GUILLAIN-BARRE SYNDROME

DESCRIPTION

Guillain-Barre syndrome (GBS), once thought to be a single entity characterized by inflammatory

peripheral neuropathy, is a combination of clinical features with various forms of presentation and
multiple pathologic processes. A full discussion of this complex condition is beyond the scope of this
chapter. Most cases of GBS do not require admission to critical care unit. However, the prototype of
GBS, known as acute inflammatory demyelinating polyradiculoneuropathy (AIDP), involves a rapidly
progressive, ascending peripheral nerve dysfunction leading to paralysis that may produce respiratory
failure. Because of the need for ventilator support. AIDP is one of the few peripheral neurologic disease
that necessitates care in a critical care environment.

The annual incidence of GBS is 1.8 cases per 100,000 persons. It occurs more often in males and
is the most commonly acquired demyelinating neuropathy. Occasionally, cluster of cases are reported,
such as occurred following the 1977 swine flu vaccination.

ETIOLOGY

The precise cause of GBS remains unknown, but the syndrome involves an immune-mediated
response involving cell-mediated immunity and development of IgG antibodies. Most patients report a
viral infection 1 to 3 weeks before the onset of clinical manifestations, usually involving the upper
respiratory tract.

Numerous antecedent causes or triggering events, have been associated with GBS. They include
viral infections (e.g., influenza; cytomegalovirus; hepatitis A, B, or C; Epstein-Barr virus; human
immunodeficiency virus), bacterial infections (e.g., gastrointestinal Campylobacter jejuni, Mycoplasma
pneumoniae), vaccines (e.g., rabies, tetanus, and influenza), lymphoma, surgery, and trauma.

PATHOPHYSIOLOGY

GBS affects the motor and sensory pathways of the peripheral nervous system, as well as automatic
nervous system functions of the cranial nerves. The major finding in the AIDP-type GBS is segmental
demyelination process of the peripheral nerves. GBS is thought to be autoimmune response to
antibodies formed in response to a recent physiologic event. T cells migrate to the peripheral nerves,
resulting in edema and inflammation. Macrophages then invade the area and break down the myelin.
Inflammation around this demyelinated area causes further dysfunction. Some axonal damage occurs.

The myelin sheath of the peripheral nerves is generated by Schwann cells and acts as an
insulator for the peripheral nerve. Myelin promotes rapid conduction of nerve impulses by allowing the
impulses o jump along the nerve by means of nodes of Ranvier. Disruption of the myelin fiber shows and
may eventually stop the conduction of impulses along the peripheral nerves. In GBS, the more thickly
myelinated fibers of motor pathways and the cranial nerves are more severely affected than are the
thinly myelinated sensory fibers of cutaneous pain, touch and temperature.

After the temporary inflammatory reaction stops, myelin producing cells begin the process of
reinsulating the demyelinated portions of the peripheral nervous system. When remyelination occurs,
normal neurologic function should return. In some instances, the axon may be damaged during the
inflammatory process. The degree of axonal damage is responsible for the degree of neurologic
dysfunction that persists after recovery.

ASSESSMENT AND DIAGNOSIS

Symptoms of GBS include motor weakness, paresthesias and other sensory changes, cranial nerve
dysfunction (especially oculomotor, facial, glossopharyngeal, vagal, spinal, accessory and hypoglossal),
and some automatic dysfuncion. The course of GBS begins with an abrupt onset of lower extremity
weakness that progresses to flaccidity and ascends over a period of hours to days. Motor loss usually is
symmetric, bilateral and ascending. In the most severe cases, complete flaccidity of all peripheral
nerves, including spinal and cranial nerves, occurs.

The patient is admitted to the hospital when lower extremity weakness prevents mobility.
Admission to the critical care unit is necessary when progression of the weakness threatens respiratory
muscles. As the patient’s weakness progresses, close observation is essential. Frequent assessment of
the respiratory system, including ventilator parameters such as inspiratory force and tidal volume, is
necessary. The most common cause of death o patients with GBS us respiratory arrest. As the disease
progresses and respiratory effort weakens, intubation and mechanical ventilation are necessary.
Frequent assessment of neurologic deterioration is continued until the patient reaches the peak of the
disease, and a plateau occurs.

