Myasthenia Gravis

An autoimmune neuromuscular disease leading to fluctuating muscle weakness and fatigability. It is an autoimmune disorder, in which weakness is caused by circulating antibodies that block acetylcholine receptors at the postsynaptic neuromuscular junction, inhibiting the stimulative effect of the neurotransmitter acetylcholine. Disease Process

Etiology:

o o o o

unknown genetic predisposition abnormality of the thymus Gender:

Antibodies directed at the acetylcholine receptor sites

Impair transmission of impulses across the myoneural junction

Fewer receptors are available for stimulation

Voluntary muscle weakness

Continues activity

Escalates weakness of voluntary muscle

Signs and Symptoms o Fatigability o Due to overworked muscle

Weakness

Muscles become progressively weaker during periods of activity and improve after periods of rest. Localized: Ptosis Diplopia Bland facial expression Dysphonia

Dysphagia

Generalized: Weakness of all extremities Intercostal muscles

Medical Intervention

Acetylcholinesterase inhibitors: neostigmine and pyridostigmine

can

improve

muscle

function

by

slowing

the

natural

enzyme cholinesterase that

degrades acetylcholine in the motor end plate; the neurotransmitter is therefore around longer to stimulate its receptor. Usually doctors will start with a low dose, e.g. 3x20mg pyridostigmine, and increase until the desired result is achieved. If taken 30 minutes before a meal, symptoms will be mild during eating. Side effects, like perspiration and diarrhea can be countered by adding atropine. Pyridostigmine is a short-lived drug with a half-life of about 4 hours.

Immunosuppressive drugs: prednisone, cyclosporine, mycophenolate mofetil and azathioprine

common for patients to be treated with a combination of these drugs with a cholinesterase inhibitor. Treatments with some immunosuppressives take weeks to months before effects are noticed. It suppress the immune response, decreasing the amount of antibody production.

o o

Other immunomodulating substances, like drugs preventing acetylcholine receptor modulation by the immune system are currently being researched. Plasmapheresis and IVIG.

If the myasthenia is serious (myasthenic crisis), plasmapheresis can be used to remove the putative antibody from the circulation. Also, Intravenous immunoglobulins (IVIG) can be used to bind the circulating antibodies. Both of these treatments have relatively short-lived benefits, typically measured in weeks.

Surgical Interventions o Thymectomy

the surgical removal of the thymus, is essential in cases of thymoma in view of the potential neoplastic effects of the tumor. However, the procedure is more controversial in patients who do not show thymic abnormalities. Although some of these patients improve following thymectomy, some patients experience severe exacerbations and the highly controversial concept of "therapeutic thymectomy" for patients with thymus hyperplasia is disputed by many experts and efforts are underway to unequivocally answer this important question.

Diagnostic Test

Physical examination Muscle fatigability can be tested for many muscles. A thorough investigation includes:

looking upward and sidewards for 30 seconds: ptosis and diplopia. looking at the feet while lying on the back for 60 seconds keeping the arms stretched forward for 60 seconds 10 deep knee bends walking 30 steps on both the toes and the heels 5 situps, lying down and sitting up completely "Peek sign": after complete initial apposition of the lid margins, they quickly (within 30 seconds) start to separate and the sclera starts to show

Blood tests If the diagnosis is suspected, serology can be performed in a blood test to identify certain antibodies.

Neurophysiology Muscle fibers of patients with MG are easily fatigued, and thus do not respond as well as muscles in healthy individuals to repeated stimulation. By repeatedly stimulating a muscle with electrical impulses, the fatiguability of the muscle can be measured. This is called the repetitive nerve stimulation test. In single fiber electromyography, which is considered to be the most sensitive (although not the most specific) test for MG, a thin needle electrode is inserted into a muscle to record the electric potentials of individual muscle fibers. By finding two muscle fibers belonging to the same motor unit and measuring the temporal variability in their firing patterns, the diagnosis can be made.

Edrophonium test

This test requires theintravenous administration of edrophonium chloride (Tensilon, Reversol) or neostigmine (Prostigmin), drugs that block the breakdown of acetylcholine

by cholinesterase (cholinesterase inhibitors) and temporarily increases the levels of acetylcholine at the neuromuscular junction. In people with myasthenia gravis involving the eye muscles, edrophonium chloride will briefly relieve weakness. o Imaging

A chest X-ray . It may identify widening of the mediastinum suggestive of thymoma Computed tomography (CT) Magnetic resonance imaging (MRI) Pulmonary function test

Spirometry (lung function testing) may be performed to assess respiratory function if there are concerns about a patient's ability to breathe adequately. The forced vital capacity may be monitored at intervals in order not to miss a gradual worsening of muscular weakness.

Pathological findings Muscle biopsy is only performed if the diagnosis is in doubt and a muscular condition is suspected. Immunofluorescence shows IgG antibodies on the neuromuscular junction. (Note that it is not the antibody which causes myasthenia gravis that fluoresces, but rather a secondary antibody directed against it.) Muscle electron microscopy shows receptor infolding and loss of the tips of the folds, together with widening of the synaptic clefts. Both these techniques are currently used for research rather than diagnostically.

Nursing Interventions o Health Teaching

It includes the medication management, energy conservation, strategies to help with ocular manifestations and prevention and management of complications.