Key Elements of Guillain-Barré Syndrome

Guillain-Barré Syndrome (GBS) is an autoimmune attack on the peripheral nervous system which results in neuropathy. It is typically a self-limiting condition that comes on rapidly and results in muscle weakness with some mild sensory loss in the distal extremities.

GBS typically follows a recent GI tract or respiratory system infection and has been associated with surgery, immunization and viral infections including Epstein-Barr, influenza and the Zika virus. In cases where the axon is targeted, it is thought to be a result of molecular mimicry of pathogen-borne antigens.

There are four subtypes of GBS:

• Acute inflammatory demyelinating polyneuropathy (AIDP) – This subtype accounts for most cases of GBS. It involves an ascending paralysis that typically starts in the lower extremities.
• Acute motor axonal neuropathy (AMAN) – This subtype involves progressive weakness of acute onset with no sensory impairment.
• Acute motor and sensory axonal neuropathy (AMSAN) – This subtype involves ascending paralysis and sensory impairment.
• Miller Fisher syndrome (MFS) – This subtype accounts for less than 5% of cases and symptoms can be similar to the common AIDP subtype. However, the typical presentation involves disruptions in cranial nerves 3, 5 and 6 resulting in ophthalmologic symptoms, areflexia and ataxia. A common trigger for MFS is infection, specifically campylobacter jejuni enteritis. 

Now that you have a basic understanding of what GBS is and what causes it, let’s break down the nursing implications using the Straight A Nursing LATTE method. Since most patients with Guillain-Barré have the AIDP subtype, that’s what we’ll focus on in this lesson.

L: How does the patient LOOK?

The clinical manifestations of GBS can vary from person to person and involves ascending flaccid paralysis that starts in the lower extremities and works its way upward to result in full quadriplegia and respiratory insufficiency.

The official diagnostic criteria include:

• Progressive weakness of more than two limbs
• Loss of neurological reflexes (areflexia)
• Duration of four weeks or less (remember, this is an acute condition)

Prior to experiencing the ascending paralysis, many patients report prodromal symptoms such as fever, cough, sore throat, pain, rhinorrhea and diarrhea. During the acute phase of the illness, signs and symptoms include: 

• Bilateral ascending paralysis
• Decreased sensation, numbness, tingling
• Diminished or absent neurological reflexes (areflexia)
• Facial weakness or paralysis that can be unilateral or bilateral. As a result, the individual could have dysphagia and dysarthria.
• Respiratory compromise due to paralysis of the muscles of respiration. A patient with respiratory compromise will be on a mechanical ventilator and this occurs in about 30% of patients. Note that the patient may also require intubation if the neck muscles are severely weakened, which puts the patient at risk for airway compromise. 
• Weakness and fatigue
• Pain that is often worse with movement. The pain with GBS can be related to joints, muscles or dysesthesia, which is an abnormal pain that can present as burning, aching or tingling. Interestingly, pain is often the first symptom seen in pediatric patients.
• Autonomic dysfunction including orthostatic hypotension, urinary retention, cardiac arrhythmias and extremes in blood pressure

A: How do you ASSESS a patient with Guillain-Barré Syndrome?

Key assessments for a patient with GBS are centered on monitoring for rapid deterioration namely due to respiratory compromise.

Respiratory status – When assessing respiratory status, monitor the patient’s respiratory effort, depth of respirations, rate, oxygen saturation level, shortness of breath, work of breathing, and lung sounds. 

Neurological status – Of course, you’re also carefully monitoring neurological status with frequent neuro assessments. In the ICU, these could be as frequent as every hour and with any change in condition. As part of this assessment, make note of the patient’s level of paralysis, their sensory and motor function (including a full cranial nerve assessment), and their level of consciousness. Make note of any dysfunction that could put the patient at risk for aspiration including dysphagia and dysarthria.

Monitor cardiovascular status – Due to autonomic dysfunction, the patient will also be at risk for cardiovascular abnormalities including hypotension and hypertension, orthostatic hypotension, tachycardia, significant bradycardia and bradyarrhythmias, and even asystole. This patient should be on continuous telemetry monitoring.

Pain assessment – Just because the condition involves paralysis and sensory impairment does not mean the patient is not at risk for pain. Also, be very aware of the effect opioids have on respiratory drive, especially when these muscles are weakened.