The diagnosis of GBD is based on clinical findings plus CSF analysis and nerve conduction studies.
The diagnostic finding is elevated CSF protein with normal cell count. The increased protein count
usually occurs after the first week but does not occur approximately 10% of all cases. Nerve conduction
studies that test the velocity at which nerve impulses are conducted show significant reduction, as the
demyelinating process of the disease suggests.

MEDICAL MANAGEMENT

With no curative treatment available, the medical management of GBS is limited. The disease must run
its course, which is characterized by ascending paralysis that advances over 1 to 3 weeks and then
remain at a plateau for 2 to 4 weeks. The plateau stage is followed by descending paralysis and return to
normal or near-normal function. The main focus of medical management is the support of bodily
function and the prevention of complication.

Plasmapheresis and intravenous immune globulin (IVIG) are used to treat GBS. They have been
shown to be equally effective. Plasmapheresis involves the removal of venous blood through a catheter,
separation of plasma or another replacement solution. Although the number of exchanges may vary,
four to six exchanges usually are performed over a 5- to 8-day period.

NURSING MANAGEMENT

The nursing management of the patient with GBS incorporates a variety of nursing diagnoses and
interventions. The goal of nursing management is to support all normal body functions until the patient
can do so on his or her own. Although the condition is reversible, the patient with GBS requires
extensive long-term care, because recovery can be a long process. Nursing priorities are directed toward
maintaining surveillance for complications, initiating rehabilitation, facilitating nutritional support, and
providing comfort and emotional support.
Maintaining Surveillance for Complications. Continuous assessment of the progressive paralysis
associated with GBS is essential to timely intervention and the prevention of respiratory arrest and
further neurologic insult. After the patient is intubated and placed on mechanical ventilation, close
observation for pulmonary complications, such as atelectasis, pneumonia and pneumothorax is
necessary. Autonomic dysfunction (dysautonomia) in the GBS patient can produce variations in the
heart rate and blood pressure that can reach extreme values. Hypertension and tachycardia may require
beta-blocker therapy. All GBS patients must be observed for this phenomenon.

Initiating Rehabilitation. In patients with GBS, immobility may last for months. The usual course of GBS
involves an average of 10 days of symptom progression and 10 days of maximal level of dysfunction,
followed by 2 to 48 weeks of recovery. Although GBS usually is completely reversible, the patient will
require physical and occupational rehabilitation because of the problems of long-term immobility.
Rehabilitation because of the problems of long-term immobility. Rehabilitation starts in the critical care
area, with multidisciplinary team and designing and implementing an individualized plan for maximizing
the patient’s potential for rehabilitation.

Facilitating Nutritional Support. Nutritional support is implemented early in the course of the disease.
Because GBS recovery is a long process, adequate nutritional support will be a problem for an extended
period. Nutritional support usually is accomplished through the use of enteral feeding.

Providing Comfort and Emotional Support. Pain control is another important component in the care of
the patient with GBS. Although patients may have minimal to no motor function, most sensory functions
remain, causing patients considerable muscle aching and pain. Because of the length of this illness, a
safe, effective, long term solution to pain management must be identified. These patients also require
extensive psychological support. Although the illness is almost 100% reversible, lack of control over the
situation, constant pain or discomfort, and the long-term nature of the disorder crate coping difficulties
of the patient.
pathophysiology

is an acute inflammatory disorder that affects the axons and or myelin of the PNS, causing
impaired mobility and sensory perception as a result of altered immunity. it is an uncommon disorder,
affecting males slightly more than females and peaking after age 55 years.

GBS may be referred to by a variety of other names, such as acute idiopathic polyneuritis, acute
inflammatory demyelinating polyneuropathy, acute motor axonal neuropathy, and acute motor and
sensory axonal neuropathy (arcia-Londono and Lewis,2012). in some forms of GBS, primarily the axons
are affected. In other forms, demyelination(destruction of myelin sheath)of the peripheral nerves
occurs. in demyelinating GBS, symptoms typically begins in the legs and spread to the arms and upper
body. this is referred as a ascending paralysis. Paralysis can increase an intensity until the muscles
cannot be used at all and the patient is almost totally immobile. As a result, some patients require
mechanical ventilation because of a weak or paralyzed diaphram and accesory muscles for respiration.
healing occurs in reverse, the neurons affected last are first to recover.

