Myasthenia Gravis (MG) o Impaired transmission of impulses

from nerve to muscles

→ A disorder affecting the neuromuscular
o Decreased muscle tone or strength or
transmission of the voluntary muscles of the
contraction
body
→ Characterized by excessive weakness &
fatigability particularly of voluntary muscles
& those innervated be cranial nerve function

Clinical Manifestation
→ Extreme muscular weakness & easy fatigability
o worsen after effort & relieve by rest
→ Symmetric muscles are involve
o First and foremost those innervated by
cranial nerve
→ Diplogia

o
→ Ptosis

o
→ Mask-like expression
Pathophysiology
→ Considered an autoimmune disease
o Antibodies are directed against
acetylcholine receptors (AChR)
o Loss of available/ normal receptors on
the postsynaptic membrane of the o
myoneural junction

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 → Act by increasing the relative concentration of
available acetylcholine at the myoneural
junction

o
→ Dysphonia
→ Difficulty in mastication & swallowing
→ Weakness of the arms & legs
→ Myasthenia crisis:
o weakness of the diaphragm &
intercostal muscles
→ an acute emergency
Diagnostic Evaluations
→ Patient’s history and physical examination
→ Injection of edrophonium (Tensilon)
o Facilitates the transmission of impulses → Drugs currently used:
at the myoneural junction (confirms the o ambenonium chloride (Mytelase)
diagnosis) o neostigmine bromide (Prostigmin)
o Within 30 seconds of IV injection of o pyridostigmine bromide (Mestinon)
Tensilon, the patient improves → the most preferred choice due to minimal side
substantially but temporarily (positive effects
result) → Common Side Effects:
o Abdominal cramps, nausea & vomiting
o Diarrhea
o Fasiculation (fine twitching), spasm &
weakness
o Irritability, anxiety, insomnia,
dysarthia, syncope, convulsion, coma
o Increased salivation & lacrimation
o Increased bronchial secretions
o o Moist skin
→ Positive AChR antibodies in the serum → Any delay in drug administration may result in
o 90% present in patient with generalized the patient’s inability to swallow.
MG
o 70% present in patient with ocular form Immunosuppressive Therapy
→ Electromyography (EMG)
→ Reduces the production of antireceptor
o Not a confirmative examination
antibody/ direct removal by plasma exchange
→ Includes:
o Corticosteroid
o Plasmapheresis
o Thymectomy
Plasma Exchange
→ Permits selective removal of the patient’s
plasma components & the remaining cells are
o reinfused
Management Surgical Management:
→ Goal: directed at improving remaining function → Thymectomy
o Anticholinesterase Drugs

