MEDICAL SURGICAL NURSING
NCM 116
GROUP 3-BSN3A
GUILLAIN BARRE SYNDROME
• Also known as acute idiopathic polyneuritis, is an autoimmune
attack on the peripheral nerve myelin.
• The nerve injury often causes muscle weakness, cause paralysis
and sensitivity problems, including pain, tingling or numbness
TYPES
Once thought to be a single disorder, Guillain-Barre syndrome is
now known to occur in several forms. The main types are:
- Acute Inflammatory Demyelinating Polyradiculoneuropathy
(AIDP) The most common sign of AIDP is muscle weakness that
starts in the lower part of your body and spreads upward.
- Miller Fisher Syndrome (MFS), in which paralysis starts in the
eyes. MFS is also associated with unsteady gait.
- Acute Motor Axonal Neuropathy (AMAN) and Acute Motor-
sensory Axonal Neuropathy (AMSAN)
ETIOLOGY
• Bacterial infection
• Viral infection (Campylobacter, cytomegalovirus, Epstein–
Barr virus, Mycoplasma pneumoniae, H. influenzae, and
Zika virus)
• Protozoan infection
• Surgeries
• Blood Transfusion
• Transplantation
• Anesthesia
• Preceding heat stroke
• *Preceding vaccination - Swine flu
PATHOPHYSIOLOGY
GBS is the result of a cell-mediated and humoral immune attack on
peripheral nerve myelin proteins that causes inflammatory
demyelination. The best accepted theory of cause is molecular
mimicry, in which an infectious organism contains an amino acid
that mimics the peripheral nerve myelin protein. The immune system
cannot distinguish between the two proteins and attacks and destroys
peripheral nerve myelin. The exact location of the immune attack
within the peripheral nervous system is the ganglioside GM1b. With
the autoimmune attack, there is an influx of macrophages and other
immune-mediated agents that attack myelin and cause inflammation
and destruction, interruption of nerve conduction, and axonal loss
(NINDS, 2018b). Myelin is a complex substance that covers nerves,
providing insulation and speeding the conduction of impulses from
the cell body to the dendrites. The cell that produces myelin in the
peripheral nervous system is the Schwann cell. In GBS, the Schwann
cell can be spared, allowing for remyelination in the recovery phase
of the disease. If damage has occurred to the axons, then regrowth is
required and takes months or years and is often incomplete (NINDS,
2018b).
CLINICAL MANIFESTATION
The clinical manifestations of GBS can vary, but commonly include:
1. Muscle weakness: Weakness usually starts in the legs and can
progress to the arms, face, and respiratory muscles. It may begin on
one side of the body and spread symmetrically. 2. Tingling or
numbness: Patients often experience a tingling sensation or
numbness in the extremities, such as the hands, feet, or both.
3. Loss of reflexes: Reflexes, such as the knee jerk reflex, may be
diminished or absent.
4. Pain: Some individuals with GBS may experience muscle pain or
aching sensations.
5. Difficulty with coordination and balance: It may become
challenging to maintain balance and coordination due to muscle
weakness.
6. Autonomic dysfunction: GBS can affect the autonomic nervous
system, leading to symptoms like fluctuations in blood pressure,
heart rate abnormalities, sweating abnormalities, and bowel or
bladder dysfunction.
7. Breathing difficulties: Severe cases of GBS may result in
respiratory muscle weakness, leading to breathing difficulties and
the need for ventilatory support.
DIAGNOSIS
1. Lumbar puncture (spinal tap): This involves the collection of
cerebrospinal fluid (CSF) through a needle inserted into the lower
back. Analysis of the CSF can reveal elevated protein levels, which
is a characteristic finding in GBS.
2. Nerve conduction studies (NCS): NCS measures the electrical
activity in the peripheral nerves. In GBS, nerve conduction studies
often show decreased nerve conduction velocity and abnormal
nerve responses.
3. Electromyography (EMG): EMG measures the electrical activity
of muscles. It can help identify abnormal patterns of muscle
activity associated with GBS.
4. Blood tests: Various blood tests may be conducted to rule out
other potential causes of similar symptoms, such as infections or
autoimmune disorders. These tests can include a complete blood
count (CBC), comprehensive metabolic panel (CMP), and specific
antibody tests.
5. Ganglioside antibody testing: Certain antibodies, such as anti-
GM1 antibodies, are associated with specific variants of GBS.
Testing for these antibodies may be performed in certain cases.
TREATMENT
• Monitor respirations and support ventilation if necessary.
• Plasmapheresis for plasma exchange to remove the antibodies
in the circulation.
• Administer immunoglobulin intravenously after drawing labs
for serum IgA.
• NG tube feeding if swallowing is a problem.
NURSING RESPONSIBILITIES
• Monitor gag reflex.
• Monitor for visual changes.
EXCLUSIVELY FOR GROUP 3-BSN3A ONLY | Page 1 of 10
 MEDICAL SURGICAL NURSING
NCM 116
GROUP 3-BSN3A
• Monitor for communication ability; the patient may need
special method to communicate with staff if not able to use
call bell system.
• Turn and reposition.
• Consult with social worker or chaplain for support services
available to patient. Explain to the patient:
• Importance of turning and positioning.
• Care of the plasmapheresis access site.
• Importance of planning for home care needs
Multiple sclerosis
Multiple sclerosis (MS) is a chronic and progressive
condition that involves demyelination of the
myelinated neurons in the central nervous system. This
is caused by an inflammatory process involving the
activation of immune cells against the myelin.
MS typically presents in young adults (under 50 years)
and is more common in women. Symptoms tend to
improve in pregnancy and in the postpartum period.
Quick Facts about Multiple Sclerosis:
* It’s an autoimmune condition, which means the
immune system is actually attacking the myelin sheath
found on the nerve.
* It affects the nerve cells in the brain and spinal cord,
and this leads to many sensory and motor type
problems.
* Symptoms vary among patients because different
areas of the central nervous system are affected.
