DISORDER OF THE NEUROMUSCULAR JUNCTION

Hillelle Elielle L. Corpuz, RN

Neuromuscular Junction

A chemical synapse between a motor neuron and a skeletal muscle fiber.

Neuromuscular Junction
Three basic components:
Presynaptic element Synaptic cleft Postsynaptic element

Neuromuscular Junction
Three basic components:
Presynaptic element Synaptic cleft Postsynaptic element
Plasma membrane of the axon terminal

Filled with a gelatinous material through which ECF may diffuse

Formed by an invagination of the muscle membrane

Neuromuscular Junction
Produces:
End-plate potential

NEUROTRANSMITTER: Acetylcholine (ACh)

Binds to:
Acetylcholine receptors

Broken down by:

Acetylcholinesterase

Neuromuscular Junction
PRESYNAPTIC MEMBRANE: Axon terminal
Synaptic vesicles

POSTSYNAPTIC MEMBRANE: Subneural clefts Acetylcholine receptors Acetylcholinesterase

Myasthenia Gravis
Grave muscle weakness Autoimmune disorder Incorporates rapid fatigue of voluntary muscle and prolonged recovery time Involves skeletal muscles

Myasthenia Gravis
TYPES:
1. Ocular Myasthenia
Males Eye muscles Good prognosis

Myasthenia Gravis
2. Generalized Myasthenia
Proximal musculature Periodic remissions, slowly or rapidly progressive course or a fulminating course

3. Bulbar Myasthenia
Cranial nerves IX, X, XI and XII Rapidly progressive or fulminating

Myasthenia Gravis
Other Types:
Transient Neonatal Myasthenia
Cause: Antibodies produced by the mother that crosses the placental barrier Lasts a few days or weeks and does not recur

Congenital or Infantile Myasthenia

Cause: Shortage of ACh receptors and/or faulty manufacturing and breakdown of Ach Begins with infancy or childhood

Myasthenia Syndrome
Associated with neoplasms Similar manifestations as with MG Results from decrease in release of ACh from nerve terminals, not destruction of ACh receptors

Myasthenia Gravis
HALLMARKS:
Fluctuating weakness of voluntary muscles: facial and extraocular Muscle weakness that increases with activity Muscle strength that improves with rest Muscle strength that improves in response to anticholinesterase medications

Normal impulse conduction:

Stimuli

Triggers release of ACh from presynaptic membrane

ACh and chemical stimuli binds to ACh receptor site on the post synaptic membrane

Depolarization

Muscle contraction

Acetylcholinisterase breaks down ACh

Frees endplate for another signal

Allows repeated muscle contraction

Myasthenia Gravis
Pathophysiology:
Pathologic thymus changes Age and Gender
F 20 M 70-80 Placental transfer of ACh receptor antibodies

Autoimmune
SLE, RhA

Mothers with MG

Recurrent respiratory infections

Problem with formation of T-lymphocyte cells

Recognizes postsynaptic ACh receptors as non-self

Generation of autoantibodies

Production of IgG antibodies against ACh receptors

Triggers immune response

Antibodies attach to and destroy ACh receptors

Blocks ACh transmission

Diminished nerve impulse transmission

Lower amplitude EEP

Lack of muscle depolarization

Ocular

Cranial nerves XI, X, XI, XII

Facial

Peripheral

Respi

Myasthenia Gravis
Manifestations:

Ocular

Ptosis: Sleepy & apathetic Diplopia

Impaired swallowing and articulation Inability to support head

Cranial Nerves XI, X, XI, XII

Peripheral

Weakness Fatigue

Myasthenia Gravis
Manifestations:
Facial Expressionless face Facial droop Snarling appearance Hanging jaw sign

Respiratory

Impaired gag and cough reflex Inability to expel secretions Respiratory distress

Diagnostic Tests
Physical Examination Medical History Immunoassay: ACh Receptor antibody Nerve stimulation studies MRI: Thymoma Tensilon test
Edrophonium chloride 25g 30 seconds (+) Improved strength for a few minutes (-) No improvement or gets worse

Crisis in Myasthenia Gravis: MEDICAL EMERGENCY!!!

1. Myasthenic Crisis
Too little medication Deficient levels of ACh High levels of anticholinesterase Profound symptoms Respiratory difficulty

Factors that predispose a patient to exacerbation: MYASTHENIC CRISIS

Infection Stress Hard physical exercise Surgery
Curare-type

Pregnancy Emotional upset Colds Alcohol ingestion

Quinine

Crisis in Myasthenia Gravis: MEDICAL EMERGENCY!!! 2. Cholinergic Crisis

Too much medication High levels of ACh Low levels of Acetylcholinesterase Profound symptoms Respiratory difficulty Muscarinic side effects Narrowed therapeutic range between underdose and overdose.

