question 4

Dystrophin

Answer 4

Protein that links mm surface membrane (sarcolemma) with contractile mm protein (actin)

Question 5

Becker’s muscular dystrophy

Answer 5

• 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

Question 6

Fascioscapulohumeral muscular dystrophy

Answer 6

• 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

Question 7

Limb girdle muscular dystrophy

Answer 7

• mild, normal lifespan
• primary deltoid, biceps, and pelvic mm
• onset late adolescence to early adulthood
• NO calf hypertrophy

Question 8

Myotonic muscular dystrophy

Answer 8

• genetic, 50% if parent carries
• onset 15-25 y/o
• Myotonia, affecting hand & forearm mm
• prominent neck weakness,
• slow progression

Question 9

Myotonia

Answer 9

Inability to relax mm promptly after nrml contraction

Question 10

Polymyositis

Answer 10

• inflammation and degeneration of mm fibers
• idiopathic
• 1* is mm weakness
• starts proximal spreads rapidly distaly and CN’s
• spontaneous remissions and exacerbations possible

Question 11

Myasthenia gravis

Answer 11

• 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

Question 12

Myasthenia gravis Rx

Answer 12

• 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

Question 13

Myasthenia crisis

Answer 13

Life threatening weakness of respiratory mm which will require a ventilator

Question 14

Peripheral neuropathy

Answer 14

• involvement of peripheral nerves with neurologic deficits along the distribution of one or more peripheral nerves
• from trauma, nutrition, cancer, infection, drugs, diabetes

Question 15

Mononeuropathy

Answer 15

• Involves only one peripheral nerve

- usually motor and sensory aspects

Question 16

Polyneuropathy

Answer 16

• all major nerves entering an anatomical area are affected

- usually distal branches (stocking glove distribution)

Question 17

Rediculoneuropathy

Answer 17

Involvement of nerve root as it emerges from spinal column

Question 18

Polyradiculitis

Answer 18

Several nerve roots involved

-occurs from inflammatory response to infection