* For example, if the patient has lesions (damage
to the myelin sheath)in the cerebellar area, the
patient may experience tremors, dysarthria (issues
with articulating words…muscles for speech aren’t
working well), ataxia (trouble controlling body
movements), and cognitive issues.
* Furthermore, if the nerve to the eyes (optic nerve)
is being affected the patient will have vision issues like
blurry vision or blindness in one eye etc.
* Women tend to be affected more than men, and
MS seems to show up in the age category of 20-40s.
* Symptoms can appear and then disappear. This is the
most common form of MS where signs and symptoms
come and go called: relapsing-remitting multiple
sclerosis (RRMS)
* Exact cause is not totally known
* There is currently no cure, but there are lifestyle
changes and medications that can improve signs and
symptoms.
Signs and Symptoms of Multiple Sclerosis
Afferent Pupillary Defect (Anisocoria): occurs in
optic neuritis if the other eye is uninvolved and
otherwise healthy.
Emotionally and cognitive:
• Drained (feel weak), fatigued, depressed,
trouble articulating speech (issues
swallowing), mood swings, trouble thinking
(focusing, solving, keeping thoughts etc.)
Sensation issues:
• involuntary tremors, spasms (painful and
strong), clumsiness (leads to unintentional
injury), numbness/tingling (face and
extremities)
• electric shock sensation that travels down the
body when moving head or neck in various
position called “Lhermitte’s sign”
• dizzy, muscles hard to move (stiff)….affects
coordination (cerebellum area)
• unable to be aware of body positon
(proprioception) when eyes are
closed….Romberg’s Sign…patient puts feet
together and closes eye…this causes them to
sway
Vision:
• nystagmus (issues with controlling eye
movement), optic neuritis (early) double
vision…blurry vision or vision is gray (dull
colors), blindness in one eye, and seeing dark
spots in vision, painful when moving eyes
Elimination
• (nerves are affected that control the
bladder/bowel and their sphincters): can’t
hold urine….overactive bladder (incontinence)
leads to nocturia problems
urinating…hesitancy leads to retaining urine
(at risk for UTI’s and renal stones) bowel:
constipation/diarrhea or incontinence
Early signs and symptoms of MS include:
• vision issues
• tingling numbness
• weakness
• dizziness
• balance issues
• bladder problems
• cognitive issue: speaking, weakness, spasms
Symptoms can get worse due to heat called Uhthoff’s
sign. Heat can be from the weather, physical exercise
etc.
EXCLUSIVELY FOR GROUP 3-BSN3A ONLY | Page 2 of 10
 MEDICAL SURGICAL NURSING
NCM 116
GROUP 3-BSN3A
How is Multiple Sclerosis Diagnosed
Diagnosing (takes time): the neurologist has to assess
several things because there is not one test that can
diagnose it:
• Assessing patient’s symptoms…may need to
rule out other diseases
• MRI to assess for lesions in the brain and
spinal cord
• Lumbar puncture: assesses spinal fluid for
specific proteins called oligoclonal bands
(which are immunoglobulins). If these are
present it shows there is inflammation in the
CNS.
• Evoked potential studies (sends electrical
signals to the CNS and sees the response).
Pathophysiology of Multiple Sclerosis
Dendrites:
Receive the signal needed to create some type of
action. This signal goes down to the:
Soma:
(Which means body) and this structure helps pass on
the signal it just received from the dendrites to the rest
of the neuron.
Then the signal goes down and passes where the soma
of the neuron and axon connect at the axon hillock.
Then the signal goes down this long area known as the
axon. For the axon to be able to deliver this signal
properly to either another neuron, muscle, or gland, it
must be nicely be insulated and protected by the
myelin sheath, which is made up of oligodendrocytes.
These cells consist of fats and proteins.
• This is our problem with MS (the myelin
sheath has experienced demyelination)…..so
guess what?! The signal is NOT being
transmitted properly to the area that the nerve
supplies!!
After the signal leaves the axon in a healthy neuron it
goes to the axon terminal (the end of the axon) where
it synapses (where a nerve signal passes) with another
neuron, muscle or glands to cause an action of some
type.
So, in MS we’re talking about the nerve cells in the
CNS, which is our BRAIN and SPINAL
CORD…..because of this we can expect to finding
sensory type problems (touch, vision), coordination,
emotional, cognitive, and bowel/bladder issues
For sign and symptoms let’s divide them by category.
Remember signs and symptoms vary in patients
depending on where the lesions have occurred due to
demyelination.
Nursing Interventions for Multiple Sclerosis
Nursing considerations: safety (vision, coordination,
decrease perception with pain), RRMS (most common
form of MS)….preventing symptoms from worsening,
bladder and bowel issues, medications
Preventing symptoms from getting worse:
• Watch the heat (keep room cool, avoid heating
blankets, pads etc.), avoid infection, stressful
events, and getting too tired…overexertion (pace
out activities and take time to have many rest
periods)
• Very important to maintain regular exercise as
tolerated…not too much because it can exacerbate
symptoms (swimming…water aerobics, keeps
energy and mood level up)
EXCLUSIVELY FOR GROUP 3-BSN3A ONLY | Page 3 of 10
 MEDICAL SURGICAL NURSING
NCM 116
GROUP 3-BSN3A
• Use assistive devices to help with walking and
preventing injury (toileting and showering) when
symptoms are presenting, clutter free environment,
especially when vision affected or experiencing
vertigo, scan environment if experiencing
blindness in one eye or dark spots
• Consult SLP (helps with speech if speech is
slurred or hard to understand, difficult
swallowing), PT (exercises, assistive devices),
support groups with others who have MS
• Bladder and bowel: make accessibility to
bathroom easy due to overactive bladder, may
need to learn how to self-cath if retaining urine,
plenty of fluids to prevent stasis of urine and to
keep it from becoming too concentrated 1-2 L,
high fiber to prevent constipation…stool softeners
Medications for Multiple Sclerosis
Medications vary depending on what symptoms the
patient is having…medications treat symptoms….
don’t cure disease
• Beta interferon: decreases the number of
relapses of symptoms by decreasing
inflammation and the immune system response
….risk of infection because decreases WBC
• Drug Names: Avonex (interferon beta 1a),
Rebif, Betaferon
• Corticosteroids: for relapses of
symptoms…methylprednisolone (solu-
medrol), prednisone
• Bladder issues:
• Oxybutynin: anticholinergic that helps with
an overactive bladder…relaxes bladder to
prevent contractions
• Bethanechol: cholinergic that helps with
completely emptying the bladder by helping
bladder contract fully.
