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Myasthenia Gravis & Guillan Barre Syndrome

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MYASTHENIA GRAVIS MYASTHENIC CRISIS

an autoimmune disorder affecting the  Respiratory distress and varying degrees of


myoneural junction, is characterized by varying degrees dysphagia (difficulty swallowing), dysarthria
of weakness of the voluntary muscles (difficulty speaking), eyelid ptosis, diplopia, and
prominent muscle weakness are symptoms of
Women are affected more frequently than men, and myasthenic crisis.
they tend to develop the disease at an earlier age (20 to  Providing ventilatory assistance takes
40 years of age, versus 60 to 70 years for men) precedence in the immediate management of
the patient with myasthenic crisis.
CLINICAL MANIFESTATIONS
The initial manifestation of myasthenia gravis in two
thirds of patients involves the ocular muscles. GUILLAN-BARRE’ SYNDROME

 Diplopia (double vision) and ptosis (drooping of  syndrome is an autoimmune attack on the
the eyelids) are common peripheral nerve myelin.
 Many patients also experience weakness of  The result is acute, rapid segmental
the muscles of the face and throat (bulbar symptoms) demyelination of peripheral nerves and some
and generalized weakness. cranial nerves, producing ascending weakness
 Laryngeal involvement produces with dyskinesia (inability to execute voluntary
dysphonia (voice impairment) and increases the risk of movements), hyporeflexia, and paresthesias
choking and aspiration. (numbness).
 Generalized weakness affects all the extremities  Campylobacter jejuni, cytomegalovirus,
and the intercostal muscles, resulting in Epstein-Barr virus, Mycoplasma pneumoniae,
decreasing vital capacity and respiratory failure H. influenzae, and HIV are the most common
 Myasthenia gravis is purely a motor disorder infectious agents that are associated with the
with no effect on sensation or coordination. development of Guillain-Barré syndrome.

ASSESSMENT AND DIAGNOSTICS SIGNS AND SYMPTOMS


 An acetylcholinesterase inhibitor test is used to
diagnose myasthenia gravis.  tingling or prickling sensations in your
 Edrophonium chloride (Tensilon), a fast-acting fingers and toes
acetylcholinesterase inhibitor, is administered IV  muscle weakness in your legs that travels to
to diagnose myasthenia gravis. your upper body and gets worse over time
 Atropine should be available to control the side  difficulty walking steadily
effects of edrophonium, which include  difficulty moving your eyes or face, talking,
bradycardia, sweating, and cramping. chewing, or swallowing
 severe lower back pain
MEDICAL MANAGEMENT  loss of bladder control
 fast heart rate
 Management of myasthenia gravis is directed at  difficulty breathing
improving function and reducing and removing  paralysis
circulating antibodies.
 administration of anticholinesterase ASSESSMENT AND DIAGNOSTICS
medications and immunosuppressive therapy,
plasmapheresis, and thymectomy. o The patient presents with symmetric weakness,
 Pyridostigmine bromide (Mestinon), an diminished reflexes, and upward progression of
anticholinesterase medication, is the first line of motor weakness.
therapy. o A history of a viral illness in the previous few
 Immunosuppressive therapy is to reduce weeks suggests the diagnosis.
production of the antibody o Changes in vital capacity and negative
inspiratory force are assessed to identify
 Corticosteroids suppress the patient’s immune
impending neuromuscular respiratory failure.
response, decreasing the amount of antibody
production
MEDICAL MANAGEMENT
 Intravenous immune globulin (IVIG) is also used
o Respiratory therapy or mechanical ventilation
to treat exacerbations, and, in selected patients,
may be necessary to support pulmonary
it is used on a long-term adjunctive basis.
function and adequate oxygenation.
 Plasmapheresis (plasma exchange) is a
o Plasmapheresis and IVIG are used to directly
technique used to treat exacerbations.
affect the peripheral nerve myelin antibody
level
SURGICAL MANAGEMENT
o The use of short-acting agents is important,
because autonomic dysfunction is very labile
Thymectomy (surgical removal of the thymus gland) can
o Hypotension is managed by increasing the
produce antigen-specific immunosuppression and result
amount of IV fluid administered.
in clinical improvement.

NURSING MANAGEMENT
Educational topics for outpatient self-care include
medication management, energy conservation,
strategies to help with ocular manifestations, and
prevention and management of complications.

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