GBS is likely the result of altered immunity. antibodies attack the myelin sheath that sourrounds
the axons of the peripheral nerves . on microscopic examination, groups of lymphocytes are seen at the
points of myelin breakdown. in some instances, secondary damage to the cell body, the neurilemma or
the axon occurs. Neurilemma and axonal injury can delay recovery or result in permanent neurologic
defects. segmental demyelination (the destruction of myelin between the nodes of Ranvier)is the major
pathologic finding in most variants of GBS.This destruction slows the transmission of impulses from
node to node. Damaged motor neurons result in impared mobility. Damaged sensory nerves sends
fewer messages to the brain, affecting the patient's sensory perception.

Three stages make up the acute course of GBS:

- the acute or initial period (1-4 weeks), which begins with the onset of the first symptoms and ends
when no further deterioration occurs.

- the palteu period (several days to 2 weeks)

-the recovery phase (gradualy over 4-6 months, maybe up to 2 years), which is thought to coincide with
remyelination and axonal regeneration (some patients do not completely recover and have permanent
neurologic deficits, referred to as chronic GBS).

ETIOLOGY

GBS is often associated with bacterial infection, especially infection with campylobacter jejuni. Influenza,
Epstein-Barr, and cytomegaly virus (CMG) viral infectionhave also been associated with GBS. In countries
where the recent zika virus has emerged, the number of GBS cases has increased, suggesting that the
zika virus infection can also trigger GBS (Sampathkumar & Sanchez 2016). There are also reports of some
vaccines increasing the risk of GBS slightly, but epidemiologc evidence is weak (Center for Disease
Control and Prevention, 2016). there are also reports of some vaccines increasing the risk of GBS slightly,
but epidemiologic evidence is weak (Center for Disease Control and Prevention, 2017). it is believed that
the precipitatimg infection or event sensitizes thet-cell to the patients myelin,resulting in production of
a demyelinating autoantibody.
INCIDENCE/PREVALENCE

GBS is a rare disease affecting men mre than women, but it is increasing in numbers as multiple viruses
affect the global poulation. in a classic, and not since replicated, systemic review of the incidence of GBS
worldwide, Sejvar et al. (2011) found that only one or two cases occur in every 100,000 people. The
incidence increases 20% for each decade of life.

INTERPERSONAL COLLABORATIVE CARE

ASSESSMENT: NOTICING

PHYSICAL ASSESSMENT/ SIGNS AND SYMPTOMS

obtain a complete health histor. ask the patient to describe GBS symptoms in chronologic order,
if possible. inquire the presence of altered comfort, numbness, and paresthesias (unpleasant sensations
such as burning, stinging, and prickly feeling)

although features vary, most people report a sudden onset of muscle weakness that affects
mobility. The common symptoms of GBS are loss of reflexes in the arms and legs, low blood pressure
contol, muscle weakness or paralysis, numbness, uncoordinated movement leading to clumpsiness and
fall, blurred or double vision (diplopia) and palpitations (McCance et al.., 2014). typically, the disease
does not affect level of consciousness, cognition, or pupillary constriction or dilation.

with any of the variants, cranial nerve involvement most often affects the facial nerve (CN VII).
assess the patients ability to smile , frown, whistle or drink from a straw. Assess the patient for
dysphagia which involves CNs V, VII,X,XI and XII. the patients inability to cough, gag, or swallow results
from the involvement of CNs IX and X. Monitor the patient closely for varying blood pressure
(hypertensive and hypotensive episodes or orthostatic hypotension), bradycardia, heart block and
possibly asystole. This symptoms are part of autonomic dysfunction which is linked to vagus nerve (CN
X) deficit. Assess CN XI (Accesory) by asking the patient to shrug the shoulders . Hypoglossal nerve (CN
XII) deficit is manifested by an inability to stick the tongue out straight.