• Fatigue: Amantadine (antiviral and
antiparkinson but has CNS effects. This helps
improve fatigue in MS patients….another drug
Modafinil (CNS stimulant)
• Spasms: baclofen (skeletal muscle relaxants
that act centrally), diazepam
• Tremors: propranolol (beta blocker), isoniazid
(antibiotic used to treat infection, especially
TB…helps with certain tremors in MS)
EXCLUSIVELY FOR GROUP 3-BSN3A ONLY | Page 4 of 10
PARKINSON’S DISEASE
• PD is a slowly progressing neurologic movement disorder
that eventually leads to disability.
• The disease affects men more often than women.
Symptoms usually first appear in the fifth decade of life;
however, cases have been diagnosed as early as 30 years
of age.
• The degenerative or idiopathic form of PD is the most
common; there is also a secondary form with a known or
suspected cause.
• Although the cause of most cases is unknown, research
suggests a multifactorial combination of age,
environment, and heredity (AANN, 2019).
PATHOPHYSIOLOGY
• PD is associated with decreased levels of dopamine
resulting from degeneration of dopamine storage cells in
the substantia nigra in the basal ganglia region of the brain.
• Fibers or neuronal pathways project from the substantia
nigra to the corpus striatum, where neurotransmitters are
vital to the control of complex body movements.
• Through the neurotransmitters acetylcholine
(excitatory) and dopamine (inhibitory), striatal
neurons relay messages to the higher motor centers that
control and refine motor movements.
• The loss of dopamine stores in this area of the brain results
in more excitatory neurotransmitters than inhibitory
neurotransmitters, leading to an imbalance that affects
voluntary movement (Hickey & Strayer, 2020).
• Clinical symptoms do not appear until 60% of the
pigmented neurons are lost and the striatal dopamine level
is decreased by 80%.
• Cellular degeneration impairs the extrapyramidal tracts
that control semiautomatic functions and coordinated
movements; motor cells of the motor cortex and the
pyramidal tracts are not affected.
• Researchers are working on uncovering the exact
mechanism of neurodegeneration.
• Current theories suggest a combined and complicated
interweaving of both environmental and genetic factors
that affect numerous fundamental cellular processes.
• Fifteen percent of early PD cases are associated with
multiple genetic mutations (Poewe, Seppi, Tanner, et al.,
2017).
• Ongoing research includes recognition of biomarkers and
development of individualized treatment options (Poewe
et al., 2017).
• Figure 65-3 • Pathophysiology of Parkinson’s disease. The
nuclei in the substantia nigra project fibers to the corpus
 MEDICAL SURGICAL NURSING
NCM 116
GROUP 3-BSN3A
striatum. The nerve fibers carry dopamine to the corpus ➢ constipation
striatum. The loss of dopamine nerve cells from the brain’s ➢ sexual dysfunction
substantia nigra is thought to be responsible for the ➢ gastric and urinary retention
symptoms of Parkinson’s disease. ➢ Dysphagia is a substantial problem
➢ reporting choking as well as vision
CLINICAL MANIFESTATIONS ➢ olfactory changes
• PD has a gradual onset, and symptoms progress slowly • Psychiatric changes
over a chronic, prolonged course. The cardinal signs are ➢ Depression
tremor, rigidity, bradykinesia/akinesia, and postural ➢ Anxiety
instability (Hickey & Strayer, 2020). ➢ Dementia
• Two major subtypes of PD are tremor dominant (most ➢ Delirium
other symptoms are absent) and nontremor dominant ➢ Hallucinations
(akinetic-rigid and postural instability). ➢ Psychosis
* Depression and anxiety are common
* In addition, auditory and visual hallucinations have been
Tremor reported in people with PD and may be associated with
depression, dementia, lack of sleep, or adverse effects of
• Variable, a slow, unilateral resting tumor medications.
• Resting tremor characteristically disappears with • Stress, medications, and depression contribute to the
purposeful movement and during sleep but is evident cognitive changes of diminished executive functions,
when the extremities are motionless or at rest. attention difficulties, decreased thinking, and word-
• The tremor may manifest as a rhythmic, slow turning
motion (pronation–supination) of the forearm and the
hand and a motion of the thumb against the fingers as
if rolling a pill between the fingers.

Rigidity
• Resistance to passive limb movement characterizes
muscle rigidity.
• Passive movement of an extremity may cause the limb to
move in jerky increments, referred to as lead-pipe or
cogwheel movements. Involuntary stiffness of the passive
extremity increases when another extremity is engaged in
voluntary active movement.
• Stiffness of the arms, legs, face, and posture are
common. Early in the disease, the patient may complain
of shoulder pain due to rigidity (Hickey & Strayer,
2020).

Bradykinesia
• A common feature of PD is bradykinesia, which refers to
the overall slowing of active movement (Bronner &
Korczyn, 2017).
• Patients may also take longer to complete activities and
have difficulty initiating movement, such as rising from a
sitting position or turning in bed.

Postural Instability

• The patient commonly develops postural and gait

problems.
• Due to a loss of postural reflexes, the patient stands with
the head bent forward and walks with a propulsive
gait. The posture is caused by the forward flexion of the
neck, hips, knees, and elbows. The patient may walk
faster and faster, trying to move the feet forward under
the body’s center of gravity (shuffling gait).
• Difficulty in pivoting causes loss of balance, either
forward (propulsion) or backward (retropulsion). Gait
impairment and postural instability place the patient
at increased risk for falls (Hickey & Strayer, 2020). finding challenges.
