MYASTHENIA GRAVIS

Etiology and Pathophysiology

 A neuromuscular disorder in which there is a

disturbance in the transmission of impulses
from nerve to muscle cells at the
neuromuscular junction, causing extreme
muscle weakness.
 Dysfunction thought to be caused by a
reduced number of acetylcholine receptors
(AchR) and an alteration of the postsynaptic
membrane of the muscle end-plate.
 Voluntary muscles are affected, especially
those muscles innervated by the cranial
nerves.
 Cause: thought to be autoimmune disorder
whereby antibodies destroy acetylcholine
receptor sites on the postsynaptic
membrane of the neuromuscular junction.
 Highest incidence between ages 15 and 35
for women, over 40 for men.
 Myasthenic crisis refers to sudden inability
to swallow or maintain respirations
because of the weakness of the muscles of
respiration
Assessment findings

Extreme muscle weakness; becomes
progressively worse with activity, but reduc
with rest.

Dyspnea

Dysphagia (difficulty chewing and swallowi

Dysarthria (difficulty speaking)

Diplopia

Ptosis

Weak voice (dysphonia), hoarseness

Myasthenic smile (snarling nasal smile)
Diagnostic tests

 Tensilon test: IV injection of Tensilon

provides spontaneous relief of symptoms
(lasts 5-10 minutes)
 Electromyography (EMG): amplitude of
evoked potentials decreases rapidly
 Presence of antiacetylcholine receptor
antibodies in the serum
Nursing Diagnosis

 Fatigue related to profound muscle weakness

 Ineffective airway clearance related to inability to
cough or swallow
Medical Management
Drug therapy
1. Anticholinesterase drugs: ambenonium (Mytelase), neostigmine
(Prostigmin), pyridostigmine (Mestinon)
 Block action of cholinesterase and increase levels of
acetylcholine at the neuromuscular junction
 Side effects: execessive salivation and sweating, abdominal
cramps, nausea and vomitting, diarrhea, fasciculations
(muscle twitching)
2. Corticosteroid: prednisone
 Used if other drugs are not effective
 Supress autoimmune response
3. Surgery (thymectomy)
 Surgical removal of the thymus gland (thought to be involved
in the production of acetylcholine receptor antibodies)
 May cause remission in some client especially if performed
early in the disease
Nursing interventions
1. Administer anticholinesterase drugs as ordered.
 Give medication exactly on time
 Give with milk and crackers to decrease GI upset
 Monitor effectiveness of drugs: assess muscle
strength and vital capacity before and after
medication
 Avoid use of the following drugs: Morphine and
strong sedatives (respiratory depressant effect),
quinine, curare, procainamide, neomycin,
streptomycin, kanamycin and other aminoglycosides
(skeletal muscle blocking effects)
 Observe for side effects.
2. Promote optimal nutrition.
 Mealtimes should coincide with the peak effects of the
drugs; give medications 30 minutes before meals.
 Check gag reflex and swallowing ability before feeding.
 Provide a mechanical soft diet.
 If the client has difficulty chewing and swallowing, do not
leave alone at mealtimes; keep emergency airway and
suction equipment nearby.
3. Monitor respiratory status frequently: rate, depth, vital
capacity, ability to deep breathe and cough. Observe for signs
of dyspnea, dysphagia, and dysarthria; may be caused by
worsening of myasthenia (myasthenia crisis) or overdose of
anticholinergic drugs (cholinergic crisis)
4. Assess muscle strength frequently; plan activity to take advantage of energy
peaks and provide frequent rest periods

