MYASTHENIA GRAVIS

MYASTHENIA GRAVIS
• Rare disorder which affects neuromuscular transmission of voluntary muscles of body
• Defect in transmission of nerve impulse at neuromuscular junction
• Affects women particularly 20-30 years old
• Characterized by extreme weakness and fatigue – particularly of voluntary muscles
o Cannot hold heads up

ETIOLOGY
• Don’t know cause
• Considered to be an autoimmune disease in which antibodies act against acetylcholine
receptor site thus impairing neuromuscular transmission
• A problem at the neuromuscular junction

PATHOPHYSIOLOGY
• Due to decreased secretion of acetylcholine, or an increase in cholinesterase at myoneural
junction, the transmission of impulses from nerve to muscle cells is defective

CLINICAL MANIFESTATIONS
• Progress slow and insidious
• Extreme muscular weakness relieved by rest
• Severe fatigue that is also relieved by rest
• Diplopia
• Ptosis
o Drooping of the eyelids
• Sleepy masklike expression
o Difficulty smiling
• Weakness of speech and chocking (HRF aspiration)
• Laryngeal and pharyngeal muscle weakness
• Mouth hangs open
• Resp distress
• Maysthenic crisis

DIAGNOSIS
• Tensilon Injection Test
o Tensilon – Neastigmin, Prostigmin
o Should have great increase in muscle strength within seconds of injection, but only
temporarily
• Physician will do this test
• EMG
o Electromyography, shows weak muscles not specific to Myasthenia
MANAGEMENT
• No Known cure
• Aimed at:
o Giving anticholinesterase drug to improve remaining function
o Reducing and removing circulating antibodies
• Drug Therapy:
o Prostigmin or Mestinan
o These drugs increase the available concentration of acetylcholine at the
neuromuscular junction, thus increasing muscular response to nerve impulses to
improve strength.
o MEDS should be taken at prescribed time daily. Ideally 1 hour before meals so that
swallowing is adequate before mealtime
o Failure to adhere to this schedule may result in dyspnea, resp distress, and inability
to swallow. MEDS AS SCHEDULED
o Medication is highly individualized – patient adjust their own medication
o Avoid muscle relaxants
o Immunosuppresive therapy
 For severe cases
 May decrease production of antireceptor antibody
 Corticosteroids will suppress the patients immune response, thereby
decreasing the amount of blocking antibody
o Plasmapherises
 Will produce a temporary reduction in titer of circulating antibodies
o Thymectomy
 Surgical removal of the Thymus gland
 The thymus produce ActiR antibodies in MG Surgical removal may lead to
remission. But takes 2-10 years

MYASTHENIC CRISIS VS. CHOLINERGIC CRISIS

• Myasthenic Crisis
o Too Little Medication
 Acute Respiratory Distress
 Can’s speak or swallow
• Cholinergic Crisis
o Too Much Medication
 Acute Respiratory Distress

• It is difficult to distinguish but the 2 crises both can result in respiratory distress which is a
medical emergency.
• Also paralysis of muscles for swallowing and increase in Myasthenic symptoms
• MD will to tenslin test to see if under medicated

Emergency Management
• Keep Airway open
• Suction oral secretions
• Trach kit
• Elevate head and shoulders
• Keep calm
 • Support respiratory function as needed

PROGNOSIS
• Can live long productive life with adequate treatment
• Course of disease varies by remission and exacerbation

NURSING CARE
• Work with nutrition
• Ineffective airway clearance - Resp Assessment
o Monitor
o Keep HOB elevated
o Get meds on time
o May need a trach at bedside
• Impaired Physical Mobility - Assess weakness
• HRF Injury
• HRF Aspiration
o Small frequent feeding
o If they rest in between the energy builds up acetochylione
• Educate and support

Frequent rest periods