• More than 80% of patients with a 20-year disease duration
Other Manifestations of PD experience dementia, a broad term for a syndrome
characterized by a general decline in higher brain
functioning, such as reasoning, with a pattern of
• The effect of PD on the basal ganglia often produces eventual decline in ability to perform even basic ADLs,
autonomic symptoms that include such as toileting and eating.
➢ excessive and uncontrolled sweating
➢ drooling
➢ paroxysmal flushing
➢ orthostatic hypotension

EXCLUSIVELY FOR GROUP 3-BSN3A ONLY | Page 5 of 10

 MEDICAL SURGICAL NURSING
NCM 116
GROUP 3-BSN3A
• Hypokinesia (abnormally diminished movement) is also
common and may appear after the tremor.
• The freezing phenomenon refers to a transient inability to
perform active movement and is thought to be an extreme
form of bradykinesia.
• The patient tends to shuffle and exhibits a decreased
arm swing as well. As dexterity declines, micrographia
(small handwriting) develops.
• The face becomes increasingly masklike and
expressionless, and the frequency of blinking decreases.
• Dysphonia (voice impairment or altered voice
production) may occur as a result of weakness and
incoordination of the muscles responsible for speech.
In many cases, the patient develops dysphagia, begins to
drool, and is at risk for choking and aspiration (AANN,
2019).
• Complications associated with PD are common and are
typically related to disorders of movement. As the disease
progresses, patients are at risk for respiratory and
urinary tract infection, skin breakdown, and injury
from falls.
• The adverse effects of medications used to treat the
symptoms are associated with numerous complications
such as dyskinesia (impaired ability to execute
voluntary movements) or orthostatic hypotension.
Assessment and Diagnostic Findings
• Although laboratory tests and imaging studies are not
helpful to the provider in diagnosing PD, ongoing research
with PET and single-photon emission CT scanning has
been helpful in understanding the disease and advancing
treatment.
• Currently, the disease is diagnosed clinically from the
patient’s history and the presence of two of the four
*Cardinal manifestations: tremor, rigidity, bradykinesia, and
postural changes.
• Early diagnosis can be challenging because patients rarely
are able to pinpoint when the symptoms started.
• Often, a family member notices a change such as stooped
posture; a stiff arm; a slight limp; tremor; or slow,
small handwriting.
• The medical history, presenting symptoms, neurologic
examination, and response to pharmacologic management
are carefully evaluated when making the diagnosis.
• Diagnosis is often confirmed by a positive response to a
levodopa trial (Hickey & Strayer, 2020).
• The Revised Movement Disorder Society Unified
Parkinson Disease Rating Scale (MDS-UPDRS) is a
helpful assessment tool as it measures the disease
progression including motor and nonmotor symptoms, and
includes treatment complications (AANN, 2019).
MEDICAL MANAGEMENT
• Treatment is directed toward controlling symptoms and
maintaining functional independence, because no medical
or surgical approaches in current use prevent disease
progression (AANN, 2019).
• Care is individualized for each patient based on presenting
symptoms and social, occupational, and emotional needs.
• Pharmacologic management is the mainstay of treatment,
although advances in research have led to more surgical
options.
• Patients are usually cared for at home and are admitted to
the hospital only for complications or to initiate new
treatments.
Pharmacologic Therapy
• Antiparkinsonian medications act by increasing striatal
dopaminergic activity; reducing the excessive influence of
excitatory cholinergic neurons on the extrapyramidal tract,
thereby restoring a balance between dopaminergic and
cholinergic activities; or acting on neurotransmitter
pathways other than the dopaminergic pathway.
• Levodopa is the most effective agent and the mainstay of
treatment. Levodopa is converted to dopamine in the basal
ganglia, producing symptom relief.
• Carbidopa is often added to levodopa to avoid metabolism
of levodopa before it can reach the brain.
• The beneficial effects of levodopa therapy are most
pronounced in the first year or two of treatment. Benefits
begin to wane and adverse effects become more severe
over time (Hickey & Strayer, 2020).
• Within 5 to 10 years, most patients develop a response to
the medication characterized by dyskinesia including
facial grimacing, rhythmic jerking movements of the
hands, head bobbing, chewing and smacking
movements, and involuntary movements of the trunk and
extremities.
• The patient may experience an on–off syndrome in which
sudden periods of near immobility (“off effect”) are
followed by a sudden return of effectiveness of the
medication (“on effect”).
• Changing the drug dosing regimen or switching to other
drugs may be helpful in minimizing the on–off syndrome.
• Other potential adverse effects include
➢ Nausea
➢ Vomiting
➢ appetite loss
➢ decreased Bp
➢ dystonia
➢ dyskinesia
➢ confusion
• To minimize adverse effects of levodopa over time,
current practice includes delaying use of levodopa-
containing drugs as long as possible, with the use of
other drugs for symptom control in the interim.
Surgical Management
• The limitations of levodopa therapy, improvements in
surgical techniques, and new approaches in
transplantation have renewed interest in the surgical
treatment of PD.
• In patients with disabling tremor, rigidity, or severe
levodopainduced dyskinesia, surgery may be
considered.
• Although surgery provides symptom relief in select
patients, it has not been shown to alter the course of the
disease or to produce permanent improvement.
Stereotactic Procedures
• Thalamotomy and pallidotomy are ablative procedures
that were formerly used to relieve symptoms of PD such
as tremors.
• However, these procedures permanently destroy brain
tissue and are rarely used today.
• Deep brain stimulation (DBS) has largely replaced
ablative procedures in the surgical treatment of PD. DBS
involves surgical implantation of an electrode into the
brain in either the globus pallidus or subthalamic
nucleus.
EXCLUSIVELY FOR GROUP 3-BSN3A ONLY | Page 6 of 10
 MEDICAL SURGICAL NURSING
NCM 116
GROUP 3-BSN3A
• Stimulation of these areas may increase dopamine release
or block anticholinergic release, thereby improving
tremor and rigidity.