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Myasthenic vs Cholinergic Crisis
→ Myasthenic Crisis:
o Sudden onset of muscular weakness in
patients with myasthenia
o usually the result of under medication
or no cholinergic medication at all
→ Cholinergic Crisis:
o Caused by overmedication with
cholinergic or anticholinesterase drugs
Nursing Interventions
→ Improve respiratory function
o Chest physiotherapy & postural
drainage
→ Increase physical mobility
o Emphasize the importance of taking
medication on time (keep a diary of s/s)
o Administer/take meds 30 mins before
meals for maximal muscle strength
o Plan adequate rest periods throughout
the day
o Setting a realistic schedule daily &
spacing of activities
o Wearing appropriate shoes to minimize
weakness & prevent injury
→ Promote communication
→ Improve vision
o Taping the eyes open for short intervals
o Instillation of artificial tears to prevent
corneal damage
o Placing a patch over one eye (diplopia)
o Wearing of sunglasses
→ Avoidance of aspiration
o Standby suction machine should be
available
o Rest before meals to lessen muscle
fatigue
o Place on upright position with neck
slightly flexed
o Soft foods than liquids
o Increase caloric intake during breakfast
o Encourage rest after eating
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o Mealtimes should coincide with the
peak effects of the anticholinesterase
(if with difficulty of swallowing)
→ Observe for signs of dyspnea, dysphagia, or
dysarthia (myasthenic crisis)
→ Have tracheostomy set at bedside
→ Instruct client to avoid people with upper
respiratory tract infections
→ Encourage to carry medical alert card
→ Do not administer morphine sulfate to client
receiving anticholinesterase (potentiate
morphine sulfate)
→ Provide emotional support & close contact to
allay anxiety
→ Administer tube feeding when necessary
→ Ensure that client understands the s/s of
myasthenic and cholinergic crisis
→ In severe instances anticipate all needs, since
the client is too weak to turn, drink, or even to
ask for assistance
Multiple Sclerosis
→ Chronic, progressive, degenerative disorder of
the CNS characterized by disseminated
demyelination of nerve fibers of the brain and
spinal cord
→ Usually affects young to middle- aged adults,
with onset between 15 and 50 years of age
→ Women affected more than men
Etiology
→ Unknown cause
→ Related to infectious, immunologic, and genetic
factors
→ Possible precipitating factors include
o Infection
o Physical injury
o Emotional stress
o Excessive fatigue
o Pregnancy
o Poor state of health
Pathophysiology
→ Mylelin sheath
o Segmented lamination that wraps
axons of many nerve cells
o Increases velocity of nerve impulse
conduction in the axons
o Composed of myelin, a substance with
high lipid content
→ Characterized by chronic inflammation,
demyelination, and gliosis (scarring) in the
CNS
→ Initially triggered by a virus in genetically
susceptible individuals
→ Subsequent antigen-antibody reaction leads to
demyelination of axons
Pathogenesis of MS o Spastic bladder: small capacity for
→ Disease process consists of loss of myelin, urine results in incontinenceFlaccid
disappearance of oligodendrocytes, and bladder: large capacity for urine and no
proliferation of astrocytes sensation to urinate
→ Changes result in plaque formation with → Sexual dysfunction
plaques scattered throughout the CNS o Erectile dysfunction
→ Initially the myelin sheaths of the neurons in the o Decreased libido
brain and spinal cord are attacked, but the nerve o Difficulty with orgasmic response
fiber is not affected o Painful intercourse
→ Patient may complain of noticeable impairment o Decreased lubrication
of function Diagnostic Studies
→ Myelin can regenerate, and symptoms → Based primarily on history, clinical
disappear, resulting in a remission manifestations, and presence of multiple
→ Myelin can be replaced by glial scar tissue lesions over time measured by MRI
→ Without myelin, nerve impulses slow down → Certain laboratory tests are used as adjuncts to
→ With destruction of axons, impulses are totally clinical exam
blocked → Diagnosis based primarily on:
→ Results in permanent loss of nerve function o history and clinical manifestations
Clinical Manifestations o ruling out other causes of symptoms
→ Vague symptoms occur intermittently over → No definitive diagnostic test
months and years → MRI – demonstrates presence of plaques
→ MS may not be diagnosed until long after the Collaborative Care
onset of the first symptom Drug Therapy
→ Characterized by → Corticosteroids
o Chronic, progressive deterioration in o Treat acute exacerbations by
some reducing edema and inflammation
o Remissions and exacerbations in others at the site of demyelination
→ Common signs and symptoms include motor, o Do not affect the ultimate outcome
sensory, cerebellar, and emotional problems or degree of residual neurologic
→ Motor manifestations impairment from exacerbation
o Weakness or paralysis of limbs, trunk, → Immunosuppressive Therapy
and head o Because MS is considered an
o Diplopia (double vision) autoimmune disease
o Scanning speech o Potential benefits counterbalanced
o Spasticity of muscles against potentially serious side
→ Sensory manifestations effects
o Numbness and tingling → Antispasmodics (muscle relaxants)
o Blurred vision → Physical therapy helps
o Vertigo and tinnitus o Relieve spasticity
o Decreased hearing o Increase coordination
o Chronic neuropathic pain o Train the patient to substitute
unaffected muscles for impaired
→ Cerebellar manifestations ones
o Nystagmus → Nutritional therapy includes megavitamins and
▪ Involuntary eye movements diets consisting of low- fat, gluten-free food,
o Ataxia and raw vegetables
o Dysarthria → High-protein diet with supplementary vitamins
▪ Lack of coordination in is often prescribed
articulating speech Nursing Assessment
o Dysphagia → Health History
▪ Difficulty swallowing o Risk factors
→ Emotional manifestations o Precipitation factors
o Anger o Clinical manifestations
o Depression Nursing Diagnoses
o Euphoria → Impaired physical mobility
Other Clinical Manifestations → Dressing/grooming self-care deficit
→ Bowel and bladder functions → Risk for impaired skin integrity
o Constipation → Impaired urinary elimination pattern
→ Sexual dysfunction
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 → Interrupted family processes o André Strohl
Nursing Planning ▪ diagnosed two soldiers with
→ Maximize neuromuscular function the illness and discovered the
→ Maintain independence in activities of daily key diagnostic abnormality of
living for as long as possible increased spinal fluid protein
→ Optimize psychosocial well-being production, but normal cell
→ Adjust to the illness
count
→ Reduce factors that precipitate exacerbations
Nursing Implementation OTHER NAMES
→ Help identify triggers and develop ways to
avoid them or minimize their effects → acute inflammatory demyelinating
→ Reassure patient during diagnostic phase polyneuropathy (AIDP)
→ Assist in dealing with anxiety caused by → acute idiopathic polyradiculoneuritis
diagnosis → acute idiopathic polyneuritis
→ Prevent major complications of immobility → French Polio
→ Focus teaching on building general resistance → Landry's ascending paralysis
to illness → Landry Guillain Barré syndrome
o Avoiding fatigue, extremes of hot and
cold, exposure to infection Pathophysiology:
→ Teach good balance of exercise and rest,
nutrition, avoidance of hazards of immobility → Presence of infection (appearance of
→ Teach self-catheterization if necessary antigens)
→ Teach adequate intake of fiber to aid in regular → Immune response to the antigens
bowel habits (antibodies)