Brittle Myasthenics:

Crisis in Myasthenia Gravis: MEDICAL EMERGENCY!!!

Myasthenic crisis: GIVE med Cholinergic crisis: HOLD med Atrophine sulfate
Useful in EITHER crisis Parenteral injection Check for respiratory status Provide airway and ventilatory equipment Suction machine

Note: Respiratory secretions may thicken.

Management
MEDICATIONS Anticholinesterase (Cholinergic)
Neostigmine (Prostigmine) Pyridostigmine (Mestinon)
Available in time span form and is often used at bedtime

Ambenonium (Mytelase) Phyostigmine (Eserine) Side effects: Muscarinic cramping, diarrhea

Management
MEDICATIONS Corticosteroids
Reduce antibody production

OTHERS Thymectomy Plasmapheresis

Plasma exchange Human and/or artificial plasma

Immunosupressants
Dont respond to steroids NR: prevent infection

Problems and Interventions

ACTIVITY Provide frequent rest periods
Rest improves muscle strength Symptoms are least evident upon arising and gets worse as day proceeds

VISION Assess vision changes Alternately patch eye

Every 2hr during waking hours Safe environment Tearisol to prevent corneal damage

Assess muscle strength Assist with ADL

Problems and Interventions

NUTRITION Allow ample time for meals Administer medications 30mins-1hr before meals Assess clients food preference Provide easy to chew and swallow food Suction as needed COMMUNICATION Assess difficulty in speaking Listen and repeat information as needed Answer signal for help promptly Alternative means of communication
Call light, magic slate, pencil and paper

Problems and Interventions

ELIMINATION Assess frequency of stools Small frequent feedings Provide adequate fiber Provide good skin care RESPIRATORY STATUS Suction as needed Tracheostomy care if applicable Administer CPT as prescribed Spend time with client

Discharge Instructions
Signs and symptoms of exacerbation Avoid overexertion and allow time for rest Avoid mycin antibiotics and meds containing quinine Avoid alcohol

 Morphine and meperidine depresses respiratory function and must be avoided Adequate nutrition: increase resistance, decrease infection Schedule of medication Wearing of tag

PERIPHERAL NERVE DISORDER

Hillelle Elielle L. Corpuz, RN

Guillain-Barr Syndrome
Acute demyelinating polyneuropathy
Involves demyelination or axonal degeneration of multiple peripheral nerves

Other names:
Infectious polyneuritis Idiopathic polyneuritis Landrys ascending paralysis Acute inflammatory polyneuropathy

MEDICAL EMERGENCY!

Causes and Risk factors:

Antecedent infection
Cold, sore throat, mononucleosis, hepatitis

Young to middle adulthood (30-50)

GI illness
Campylobacter jejuni

Sequence of events:
1. Precipitating event 2. Changes the cells in the nervous system 3. Immune system recognizes cells as nonself or foreign 4. Sensitized T lymphocytes ad macrophages attack myelin 5. T lymphocytes stimulate B lymphocytes to produce antibodies against myelin sheaths

RESULT: Demyelinating injury that interferes with impulse conduction in the affected peripheral nerve If cell body is not destroyed, peripheral nerve regeneration may take place If cell body dies, nerve regeneration cant take place

Signs and Symptoms

POSITIVE Pain Paresthesia
Caused by abnormal impulse activity

NEGATIVE Muscle weakness or paralysis Loss of tendon reflexes

Motor axon damage

Decreased sensation
Sensory fiber damage

Assessment
Skeletal muscle Loss of muscle tone Areflexia Tenderness
Upon deep pressure

Autonomic symptoms Postural hypotension

Decreased peripheral vascular tone

Arms less weakened than legs Symmetric ascending weakness

Sinus tachycardia Lack of ability to sweat Urinary retention

Assessment
Cranial Nerves Facial palsy Speech difficulties Visual disturbances Swallowing difficulties Bulbar palsy
Paralysis of the jaw, pharynx and tongue

Parasympathetic Sweating Dilated pupils Vasoconstriction Others Paresthesia Paralysis

Accompany loss of motor function

Diagnostic Tests
1. EMG 2. Nerve conduction velocity 3. Lumbar puncture LUMBAR PUNCTURE High protein levels in CSF later in the course
Active demyelination

Management
Supportive
Pain, ventilation, blood pressure and cardiac function

Plasmapheresis Immunoglobulins

Intensive physiotherapy
As soon as voluntary movement returns to prevent muscle and joint contractures

Problems and Interventions

ACTIVITY Anticoagulant
Prolonged immobility and bed rest

Rehabilitation

RESPIRATORY STATUS Ensure oxygenation via ventilatory assistance Prevent immobility complications Tracheostomy

Problems and Interventions

NUTRITION Positive nitrogen balance Fluid intake Caloric requirements PAIN Assess pain characteristics Administer meds as ordered