• Levodopa medication dose may be able to be reduced,
thus improving dyskinesias.
• Patients eligible for DBS are those who have responded to
levodopa but are impaired by dyskinesias, have had the
disease for at least 5 years, and are disabled by tremor.
• Patients with dementia and atypical PD are usually not
considered for surgical procedures.
• PD rating scales and specific neurologic tests are used to
identify patients who are eligible.
• Surgical treatment typically occurs 10 to 13 years after
diagnosis (AANN, 2019).
• A CT or MRI scan is used to localize the appropriate
surgical site in the brain.
• Then, the patient’s head is positioned in a stereotactic
frame.
• After the surgeon makes an incision in the skin and a
burr hole, an electrode is passed through to the target area
to the subthalamic nuclei or globus pallidus.
• The desired response of the patient to the electrical
stimulation (i.e., a decrease in rigidity) is used to
confirm electrode placement.
• Electrode placement is completed on one side of the
brain at a time; bilateral electrodes are usually placed
(AANN, 2019).
• Electrodes are then connected to a pulse generator that is
implanted in a subcutaneous subclavicular or
abdominal pouch.
• The battery-powered pulse generator sends
highfrequency electrical impulses through a wire placed
under the skin to a lead anchored to the skull.
• These devices are not without complications that can
result from both the surgical procedure needed for
implantation (e.g., weakness, paresthesias, confusion,
hemorrhage) and the device itself (e.g., infection, lead
leakage) (Hickey & Strayer, 2020).
Neural Transplantation
• Ongoing research is exploring transplantation of porcine
neuronal cells, human fetal cells, and stem cells to replace
degenerated striatal cells (Kirkeby, Parmar, & Barker,
2017).
• Legal, ethical, and political concerns surrounding the use
of fetal brain cells and stem cells have limited the
exploration of these procedures.
MYASTHENIA GRAVIS
• An autoimmune disorder affecting the myoneural
junction, is characterized by varying degrees of weakness
of the voluntary muscles.
• More women than men are affected, commonly in the
second and third decades of life; however, after age 50, the
gender distribution is more equal.
Pathophysiology
• Normally, a chemical impulse precipitates the release of
acetylcholine from vesicles on the nerve terminal at the
myoneural junction.
• The acetylcholine attaches to receptor sites on the motor
endplate and stimulates muscle contraction.
• Continuous binding of acetylcholine to the receptor site is
required for muscular contraction to be sustained.
• In myasthenia gravis, antibodies directed at the
acetylcholine receptor sites impair transmission of
impulses across the myoneural junction.
• Therefore, fewer receptors are available for stimulation,
resulting in voluntary muscle weakness that escalates with
continued activity.
• These antibodies are found in 80% to 90% of people with
myasthenia gravis.
• Of people with myasthenia gravis, 75% have either thymic
hyperplasia or a thymic tumor, and the thymus gland is
believed to be the site of antibody production.
• In patients who are antibody negative, researchers believe
that the offending antibody is directed at a portion of the
receptor site rather than the whole complex.
CLINICAL MANIFESTATIONS
• The initial manifestation of myasthenia gravis in 80% of
patients involves the ocular muscles.
• Diplopia and ptosis (drooping of the eyelids) are
common.
• Many patients also experience weakness of the muscles of
the face and throat (bulbar symptoms) and generalized
weakness.
• Weakness of the facial muscles results in a bland facial
expression.
• Laryngeal involvement produces dysphonia (voice
impairment) and dysphagia, which increases the risk of
choking and aspiration.
EXCLUSIVELY FOR GROUP 3-BSN3A ONLY | Page 7 of 10
 MEDICAL SURGICAL NURSING
NCM 116
GROUP 3-BSN3A
• Generalized weakness affects all extremities and the
intercostal muscles, resulting in decreasing vital capacity
and respiratory failure.
• Myasthenia gravis is purely a motor disorder with no
effect on sensation or coordination.
ASSESSMENT AND DIAGNOSTIC FINDINGS
• A common test used to diagnose myasthenia gravis is the
acetylcholinesterase inhibitor test.
• It is performed by administering edrophonium chloride
(Tensilon) IV; 30 seconds after injection, facial muscle
weakness and ptosis should resolve for about 5 minutes.
• Immediate improvement in muscle strength after
administration of this agent represents a positive test and
usually confirms the diagnosis.
• Atropine should be available to control potential side
effects of this medication, which include bradycardia,
asystole, bronchoconstriction, sweating, and cramping.
• Another study, the ice test, is indicated in patients who
have cardiac conditions or asthma. With this test, an ice
pack is held over the patient’s eyes for 1 minute; the ptosis
should temporarily resolve in a patient with myasthenia
gravis.
• Several blood tests for acetylcholine antibodies are also
used to confirm the diagnosis.
• Repetitive nerve stimulation (RNS) demonstrates a
decrease in successive action potentials.
• A single-fiber electromyography (EMG) detects a delay or
failure of neuromuscular transmission and is about 99%
sensitive in confirming the diagnosis of myasthenia
gravis.
• This is an uncomfortable test for the patient.
• The thymus gland, a site of acetylcholine receptor
antibody production, may be enlarged in myasthenia
gravis and may be identified by MRI scan.
MEDICAL MANAGEMENT
• Management of myasthenia gravis is directed at
improving function and reducing and removing
circulating antibodies.
• Therapeutic modalities include administration of
anticholinesterase medications and immunosuppressive
therapy, intravenous immune globulin (IVIG), therapeutic
plasma exchange (TPE), and thymectomy.
• There is no cure for myasthenia gravis; treatments do not
stop the production of the acetylcholine receptor
antibodies.
PHARMACOLOGIC THERAPY
• Pyridostigmine bromide (Mestinon), an anticholinesterase
medication, is the first line of therapy.
• It provides symptomatic relief by inhibiting the
breakdown of acetylcholine and increasing the relative
EXCLUSIVELY FOR GROUP 3-BSN3A ONLY | Page 8 of 10
concentration of available acetylcholine at the
neuromuscular junction.
