• Nerve cells that control muscle are destroyed leads to atrophy • The signal can not reach muscle to activate them Clinical Manifestations • Muscle weakness (involuntary twitching) • Impaired speech • Decrease ability to chew, swallow • Difficulty breathing • Excessive drooling • Depression • Spasticity • Respiratory infection death
Diagnosis CSF analysis (protein) Muscle biopsy (r/o muscle disease) Electromyography (nerve damage) Treatment • Riluzole (Rilutek) – Glutamate agonist • Slows neuron destruction (adds 2-3mths of life) Nursing intervention • Monitor liver function (LFT’s) • No alcohol • Administered evenly spaced For spasticity: • Baclofen • Dantrolene (dantrium) • Diazepam (valium)
Myasthenia Gravis • Sporadic, progressive descending muscle weakness • Abnormal fatigue of voluntary skeletal muscles • Increase with exercise and repeated movements • Affects women age 20-30, men age 70-80 • Unknown etiology • Autoimmune • Thymus gland disorder • Blood cell + thymus antibodies destroy neuroreceptors
What is happening in Myasthenia Graves? Acetylcholine (chemical neurotransmitter) stimulated by nerve impulse attach to receptor sites and depolarize muscle fiber MG – antibodies attach to receptor site and destroy Ach receptor site – decrease muscle contraction Muscle Affected: • Face • Lips • Tongue • Neck • Throat Eventually: muscle fibers degenerate (head ,neck, truck, limbs) • Respiratory life threatening
Clinical Manifestations • Extreme muscle weakness • Fatigue • Ptosis (drooping eye) • Diplopia (double vision) • Difficulty chewing/swallowing • Sleepy, mask like expression • Drooping jaw • Bobbing head • Upper extremity weakness • Respiratory involvement hypoventilation decrease tidal volume
Myasthenia Gravis Crisis Myasthenia Crisis • Exacerbation or under-medicated • Respiratory distress • Hypoxia • Tachycardia • Decease gag reflex • Visual changes Cholinergic Crisis • usually from over medicated • Symptoms worsen Tensilon Test • Anticholinesterase (inhibits the break down of Ach) • Nursing Intervention – atropine at bedside Nursing Intervention • Airway!!! – suction and O2 at bedside • Energy conservation • Sit up while eating thicken fluids • Lubricate eyes • Medical ID
Myasthenia Gravis Treatment & Medications Anticholinesterase 1 st line therapy • Inhibits breakdown of acetylcholine • Pyridostigmine (Mestinon) • Neostigmine (prostigmine) Nursing Intervention • Give at specific time level increase within 1hr improve weakness • Give food within 45mins of taking medication increase strength decrease risk for aspiration • May cause GI upset • Check therapuetic levels (think cholinergic crisis)
MG Treatment & Medications Immunosuppressant's • During exacerbations • Decrease the production of antibodies 1 st – Prednisone 2 nd – Azathioprine (Imuran) Nursing Intervention • Taper off slowly • Increase risk for infection • GI bleeding • Hypernatremia and increase fluid retention • Hypokalemia • Increase glucose • Hypocalcaemia prone to fractures
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- Summer '19
- Myasthenia gravis