Guillain-Barre Syndrome

Andary, M. T. (2018, May 4). Guillain-Barré syndrome. In M. J. Klein (Ed.), Medscape. Retrieved from https://emedicine.medscape.com/article/315632-overview

---

Guillain-Barré syndrome (GBS) can be described as a collection of clinical syndromes that manifests as an acute inflammatory polyradiculoneuropathy with resultant weakness and diminished reflexes. Although the classic description of GBS is that of a demyelinating neuropathy with ascending weakness, many clinical variants have been well documented in the medical literature.

GBS can cause symptoms that last for a few weeks to several years. Most people recover fully, but some have permanent nerve damage. Some people have died of GBS (CDC, 2017).

Etiology

About two-thirds of people with GBS had diarrhea or a respiratory illness several weeks before developing symptoms. Infection with Campylobacter jejuni, which causes diarrhea, is one of the most common risk factors for GBS. People also can develop GBS after some other infections, such as flu, cytomegalovirus, Epstein Barr virus, and Zika virus. Very rarely, people have developed GBS in the days or weeks after receiving certain vaccines. About 1 in every 1,000 reported Campylobacter illnesses leads to GBS. As many as 40% of GBS cases in the United States are thought to be triggered by Campylobacter infection (CDC, 2017).

Common complaints associated with cranial nerve involvement in GBS include the following:

Most patients complain of paresthesias, numbness, or similar sensory changes. Paresthesias generally begin in the toes and fingertips, progressing upward but generally not extending beyond the wrists or ankles.

Pain associated with GBS is most severe in the shoulder girdle, back, buttocks, and thighs and may occur with even the slightest movements. The pain is often described as aching or throbbing in nature.

Autonomic changes in GBS can include the following:

• Tachycardia
• Bradycardia
• Facial flushing
• Paroxysmal hypertension
• Orthostatic hypotension
• Anhidrosis and/or diaphoresis
• Urinary retention

Typical respiratory complaints in GBS include the following:

• Dyspnea on exertion
• Shortness of breath
• Difficulty swallowing
• Slurred speech

Ventilatory failure with required respiratory support occurs in up to one third of patients at some time during the course of their disease.

See Clinical Presentation for more detail.

Diagnosis

GBS is generally diagnosed on clinical grounds. A basic peripheral neuropathy workup is recommended in cases in which the diagnosis is uncertain. Biochemical screening can also be conducted and would include the following studies:

Needle EMG and nerve conduction studies 

Pulmonary function. Maximal inspiratory pressures and vital capacities are measurements of neuromuscular respiratory function and predict diaphragmatic strength.

Cerebrospinal fluid studies. Most, but not all, patients with GBS have an elevated cerebrospinal fluid (CSF) protein level (>400 mg/L), with normal CSF cell counts. Elevated or rising protein levels on serial lumbar punctures and 10 or fewer mononuclear cells/mm³ strongly support the diagnosis.

See Workup for more detail.

Management

Intensive care unit. Admission to the intensive care unit (ICU) should be considered for all patients with labile dysautonomia, a forced vital capacity of less than 20 mL/kg, or severe bulbar palsy. Any patients exhibiting clinical signs of respiratory compromise to any degree also should be admitted to an ICU.

Immunomodulation. Immunomodulatory treatment in GBS has been used to hasten recovery. Intravenous immunoglobulin (IVIG) and plasma exchange have proved equally effective.

Physical, occupational, and speech therapy. Addressing upright tolerance and endurance may be a significant issue during the early part of physical rehabilitation. Occupational therapy professionals should be involved early in the rehabilitation program. Speech therapy is aimed at promoting speech and safe swallowing skills.

See Treatment and Medication for more detail.