• The dosage is gradually increased to a daily maximum and
is given in divided doses (usually four times a day).
• Adverse effects of anticholinesterase medications include
diarrhea, abdominal cramps, and/or excessive saliva.
• Pyridostigmine tends to have fewer side effects than other
anticholinesterase medications.
• If pyridostigmine bromide does not improve muscle
strength and control fatigue, the next agents used are the
immunomodulating drugs.
• The goal of immunosuppressive therapy is to reduce
production of the antibody.
• Corticosteroids suppress the patient’s immune response,
decreasing the amount of antibody production, and this
correlates with clinical improvement.
• An initial dose of prednisone is given daily and maintained
for 1 to 2 months; as symptoms improve, the medication
is tapered.
• As the corticosteroid medications take effect, the dosage
of anticholinesterase medication can usually be lowered.
• Cytotoxic medications are used to treat myasthenia gravis
if there is inadequate response to steroids. Azathioprine
(Imuran) inhibits T lymphocytes and Bcell proliferation
and reduces acetylcholine receptor antibody levels.
• Therapeutic effects may not be evident for 3 to 12 months.
• Leukopenia and hepatotoxicity are serious adverse effects,
so monthly evaluation of liver enzymes and white blood
cell count is necessary.
• Intravenous immune globulin (IVIG) can be used to treat
exacerbations; however, in selected patients, it is used on
a long-term adjunctive basis.
• IVIG treatment involves the administration of pooled
human gammaglobulin and improvement occurs in a few
days.
• The effects of IVIG typically last only about 28 days after
infusion, and complications include headache, migraine
exacerbation, aseptic meningitis, and flulike symptoms.
• A number of medications are contraindicated for patients
with myasthenia gravis because they exacerbate the
symptoms.
• The primary provider and the patient should weigh risks
and benefits before any new medications are prescribed.
• Procaine (Novocaine) should be avoided, and the patient’s
dentist is informed of the diagnosis of myasthenia gravis.
THERAPEUTIC PLASMA EXCHANGE
• A technique called TPE, formerly referred to as
plasmapheresis, is used to treat exacerbations. The
patient’s plasma and plasma components are removed
through a centrally placed large-bore double-lumen
catheter.
• The blood cells and antibody-containing plasma are
separated, after which the cells and a plasma substitute are
reinfused.
• Temporary reduction in the level of circulating antibodies
is provided with TPE.
• A typical course consists of daily or alternate-day
treatment, and the number of treatments is determined by
the patient’s response.
• Symptoms improve in 75% of patients undergoing TPE;
however, improvement lasts only a few weeks after
treatment is completed.
SURGICAL MANAGEMENT
• Thymectomy (surgical removal of the thymus gland) can
produce antigenspecific immunosuppression and result in
clinical improvement.
 MEDICAL SURGICAL NURSING
NCM 116
GROUP 3-BSN3A
• This surgery is frequently performed in patients younger
than 65 years who have had myasthenia gravis diagnosed
within the past 3 years.
• It is the only treatment that can result in complete
remission, which occurs in approximately 35% of patients.
• Improvements may take several months to several years
after surgery to occur.
• A course of preoperative TPE decreases the time needed
for postoperative mechanical ventilation.
• The entire thymus gland must be removed for optimal
clinical outcomes. There are three surgical approaches:
transsternal, transcervical thymectomy, and video-assisted
thoracoscopic surgery (AANN, 2013).
• After surgery, the patient is monitored in an intensive care
unit, with special attention to respiratory function.
• The patient is weaned from mechanical ventilation after
thorough respiratory assessment.
• After the thymus gland is removed, it may take up to 3
years for the patient to benefit from the procedure because
of the long life of circulating T cells.
• The earlier the surgery in the disease process, the better
the prognosis.
COMPLICATIONS
• A myasthenic crisis is an exacerbation of the disease
process characterized by severe generalized muscle
weakness and respiratory and bulbar weakness that may
result in respiratory failure.
• Crisis may result from disease exacerbation or a specific
precipitating event.
• The most common precipitator is respiratory infection;
others include medication change, surgery, pregnancy, and
medications that exacerbate myasthenia.
• A cholinergic crisis caused by overmedication with
cholinesterase inhibitors is rare.
• Neuromuscular respiratory failure is the critical
complication in myasthenic and cholinergic crises.
• Respiratory muscle and bulbar weakness combine to cause
respiratory compromise.
• Weak respiratory muscles do not support inhalation.
• An inadequate cough and an impaired gag reflex, caused
by bulbar weakness, result in poor airway clearance.
• A downward trend of two respiratory function tests, the
negative inspiratory force and vital capacity, is the first
clinical sign of respiratory compromise.
• Endotracheal intubation and mechanical ventilation may
be needed.
• Noninvasive positive-pressure ventilation uses an external
device in the form of a vest that provides respiratory
support without endotracheal intubation.
• Cholinesterase inhibitors are stopped when respiratory
failure occurs and gradually restarted after the patient
demonstrates improvement with a course of TPE or IVIG.
• Nutritional support may be needed if the patient is
intubated for a long period or swallowing ability is
affected.
NURSING MANAGEMENT
• Because myasthenia gravis is a chronic disease and most
patients are seen on an outpatient basis, much of the
nursing care focuses on patient and family education.
• Educational topics for outpatient self-care include
medication management, energy conservation, strategies
to help with ocular manifestations, and prevention and
management of complications.
• Medication management is a crucial component of
ongoing care.
• Understanding the actions of the medications and taking
them on schedule is emphasized, as are the consequences
EXCLUSIVELY FOR GROUP 3-BSN3A ONLY | Page 9 of 10
of delaying medication and the signs and symptoms of
myasthenic and cholinergic crises.
• The patient can determine the best times for daily dosing
by keeping a diary to determine fluctuation of symptoms
and to learn when the medication is wearing off.
• The medication schedule can then be manipulated to
maximize strength throughout the day.
