1. Manage a Neurological Mystery
Linda Smith MS RN CCRN

2. Identify the neurological disorder
1. Visual problems, fatigue, weakness of the limbs, remissions and exacerbations
2. Evolving weakness of the lower extremities, pain, respiratory failure, autonomic dysfunction
3. Paralysis of motor branches of facial nerve, ear pain, loss of taste, difficulty chewing
4. Muscle wasting, fasiculations, spasticity, cognition remains intact
5. Tremor, rigidity, dysphagia, neuropsychiatric problems
MS (250, ,000 cases in U.S.)
Guiilain-Barre (1-2 cases/100,000 people in western countries)
Bell’s Palsy (40,000 cases in U.S.)
ALS (30,000 cases in U.S.-about 5000 people with ALS die every year; about 5600 new cases are diagnosed)
5. PD (500,000 cases in the U.S.; 50,000 new cases every year).

3. What is Myasthenia Gravis?
Myasthenia Gravis (MG) is an autoimmune disease characterized by fluctuating weakness of certain muscle groups.
MG can happen to either gender and any ethnicity.
Prevalence rate of the disease in the U.S. is about persons per 100,000; however MG is underdiagnosed and the prevalence is probably higher.

5. Mg Clinical Manifestations
Eyes – double vision (diplopia). droopy eye lids (ptosis)
• Face – facial weakness (flat smile, drooping lips, poor brow movement, weak pucker, unable to puff cheeks out or suck)
• Speech – slurred (dysarthria), nasal or hoarse
• Oropharyngeal - chewing fatigue and dysphagia - swallowing problems may include gagging, choking, nasal regurgitation, difficulty clearing secretions
• Diaphragm - breathing shallow, decreased chest expansion,
short of breath, increased difficulty breathing bending over
or supine
• Neck and Proximal Limb Muscles –
- Head - dropped
- Arms – difficulty carrying, lifting, gripping
- Legs – difficulty climbing stairs, getting out of chairs/bed
Fatigue can increase in warm weather and high humidty.

6. MG Clinical Manifestations

7. Diagnostic Studies History and Physical Muscle Weakness EMG
Tensilon test
ACh receptor antibodies
CXR/CT/MRI
Ice Pack Test

8. Tensilon Test
Select a weak muscle and inject it.

9. Collaborative care-Myasthenia gravis
Drug Therapy- Mestinon; steroids; immnosuppressants.
Other Therapies-Plasmapheresis and IV G
Surgical Therapy-Thymectomy
Drug therapy- Mestinon (Pyridostigmine) most successful in treating MG long-term. Action is to inhibit destruction of Ach and permit freer transmission of nerve impulses across the neuromuscular junction. Symptomatic relief only; doesn’t solve the underlying immune system problem. May be taken by mouth (oral or liquid). Excreted by the kidney. Adverse reactions-(Muscarinic- can be counteracted by Atropine)-N/V, diarrhea, increased peristalsis, increased salivation, bronchial secretions, diaphoresis. Nicotinic side effects (stimulating effect of Ach) are muscle cramps, fasiculations, and weakness.
Steroids-(Prednisone, methylprednisolone)-long term used in severe MG cases
Immunosuppressants-goal is to limit production of antibodies. Imuran most commonly used, allows lower doses of steroids. Side effects of Imuran:flu-like symptoms, bone marrow suppression; hepatotoxic and nephrotoxic.
Plasmapheresis-depletes the Ach receptor antibodies. Frequency depends on protocol-may need daily, weekly, or monthly treatments. Improves 70% of patients within 2 weeks, risks include depletion of clotting factors, hypotension.
Immune gamma globulin (IV IG)-IVIG seems to alter the function or production of antibodies. Side effects include analyphylaxis.
Thymectomy-70% of patients with abnormal thymus will benefit from this surgery, but it may take several months.

10. Nursing management-myasthenia Gravis
Assessment
Goals
Interventions
Teaching
Nursing assessment includes muscles strength of all face and limb muscles, swallowing, speech (volume and clarity), cough and gag muscles.
Focused respiratory assessment includes O2 sat, ABG’s. and pulmonary function tests.
Goals:
1, Return of normal muscular function
2. Prevention of complications-respiratory, malnutrition, injury, inability to communicate, disturbed body image.
3. Demonstrates energy conservation techniques
Interventions:
Identify factors that increase activity
intolerance.
2. Rest periods prior to and following
activities.
3. Develop energy conservation strategies to
decrease fatigue and optimize activities.
4. Offer alternative forms of communication
5. Rest prior and after eating; foods that are easy to swallow; eat more early in the day.
6. Aspiration precautions
7. Referrals to PT, OT, dietician, speech therapy, psycho-social needs.
8. Educational needs-Disease process, therapies and their side effects; enegy conservation strategies;

11. MG Research
Research agenda includes search for biomarkers; mechanisms of disease, therapies, and improving patient outcomes.
Randomized trial of Thymectomy in MG, published by the New England Journal of Medicine. The study shows surgery plus prednisone had a decrease in weakness, and lower requirements of steroids.

13. References
Drugs.com. (2017) Mestinon. Retrieved from
Lewis, S.L., Dirksen, S.R., Heitkemper, M.M., & Bucher, L. (2014) Medical- Surgical Nursing, 9th ed. Elsevier: St. Louis, MO.
Myasthenia Gravis Association of America. (2017) Clinical Overview of MG. Retrieved from
Netwellness.org. (2017) What is remission for Myasthenia Gravis? Retrieved from
Youtube.com. (2017) Positive tensilon test. Retrieved from