in addition to determining the usual roles and responsibilities, occupation, motivation, and
available support systems, assess the patient's ability to cope with this devastating illness and
accompanying fear and anxiety. in general, GBS is self limiting and the paralysis is temporary. It is not
unusual of the patient to have depression throughout the recovery period or feel significant
powerlessness

Diagnostic Assessment

Although no single clinical or laboratory finding confirms the diagnosis of GBS, the primary
health care provider may perform a lumbar puncture (LP) to evaluate cerebrospinal fluid (CSF). An
increase in CSF protein level can occur from inflammatory plasma proteins, myelin breakdown, and
damage to nerve roots. However, high protein levels may not occur until after 1 to 2 weeks of illness,
reaching a peak in 4 to 6 weeks. The CSF lymphocyte count is normal.

Peripheral blood tests may show a moderate leukocytosis early in the illness. The number of
leukocytes rapidly returns to normal if there are no complications or concurrent illness.
 Electrophysiologic studies (EP's) demonstrate demyelinating neuropathy. The degree of
abnormality found on testing does not always correlate with clinical severity. Within 3 weeks of
symptoms, nerve conduction velocities are depressed. In some cases, denervated potentials
(fibrillations) develop later in the illness. Electromyographic (EMG) findings, which reflect peripheral
nerve function, are normal early in the illness. Electrophysiologic changes appear only after denervation
of muscle has been present for 4 weeks or longer. Nerve conduction velocity (NV) testing is performed
with the EMG. Nerve damage or disease may still exist despite normal NCV results. An MRI or CT scan
may be requested to rule out other causes of motor weakness.

Respiratory function manifested by poor gas exchange is often comprised in patients with GBS.
Therefore vital capacity or tidal volume may be decreased, and respiratory rate increased. Arterial blood
gas (ABG) values may be abnormal with a decreased partial pressure of arterial oxygen (PaO2),
increased partial pressure of arterial carbon dioxide or decreased pH.

Analysis Interpreting

The priority collaborative problems for patients with GBS specifically include:

1. Potential for respiratory and/or cardiovascular distress or failure due to thoracic muscle weakness
and hypotension.

2. Decreased mobility due to skeletal muscle weakness.

Planning and Implementation: Responding

Preventing Respiratory and/or Cardiovascular Distress or Failure

Planning: Expected Outcomes. The desired outcome for the patient with GBS is that the patient
will not experience cardiopulmonary failure as a result of managing the airway, promoting gas exchange
and maintaining normal-range blood pressure.

Interventions: The primary health care provider follows the most recent best practice guidelines
from the American Academy of Neurology for the treatment of GBS. The patient may receive either
plasma exchange (also known as plasmapheresis) or IV immunoglobulin (IVIG). Corticosteroids are not
used unless medically necessary to treat other associated diseases.

Plasmapheresis removes the circulating antibodies thought to be responsible for the disease. In
this procedure, plasma is selectively separated from the whole blood. The blood cells are returned to
the patient without the plasma. Plasma usually replaces itself, or the patient is transfused with a
colloidal substitute such as albumin. Fresh frozen plasma is generally not used because of the associated
risk for infection and allergic pulmonary edema. Plasmapheresis should be done within several days
after the onset of the illness, although some patients benefit up to 30 days after the onset of symptoms.
The patient usually receives three to four treatments, 1 to 2 days apart. Some patients may require a
second round of treatment if they deteriorate after the first plasmapheresis.

Nursing interventions for the patient undergoing plasmapheresis include providing information
and reassurance, weighing the patient before and after the procedure, caring for the shunt or venous
access site, and preventing the complication.
 IV Immunoglobulin (IVIG) has been shown to be as effective as plasmapheresis and is
immediately available in most settigs. Side effects of immunoglobulin therapy range from minor
discomforts (e.g., chills,mild fever, myalgia and headache) to major complications (e.g., anaphylaxis,
aseptic meningitis, retinal necrosis, acute renal failure). Infused IVIG slowly when it is started. Observe
for and document side and adverse effects and report their occurence to the health care provider. The
rate of administration can be increased based on the patient's tolerance and agency protocol.