Blood glucose monitoring – During the paralysis phase of GBS, some patients can develop hyperglycemia or hyperinsulinemia. Note that this may be due to treatment with corticosteroids, but can be present even if these medications are not used. Note that corticosteroids are not currently recommended as a treatment modality, but that doesn’t mean you won’t see them used.

Skin assessment – Neuropathy and decreased mobility both put your patient with GBS at high risk for skin breakdown.

Disability scoring – Though this will be up to the physician, it’s helpful to understand how the functional status of patients with GBS is classified. 

The Guillain Barré Disability Score:

• 0: Healthy, no symptoms
• 1: Minor symptoms and capable of manual work or running 
• 2: Able to walk 5-10 meters or more without assistance but unable to run 
• 3: Able to walk 5-10 meters across an open space with assistance 
• 4: Bedridden or chair bound 
• 5: Requiring assisted ventilation for at least part of the day 
• 6: Deceased

T: What TESTS will be utilized to monitor a patient with GBS?

The diagnostic tests for GBS include: 

• Nerve conduction studies – In this test, small electrodes are attached to the skin and minor electrical shocks are delivered to activate the nerves. The test measures how quickly the signals travel along nerve fibers and helps determine if the GBS is demyelinating or axonal type.
• Needle electromyography – This test involves tiny needles inserted into the muscles while electrical recordings are taken. These recordings show how the muscles react when associated nerves are activated.
• Lumbar puncture with CSF analysis – An analysis of the cerebrospinal fluid can show if albuminocytologic dissociation is present. It looks to see if the CSF has more WBC and higher CSF protein than normal without an overall increase in cell count. It is present in about 80% of patients approximately 2 weeks after symptom onset.
• Spinal MRI – This imaging study may be useful to rule out other conditions that could cause the patient’s symptoms
• Blood test for ganglioside antibodies – Though these antibodies are detected in 50% of patients with GBS, the turnaround time for the test is relatively long so it may not be especially beneficial when quick diagnosis is needed.
• Negative inspiratory force (NIF)  – This test is often conducted by the respiratory therapist and helps determine the risk for respiratory compromise. If intubated, it also can be used to help determine if the patient is ready to be weaned from mechanical ventilation. 
• Speech evaluation – To assess your patient’s ability to swallow safely, the MD will order a speech therapy evaluation.

T: What TREATMENTS will be provided for a patient with GBS? 

Immunomodulatory therapy – Two types of immunomodulatory therapy are utilized in the treatment of GBS, and these are intravenous immunoglobulin (IVIG) and plasma exchange. This type of therapy is typically considered in patients who are within four weeks of symptom onset, though it’s important to note that not all patients will benefit from treatment. 

The preferred therapy is generally intravenous immunoglobulin which is better tolerated in most cases and easier to administer than plasma exchange. 

IVIG, which contains many antibodies, is administered at a weight-based dose for a period of five days. Diphenhydramine, acetaminophen and fluids are often given prior to the infusion. During administration, the nurse monitors the patient for adverse reactions which can include hypotension, headache, nausea, rash, acute kidney failure, and transfusion reactions. Note that if the dose is condensed into fewer than five days, the risk for cardiac and renal complications increases.

The process of plasma exchange filters out pathogenic antibodies as well as the mediatory and complement proteins involved in Guillain-Barré syndrome. The process of performing plasma exchange requires specialized training and may be done by a dialysis RN. Patients typically receive four to six treatments over a period of eight to ten days. Complications include transfusion reactions, sepsis and hypotension.

Other treatments include: 

• Physical therapy during the recovery phase
• Insulin to manage blood glucose levels
• Enteral feeding as needed
• Mechanical ventilation as needed (the patient will likely have a tracheostomy if ventilatory support is required for more than a week or two)
• Regular oral care for ventilated patients to reduce the risk of ventilator acquired pneumonia
• A pacemaker may be needed if the patient has significant bradycardia as a result of autonomic dysfunction
• DVT prophylaxis
• Bowel care regimen to avoid constipation

E: How do you EDUCATE the patient and family?

In addition to ensuring your patient understands all medications and key treatments, some key things to teach are: 

• GBS rarely recurs and more than 80% of patients are independent within six months and about 65% have a full recovery. However, about 20% do have significant disability even with treatment.
• Recovery will include extensive PT and OT. 
• The patient may continue to experience fatigue, pain, and paresthesias for several years.

Take this topic on the go by tuning in to episode 281 of the Straight A Nursing podcast. Listen from any podcast platform, or straight from the website here.

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