PATHOPHYSIOLOGY OF MYASTHENIA GRAVIS

Normally, an impulse from brain is sent down to Spinal cord to its peripheral nervous system.

It will go to the Motor neuron where the muscle and nerve meet at the neuromuscular junction.

The nerve will release a neurotransmitter called acetylcholine.

Acetylcholine will bind to the acetylcholinereceptors.

Triggers an action potential which will lead to muscle contraction and body movement.

In myasthenia gravis, the bodys immune system creates antibodies that attack the receptor sites for acetylcholine.

Acetylcholine is now unable to bind to the muscle.

Because of that, the muscle contraction is now impaired.

ANATOMY

Neuromuscular Junction

Neuromuscular junction can be defined as a synapse the motor neuron and the muscle fiber. Actually the synapse occurs between the synaptic end bulbs of the motor neuron and motor end plate of muscle fiber. The motor end plate is in fact the highly excitable region of muscle fiber plasma membrane and it is responsible for initiating action potentials across the muscle surface. This effect ultimately results in muscle contraction.

The neuromuscular junction is composed of three parts: Synaptic end bulbs:

As the axon of the motor neuron enters the skeletal muscle, it forms many branches called axon terminals. At the end of each axon terminal, there is a bulbous swelling called synaptic end bulb. Each synaptic end bulb contains many synaptic vesicles. These vesicles contain the allimportant neurotransmitter substances such as acetylcholine. These neurotransmitter substances are responsible for transmission of impulse from axon to muscle fiber through the synapse.

 Motor end plate: It is the part of the sarcolemma of muscle cell, which is in closest proximity to the synaptic end bulb. It shows certain specific features different than those of other regions of muscle cell sarcolemma, including: Synaptic Gutter: It is the invaginated membrane, which forms space for the synaptic end bulbs to reach close to the muscle fiber sarcolemma.

Subneural Clefts: These are small folds of the muscle membrane present at the bottom of the synaptic gutter. They greatly increase the surface area at which the neurotransmitter can act. Increased number of mitochondria: The area of the muscle fiber surrounding the motor end plate shows a considerable increase in the number of mitochondria. The obvious reason for this is the energy demand of the neuromuscular junction.

 The Synaptic cleft:

Synaptic cleft is the space between the motor end plate (muscle fiber part) and synaptic end bulb (motor neuron part) of the neuromuscular junction. It is 20 to 30 nanometers wide. Because of this cleft, the connection between the motor neuron and the muscle fiber is not continuous and there is a break. This break is traversed by the neurotransmitters. This shows the importance of these substances in the activity of muscles (and all other nervous control mechanisms).

Nursing Diagnosis and Interventions

Ineffective breathing pattern related to intercostal muscle weakness

Assess the respiratory rate ,depth and breath sounds and monitor the results of pulmonary function tests(tidal volume, vital capacity, inspiratory force) Encourage deep breathing and coughing exercise if the patient can tolerate Chest physiotherapy, postural drainage is given to immobilize the secretions Suctioning is done to promote the drainage of secretions Provide psychological support to the patient

Impaired physical mobility related to voluntary muscle weakness

The patients goal is the improvement of the strength of the weakened muscles The patient should be educated about the basic facts about anticholinesterase agents-their action, timing, dosage, adjustment symptoms of overdose and toxic effects. The importance of taking the medication and the time should be emphasized. The patient is encouraged to keep a diary to note the fluctuation in symptoms. Time the meals to coincide with the peak effects of anticholinesterase medications

 Plan adequate rest periods throughout the day. Wear appropriate shoes to minimize injury. Eliminate the factors which will cause elevation of the symptoms (emotional upset, infections, particularly respiratory infections, vigorous physical activity etc. Encourage to use cervical collar if there is neck weakness

Risk for aspiration related to weakness of the bulbar muscles

The patient should be assessed for drooling, regurgitation through the nose, choking while attempting to swallow etc. Rest before the meals is encouraged to reduce muscle fatigue The patient is seated in an upright position with the neck slightly flexed to facilitate swallowing Soft food is encouraged Since the muscles of swallowing will be stronger in the morning, caloric intake can be increased for breakfast. The patient is encouraged to rest after breakfast Suction should be available at the home if there is risk of aspiration

Impaired verbal communication related to weakness of the larynx, lips, mouth, pharynx and jaw
The weakened muscles will interfere with verbal communication Techniques for improving the communication includes listening to the patients, repeating what they are trying to communicate and asking the patient to blink their eyes for yes or no answers

Disturbed sensory perception related to ptosis, and decreased eye movements

Impaired vision results from ptosis, decreased eye movements or double vision Measures includes instilling artificial tears into the eyes to prevent corneal damage when the eyelids will not close completely Placing eye patch when there is double vision. Applying a thin adhesive tape over the eyelid if there is ptosis. Sunglasses reduces the effect of bright light