GUILLAIN-BARRE SYNDROME Antibodies attack the gangliosides, Compounds

(POLYRADICULONEURITIS) naturally present in large quantities in human nerve
tissues
GBS
→ is an acute inflammatory demyelinating
polyneuropathy (AIDP), an autoimmune
disorder affecting the peripheral nervous
system, usually triggered by an acute infectious
process
→ It is frequently severe and usually exhibits as an
ascending symmetric flaccid paralysis noted by
weakness in the legs that spreads to the upper
limbs and the face along with complete loss of
deep tendon reflexes
→ In most cases, the syndrome is preceded by an
infection (respiratory or gastrointestinal) 1-4
weeks before the neurogical deficits
→ In some instances it has occurred after
vaccination or surgery
→ it is usually linked to infection with:
o Camylobacter jejuni
o Cytomegalovirus
o Epstein-Barr virus
o Mycoplasma pneumoniae
o Mistargeted the host nerve tissues
HISTORY
o Results into damage of the myelin
→ first described by the French physician Jean sheath of the neurons (the fatty
Landry in 1859 insulating layer of the nerve)
→ 1916 ▪ demyelination
o Georges Guillain
o Jean Alexandre Barré

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A nerve conduction block occurs Lou Gehrig's disease (ALS)

o Leading to a muscle paralysis that may

be accompanied by sensory or
autonomic disturbances
Mild Cases:
→ Nerve axon (the long slender
conducting portion of a nerve)
function remains intact and
recovery can be rapid if
remyelination occurs (80%)

CLASSIFICATION
→ Acute inflammatory demyelinating
polyneuropathy (AIDP)
• is the most common form of GBS,
and the term is often used
synonymously with GBS
• is caused by an auto-immune
response directed against Schwann
cell membranes
→ Acute motor axonal neuropathy (AMAN), aka
Chinese Paralytic Syndrome
→ o attacks motor nodes of Ranvier and is
prevalent in China and Mexico
Severe Cases:
o likely due to an auto-immune response
→ Axonal damage occurs, and recovery directed against the axoplasm of
depends on the regeneration of this peripheral nerves
important tissue o the disease may be seasonal and
recovery can be rapid
o anti-GD1a antibodies are present;
Anti-GD3 antibodies are found more
frequently in AMAN