• Regular administration of IVIG or subcutaneous
immunoglobulin (SCIG) may be prescribed. The patient
and family are educated about managing immunoglobulin
therapy.
AMYOTROPHIC LATERAL SCLEROSIS
• ALS is a disease of unknown cause in which there is a loss
of motor neurons (nerve cells controlling muscles) in the
anterior horns of the spinal cord and the motor nuclei of
the lower brainstem.
• It is often referred to as Lou Gehrig disease, after the
famous baseball player who suffered from the disease.
• As motor neuron cells die, the muscle fibers that they
supply undergo atrophic changes.
• Neuronal degeneration may occur in both the upper and
lower motor neuron systems.
• The leading theory held by researchers is that
overexcitation of nerve cells by the neurotransmitter
glutamate results in cell injury and neuronal degeneration.
• Risk factors that have been identified include:
o smoking,
o viral infections,
o autoimmune disease, and
o environmental exposures to toxins
• For example, veterans who deployed during the 1991 Gulf
War had an increased number of cases of ALS compared
to those who did not serve in that region.
• However, the exact cause is still unknown and requires
further exploration.
• ALS most commonly occurs between 40 and 60 years of
age and affects all social, racial, and ethnic backgrounds,
with men being affected at slightly higher rates than
women.
• The majority of cases of ALS arise sporadically, but 5% to
10% of cases are familial ALS resulting from an
autosomal dominant trait carried by one parent.
CLINICAL MANIFESTATIONS
• Clinical manifestations depend on the location of the
affected motor neurons, because specific neurons activate
specific muscle fibers.
• The chief symptoms are:
o fatigue,
o progressive muscle weakness,
o cramps,
o fasciculations (twitching), and
o lack of coordination.
• Loss of motor neurons in the anterior horns of the spinal
cord results in progressive weakness and atrophy of the
muscles of the arms, trunk, or legs.
• Spasticity usually is present, and the deep tendon stretch
reflexes become brisk and overactive.
• Usually, the function of the anal and bladder sphincters
remains intact, because the spinal nerves that control
muscles of the rectum and urinary bladder are not affected.
• In about 25% of patients, weakness starts in the muscles
supplied by the cranial nerves, and difficulty in talking,
swallowing, and ultimately breathing occurs.
• When the patient ingests liquids, soft palate and upper
esophageal weakness cause the liquid to be regurgitated
through the nose.
• Weakness of the posterior tongue and palate impairs the
ability to:
 MEDICAL SURGICAL NURSING
NCM 116
GROUP 3-BSN3A
o laugh, the decision about whether to undergo a tracheotomy for
o cough, or long-term mechanical ventilation.
o even blow the nose. • A patient experiencing aspiration and swallowing
• If bulbar muscles are impaired, speaking and swallowing difficulties may require enteral feeding.
are progressively difficult, and aspiration becomes a risk. • A PEG tube is inserted before the forced vital capacity
• The voice assumes a nasal sound, and articulation drops below 50% of the predicted value.
becomes so disrupted that speech is unintelligible. The tube can be safely placed in patients who are using noninvasive
• Some emotional lability may be present. positive-pressure ventilation for ventilatory support.
• It was traditionally believed that ALS spared cognitive
function, but it is now recognized that some patients
experience cognitive impairment.
• The prognosis generally is based on the area of CNS
involvement and the speed with which the disease
progresses.
• Eventually, respiratory function is compromised.
• Death usually occurs as a result of infection, respiratory
insufficiency, or aspiration.

ASSESSMENT AND DIAGNOSTIC FINDINGS

• ALS is diagnosed on the basis of the signs and symptoms,
because no clinical or laboratory tests are specific to
this disease.
• Electromyography and muscle biopsy studies of the
affected muscles indicate reduction in the number of
functioning motor units.
• An MRI scan may show high signal intensity in the
corticospinal tracts; this differentiates ALS from a
multifocal motor neuropathy. Neuropsychological testing
can assist in assessment and diagnosis.

MANAGEMENT
• No cure exists for ALS.
• The main focus of medical and nursing management is on
interventions to maintain or improve function, wellbeing,
and quality of life.
• Because ALS is a progressive disease, the therapeutic
needs are different than those of patients with acute
processes. Insurance carriers tend to limit the number of
therapy sessions, but with early integration into ALS
clinics, alliances are developed for future contact with
therapists familiar with the disease process.
• Riluzole (Rilutek), a glutamate antagonist, has been
shown to prolong survival for persons with ALS for 3 to 6
months.
• The action of riluzole is not clear, but its pharmacologic
properties suggest that it may have a neuroprotective
effect in the early stages of ALS.
• Symptomatic treatment and rehabilitative measures are
used to support the patient and improve the quality of life.
• Baclofen (Lioresal), dantrolene sodium (Dantrium), or
diazepam (Valium) may be useful for patients troubled by
spasticity, which causes pain and interferes with self-care.
• Modafinil (Provigil) may be used for fatigue, and
additional medications may be added to manage the pain, 0
depression, drooling, and constipation that often
accompany the disease.
• Ongoing research for treatment options include neural
transplantation.
• The most common reasons for hospitalization are
dehydration and malnutrition, pneumonia, and respiratory
failure; recognizing these problems at an early stage in the
illness allows for the development of preventive
strategies.
• End-of-life issues include pain, dyspnea, and delirium.
• Mechanical ventilation (using negative-pressure
ventilators) is an option if alveolar hypoventilation
develops.
• Noninvasive positive-pressure ventilation is also an
option. The use of noninvasive positive-pressure
ventilation is particularly helpful at night and postpones

EXCLUSIVELY FOR GROUP 3-BSN3A ONLY | Page 10 of 10

 MEDICAL SURGICAL NURSING
NCM 116
GROUP 3-BSN3A
AMYOTHROPHIC LATERAL SCLEROSIS
• ALS is a disease of unknown cause in which there is
a loss of motor neurons (nerve cells controlling
muscles) in the anterior horns of the spinal cord and
the motor nuclei of the lower brainstem.