5. Observe for signs of myasthenic or cholinergic crisis.

Myasthenic crisis
 Abrupt onset of severe generalized muscle weakness with inability to
swallow, speak, or maintain respirations.
 Caused by undermedication, physical or emotional stress, infection
 Symptoms will improve temporarily with Tensilon test
Cholinergic crisis
 Symptoms similar to myasthenic crisis and, in addition, the side effects of
anticholinesterase drugs (e.g. excessive salivation and sweating,
abdominal cramps, nausea and vomiting, diarrhea, fasciculations)
 Caused by overmedication with the cholinergic (anticholinesterase) drugs
 Symptoms worsen with Tensilon test; keep atropine sulfate and
emergency equipment on hand.
 Have and emergency tracheostomy set at
bedside
 Teach to avoid people with upper respiratory
tract infections, because pneumonia may
develop as a result of respiratory impairment
 Encourage use of a medical alert card
 Avoid administering morphine to clients
receiving cholinesterase inhibitors; these drugs
potentiate effects of morphine and may cause
respiratory depression
 Provide emotional support and close client
contact to allay anxiety
 Administer tube feedings to avoid aspiration if client
has difficulty swallowing
 Administer artificial tears to keep cornea moist if
client has difficulty closing eyes
 Encourage client and family to participate in
planning care
 Ensure that client understands the signs and
symptoms of myasthenic and cholinergic crises
 Refer client and family to Myasthenia Gravis
Foundation and local self-help groups
Evaluation/Outcomes

 Maintains a balance between activity and

rest
 Maintains effective respiratory function
 Identifies signs and symptoms of crises
GUILLAIN-
BARRÉ
SYNDROME
Etiology and Pathophysiology

 Also known as Polyradiculoneuritis

 Symmetrical, bilateral, peripheral polyneuritis
characterized by ascending paralysis
 Can occur at any age; affects men and
women equally
 Progression of disease is highly individual;
90% of clients stop progression in 4 weeks;
recovery is usually from 3-6 months; may
have residual deficits
Assessment findings

 Changes in motor cells of spinal cord and medulla

with areas of demyelination
 Cause unknown; though to be linked to
immunologic status; often follows respiratory or
gastrointestinal infection; may be linked to
cytomegalovirus and Epsteinn-Barr virus
 After initial and plateau periods, recovery may
take up to a year; although most recover, some
experience residual deficits or die of complications
Assessment findings

Mild sensory changes in some clients severe misinterpreta
of sensory stimuli resulting in extreme discomfort

Clumsiness usually first symptom

Progressive motor weakness in more than limb (classically
ascending and symmetrical)

Cranial nerve involvement (dysphagia)

Ventilatory insufficiency if paralysis ascends to respiratory
muscles

Absence of deep tendon reflexes

Autonomic dysfunction

Diplopia
Objective findings

Paralysis begins in lower extremities; ascends
within the body; maximal deficit usually by 4
weeks

Respiratory paralysis

Autonomic neuropathy (e.g. hypertension,
tachycardia, diaphoresis)

Abnormal CSF and electrophysiologic studies
Diagnostic Tests


CSF studies – increased
protein

Electromyography
(EMG) - slowed nerve
conduction

Plasmapheresis –
removal, treatment
and return of blood
plasma
Assessment

 Respiratory function including airway,

respiratory rate, breath sounds , and
arterial blood gases
 Neurologic status
 History of any recent illness (particularly
viral infections)
 Onset and progression of symptoms
Nursing Diagnosis

 Impaired spontaneous
ventilation related to muscle
weakness
 Ineffective role performance
related to lengthy recovery
period
 Impaired physical mobility
related to impaired
neuromuscular function
Planning/Implementation
 Maintain adequate ventilation

Monitor depth and rate of respirations; serial vital
capacities

Observe for ventilatory insufficiency

Maintain mechanical ventilation as needed; keep
airway free of secretions and prevent pneumonia.
 Monitor vital signs, breath sounds, and arterial blood
gases and observe for signs of autonomic dysfunction
such as acute periods of hypertension fluctuating with
hypotension, tachycardia, arrythmias.
 Maintain airway and keep tracheostomy set at the
bedside
 Check individual muscle group every 2 hours in
acute phase to check for progression of muscle
weakness
 Prevent complications of immobility; skin care,
range-of-motion exercises, position changes,
coughing and deep breathing, antiembolism
stockings
 Assess cranial nerve function: check gag reflex
and swallowing ability; ability to handle
secretions; voice.
 Administer corticosteroids to suppress immune
reaction as ordered.
 Promote optimum nutrition.
 Check gag reflex before feeding.
 Start with pureed foods.
 Assess need for nasogastric tube feedings if unable to
swallow.
 Provide emotional support for the client and family
because of the severity of adaptations and lengthy
convalescent period
 Refer client and family to Guillain-Barré Foundation
for additional information and community resources
Evaluation/ Outcomes

 Maintains effective respiratory function

 Remains free from complications of
immobility
 Discusses feelings with family and other
health-team members
Thank you for listening 