→
Note: Guillain–Barré, unlike disorders such as multiple
sclerosis (MS) and Lou Gehrig's disease (ALS), is a
peripheral nerve disorder and does not generally cause
nerve damage to the brain or spinal cord.
o
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→ Miller Fisher syndrome (MFS)
o a rare variant of GBS and manifests as
a descending paralysis, proceeding in
the reverse order of the more common
form of GBS
o usually affects the eye muscles first and
presents with the triad
ophthalmoplegia, ataxia, and areflexia.
o Anti-GQ1b antibodies are present in
90% of cases
→ Acute Motor Sensory Axonal Neuropathy
(AMSAN)
o similar to AMAN but also affects
sensory nerves with severe axonal
damage
o it is likely due to an auto-immune
response directed against the axoplasm
of peripheral nerves
o recovery is slow and often incomplete
→ Acute Panautonomic Neuropathy
o the most rare variant of GBS,
sometimes accompanied
encephalopathy
o associated with a high mortality rate,
due to cardiovascular involvement, and
associated dysrhythmias
o the most common symptoms at onset
are related to orthostatic intolerance, as
well as gastrointestinal and sudomotor
dysfunction
→ Parasympathetic impairment may also be
observed:
o abdominal pain
o vomiting
o obstipation and ileus
o urinary retention
o dilated unreactive pupils
o loss of accommodation
→ Bickerstaff’s Brainstem Encephalitis (BBE)
o characterized by acute onset of
ophthalmoplegia, ataxia, disturbance
of consciousness, hyperreflexia or
Babinski’s sign
o the course of the disease can be
monophasic or remitting-relapsing
o large, irregular hyperintense lesions
located mainly in the brainstem,
especially in the pons, midbrain and
medulla are described
o BBE despite severe initial presentation
usually has a good prognosis
o Magnetic resonance imaging (MRI)
plays a critical role in the diagnosis of
BBE.
DIAGNOSTIC EVALUATIONS
→ Cerebrospinal Fluid Examination
▪ elevated protein level (100–
1000 mg/dL), without an
accompanying increased cell
count pleocytosis
of
o A sustained increased white blood cell
count may indicate an alternative
diagnosis such as infection
→ Electrodiagnostics
o Electromyography (EMG) and nerve
conduction study (NCS)
▪ may show prolonged distal
latencies, conduction slowing,
conduction block, and
temporal dispersion of
compound action potential in
demyelinating cases
▪ in primary axonal damage, the
findings include reduced
amplitude of the action
by
potentials without conduction
slowing
Clinical Manifestations
→ Paresthesia (tingling and numbness)
→ Muscle weakness of the legs, which may
progress to the upper extremities, trunk, and
facial muscles
→ Muscle weakness may be followed quickly by
complete paralysis
→ Cranial nerves are frequently affected:
o paralysis of the ocular, facial and
oropharhyngeal muscles
o difficulty talking, masticating, and
swallowing
Autonomic dysfunction:
→ over-reactivity or under-reactivity of the
sympathetic or parasymphatetic NS manifested
by:
▪ disturbance in heart rate and
rhythm
▪ blood pressure changes
→ vasomotor disturbances
▪ severe and persistent pain in
the back and calves of the leg
▪ loss of position change
▪ absent of tendon reflexes
Most patient make a full recovery over several months
to a year, but about 10% are left with residual
disability
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Management → Impaired physical mobility r/t
paralysis
→ considered as medical emergency and the
→ Altered nutrition, less than body
patient is managed in the ICU
requirement r/t inability to swallow
→ patient may require mechanical ventilator
secondary to cranial nerve
→ plasmapheresis (plasama exchange) –
dysfunction
o reduce circulating antibodies:
→ Impaired verbal communication r/t
▪ for severely affected patient
cranial nerve dysfunction
and deteriorating patient to
→ Fear r/t loss of control and
limit the deterioration and
paralysis
demyelination
→ continuous ECG monitoring Nursing Interventions:
→ Propranolol
→ Respiratory Function:
o for arrhythmia associated with
o care of respiratory ventilator
abnormalities (prevents tachycardia &
o suctioning of secretions
hypertension)
▪ risk for aspirating the
→ atropine sulfate
secretions
→ to avoid bradycardia during endotracheal
o chest physical therapy
suctioning and physical therapy
→ Promotion of Mobility:
NURSING PROCESS o support paralyzed extremities in a
functional position and give passive
Assessment:
range-of-motion exercise 2x daily
o Careful assessment of respiratory ▪ prevent contractures &
function deformities; DVT
→ vital capacity is monitored o administration of the prescribed
RTC to anticipate respiratory anticoagulant medications
failure o adequate hydration
→ check for weakness of muscles o padding over elbows and head of the
used for swallowing that cause fibula
difficulty in swallowing and ▪ to prevent ulnar & peroneal
coughing nerve compression
→ breathlessness while talking neuropathies
→ shallow and irregular o prevention of pressure ulcers
breathing & increased pulse → Adequate Nutrition:
rate o IV feeding will be given until bowel
→ use of accessory muscles of sounds are heard to prevent paralytic
respiration ileus
→ paradoxical inward movement o When patient can swallow normally
of the upper abdominal wall and has normal bowel sounds, oral
while in a supine position: feeding is gradually resumed
• sign of impending → Improved Communication
paralysis of the o use of lipreading and use picture cards
diaphragm with a combined system of blinking the
• Respiratory eyes to indicate yes or no may be used
deterioration is the o patient on prolonged mechanical
main threat to life of ventilator may be referred to speech-
the patient. language pathologists
→ Relief of Anxiety and Fear:
Possible Nursing Diagnosis: o involve family and friends with
selected patient care activities to reduce
→ Ineffective breathing pattern & gas
feeling of isolation
exchange r/t rapidly progressive
o activities that help increase patient’s
weakness and impending
sense of control (thus reduce fear):
respiratory failure
▪ giving information about his
condition
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 ▪ emphasizing positive appraisal
of coping resources
▪ involving in the relaxation
exercise and distraction
techniques
▪ giving positive feedback
o giving expert nursing care,
explanations, and reassurance helps the
patient gain some control over his
situation
→ Patient Education:
o caution patient on becoming fatigued
and overworking of muscles
o patient is encouraged to continue the
exercise program after discharge
o advise patient to “take one at a time”
when overwhelmed with problems of
fatigue
o refer to Guillain-Barré support groups

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