• It is often referred to as Lou Gehrig disease, after
the famous baseball player who suffered from the
disease.
• As motor neuron cells die, the muscle fibers that they
supply undergo atrophic changes.
• Neuronal degeneration may occur in both the upper
and lower motor neuron systems.
• The leading theory held by researchers is that
overexcitation of nerve cells by the neurotransmitter
glutamate results in cell injury and neuronal
degeneration.
• ALS most commonly occurs between 40 and 60
years of age and affects all social, racial, and ethnic
backgrounds, with men being affected at slightly
higher rates than women.
• The majority of cases of ALS arise sporadically, but
5% to 10% of cases are familial ALS resulting from
an autosomal dominant trait carried by one parent.
Familial ALS occurs 10 years earlier than the ALS
average, and those afflicted tend to have a shorter
life span (Hardiman, Al- Chalabi, Chio, et al., 2017).
CLINICAL MANIFESTATIONS
• Clinical manifestations depend on the location of the
affected motor neurons, because specific neurons
activate specific muscle fibers.
• The chief symptoms are fatigue, progressive
muscle weakness, cramps, fasciculations
(twitching), and lack of coordination. Loss of
motor neurons in the anterior horns of the spinal
cord results in progressive weakness and
atrophy of the muscles of the arms, trunk, or
legs. Spasticity usually is present, and the deep
tendon stretch reflexes become brisk and overactive.
• Usually, the function of the anal and bladder
sphincters remains intact, because the spinal nerves
that control muscles of the rectum and urinary
bladder are not affected.
• In about 25% of patients, weakness starts in
the muscles supplied by the cranial nerves,
and difficulty in talking, swallowing, and
ultimately breathing occurs (Conde, Martin,
& Winck, 2019; Vacca, 2020).
• When the patient ingests liquids, soft palate
and upper esophageal weakness cause the
liquid to be regurgitated through the nose.
• Weakness of the posterior tongue and palate
impairs the ability to laugh, cough, or even blow
the nose.
• If bulbar muscles are impaired, speaking
and swallowing are progressively difficult,
and aspiration becomes a risk.
• The voice assumes a nasal sound, and
articulation becomes so disrupted that speech
is unintelligible.
• Some emotional lability may be present. It was
traditionally believed that ALS spared cognitive
function, but it is now recognized that some
patients experience cognitive impairment.
• The prognosis generally is based on the area of
CNS involvement and the speed with which the
disease progresses.
• Eventually, respiratory function is compromised
(Conde et al., 2019). Death usually occurs as a
result of infection, respiratory insufficiency, or
aspiration.
ASSESSMENT AND DIAGNOSTIC
• ALS is diagnosed on the basis of the signs and
symptoms, because no clinical or laboratory tests are
specific to this disease.
• Electromyography and muscle biopsy studies of
the affected muscles indicate reduction in the
number of functioning motor units.
• An MRI scan may show high signal intensity in the
corticospinal tracts; this differentiates ALS from a
multifocal motor neuropathy. Neuropsychological
testing can assist in assessment and diagnosis
(Hickey & Strayer, 2020).
MANAGEMENT
• No cure exists for ALS (Vacca, 2020).
• The main focus of medical and nursing management
is on interventions to maintain or improve function,
well-being, and quality of life. Because ALS is a
progressive disease, the therapeutic needs are
different than those of patients with acute processes.
• Insurance carriers tend to limit the number of therapy
sessions, but with early integration into ALS clinics,
alliances are developed for future contact with
therapists familiar with the disease process (Hogden,
Foley, Henderson, et al., 2017).
• Two drugs are used to treat ALS, riluzole and
edaravone (Vacca, 2020). The precise action of
these drugs is not clear, but both are considered
disease modifying treatments for ALS (Vacca, 2020).
• Symptomatic treatment and rehabilitative measures
are used to support the patient and improve the
quality of life. Baclofen, dantrolene sodium, or
diazepam may be useful for patients troubled by
spasticity, which causes pain and interferes with self-
care. Modafinil may be used for fatigue, and
additional medications may be added to manage the
pain, depression, drooling, and constipation that
often accompany the disease. Research suggests
that greater functional impairment is associated with
greater depressive symptoms; consequently,
managing depression helps to maintain a better
quality of life (Soofi, Bello-Haas, Kho, et al., 2018).
Many clinical trials and an ALS registry contribute to
EXCLUSIVELY FOR BSN3A ONLY | Page 1 of 2
 MEDICAL SURGICAL NURSING
NCM 116
GROUP 3-BSN3A
the ongoing study of this devastating disease (Vacca,
2020).
• Most patients with ALS are managed at home and in
the community, with hospitalization for acute
problems.
• The most common reasons for hospitalization
are dehydration and malnutrition, pneumonia,
and respiratory failure; recognizing these
problems at an early stage in the illness allows for
the development of preventive strategies.
• End-of-life issues include pain, dyspnea, and
delirium (Hickey & Strayer, 2020).
• Mechanical ventilation (using negative-pressure
ventilators) is an option if alveolar hypoventilation
develops.
• Noninvasive positive-pressure ventilation is also an
option.
• The use of noninvasive positive-pressure
ventilation is particularly helpful at night and
postpones the decision about whether to undergo a
tracheotomy for long-term mechanical ventilation
(Conde et al., 2019).
• A patient experiencing aspiration and swallowing
difficulties may require enteral feeding. A PEG
tube is inserted before the forced vital capacity drops
below 50% of the predicted value.
• The tube can be safely placed in patients who are
using noninvasive positive-pressure ventilation for
ventilatory support (Hickey & Strayer, 2020).
• Decisions about life support measures are made by
the patient and family and should be based on a
thorough understanding of the disease, the
prognosis, and the implications of initiating such
therapy.
• Patients are encouraged to complete an advance
directive to preserve their autonomy in decision
making.

EXCLUSIVELY FOR BSN3A ONLY | Page 2 of 2