Guillain-Barre Syndrome Clinical Presentation

Updated: May 04, 2018
  • Author: Michael T Andary, MD, MS; Chief Editor: Milton J Klein, DO, MBA  more...
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Presentation

History

The typical patient with Guillain-Barré syndrome (GBS), which in most cases will manifest as acute inflammatory demyelinating polyradiculoneuropathy (AIDP), presents 2-4 weeks following a relatively benign respiratory or gastrointestinal illness with complaints of finger dysesthesias and proximal muscle weakness of the lower extremities. The weakness may progress over hours to days to involve the arms, truncal muscles, cranial nerves, and muscles of respiration. Variants of GBS may present as pure motor dysfunction or acute dysautonomia.

The mean time to the clinical function nadir is 12 days, with 98% of patients reaching a nadir by 4 weeks. A plateau phase of persistent, unchanging symptoms then ensues, followed days later by gradual symptom improvement. [3] Progression of symptoms beyond that point brings the diagnosis under question. Recovery usually begins 2-4 weeks after the progression ceases. [94] The mean time to clinical recovery is 200 days.

Some patients with atypical presentation, incomplete weakness, and some inconsistencies in their physical examination are frequently diagnosed as having a psychological reaction or hysteria, and the diagnosis is very difficult. They are frequently sent home from the emergency department and then return with persistent or progressive symptoms hours to days later.

Weakness

The classic clinical picture of weakness is ascending and symmetrical in nature. The lower limbs are usually involved before the upper limbs. Proximal muscles may be involved earlier than the more distal ones. Trunk, bulbar, and respiratory muscles can be affected as well.

Patients may be unable to stand or walk despite reasonable strength, especially when ophthalmoparesis or impaired proprioception is present. Respiratory muscle weakness with shortness of breath may be present.

Weakness develops acutely and progresses over days to weeks. Severity may range from mild weakness to complete tetraplegia with ventilatory failure.

Cranial nerve involvement

Cranial nerve involvement is observed in 45-75% of patients with GBS. Cranial nerves III-VII and IX-XII may be affected. Common complaints include the following:

  • Facial droop (may mimic Bell palsy)

  • Diplopias

  • Dysarthria

  • Dysphagia

  • Ophthalmoplegia

  • Pupillary disturbances

Facial and oropharyngeal weakness usually appears after the trunk and limbs are affected. The Miller-Fisher variant of GBS is unique in that this subtype begins with cranial nerve deficits. [95]

Sensory changes

Most patients complain of paresthesias, numbness, or similar sensory changes. Sensory symptoms often precede the weakness. Paresthesias generally begin in the toes and fingertips, progressing upward but generally not extending beyond the wrists or ankles. Loss of vibration, proprioception, touch, and pain distally may be present.

Sensory symptoms are usually mild. In most cases, objective findings of sensory loss tend to be minimal and variable.

Pain

In a prospective, longitudinal study of pain in patients with GBS, 89% of patients reported pain that was attributable to GBS at some time during their illness. On initial presentation, almost 50% of patients described the pain as severe and distressing. The mechanism of pain is uncertain and may be a product of several factors. Pain can result from direct nerve injury or from the paralysis and prolonged immobilization.

Pain 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.

Dysesthetic symptoms are observed in approximately 50% of patients during the course of their illness. Dysesthesias frequently are described as burning, tingling, or shocklike sensations and are often more prevalent in the lower extremities than in the upper extremities. Dysesthesias may persist indefinitely in 5-10% of patients.

Other pain syndromes in GBS include the following:

  • Myalgic complaints, with cramping and local muscle tenderness

  • Visceral pain

  • Pain associated with conditions of immobility (eg, pressure nerve palsies, decubitus ulcers)

The intensity of pain on admission correlates poorly with neurologic disability on admission and with the end outcome.

Autonomic changes

Autonomic nervous system involvement with dysfunction in the sympathetic and parasympathetic systems can be observed in patients with GBS.

Autonomic changes can include the following:

  • Tachycardia

  • Bradycardia

  • Facial flushing

  • Paroxysmal hypertension

  • Orthostatic hypotension

  • Anhidrosis and/or diaphoresis

Urinary retention due to urinary sphincter disturbances may be noted. Constipation due to bowel paresis and gastric dysmotility may be present. Bowel and bladder dysfunction are rarely early or persistent findings.

A study by Anandan et al indicated that in hospitalized patients with GBS, autonomic dysfunction most frequently manifests as diarrhea/constipation (15.5%), hyponatremia (14.9%), syndrome of inappropriate antidiuretic hormone secretion (SIADH, 4.8%), bradycardia (4.7%), and urinary retention (3.9%). The study included 2587 patients with GBS and 10,348 controls. [96]

Dysautonomia is more frequent in patients with severe weakness and respiratory failure. Autonomic changes rarely persist in a patient with GBS.

Respiratory involvement

Upon presentation, 40% of patients have respiratory or oropharyngeal weakness. Typical complaints 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.

Antecedent illness

Up to two thirds of patients with GBS report an antecedent illness or event 1-3 weeks prior to the onset of weakness. Upper respiratory and gastrointestinal illnesses are the most commonly reported conditions. [22, 24] Symptoms generally have resolved by the time the patient presents for the neurologic condition.

Campylobacter jejuni is the major causative organism identified in most studies and is responsible for cases of AIDP and acute motor axonal neuropathy (AMAN). In one major study, previous diarrheal illness had occurred in 60% of patients with axonal GBS (by neurophysiologic testing).

Vaccinations, surgical procedures, and trauma have been reported to trigger the development of GBS. [41] Much of this information is anecdotal, although vaccination with the swine flu vaccine administered in 1976 was shown to increase the risk of contracting GBS to a small, but definable, degree. Rabies vaccine prepared from infected brain tissue also was found to have an association with GBS. Studies of other vaccines, however, have not shown a significant relationship between these drugs and GBS or have been inconclusive. [43, 49]

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Physical Examination

Cardiac arrhythmias, including tachycardias and bradycardias, can be observed as a result of autonomic nervous system involvement. Tachypnea may be a sign of ongoing dyspnea and progressive respiratory failure.

Blood pressure lability is another common feature, with alterations between hypertension and hypotension. Temperature may be elevated or low.

Respiratory examination may be remarkable for poor inspiratory effort or diminished breath sounds. On abdominal examination, paucity or absence of bowel sounds suggests paralytic ileus. Suprapubic tenderness or fullness may be suggestive of urinary retention.

Neurologic examination

Facial weakness (cranial nerve VII) is observed most frequently, followed by symptoms associated with cranial nerves VI, III, XII, V, IX, and X. Involvement of facial, oropharyngeal, and ocular muscles results in facial droop, dysphagia, dysarthria, and findings associated with disorders of the eye.

Ophthalmoparesis may be observed in up to 25% of patients with GBS. Limitation of eye movement most commonly results from a symmetrical palsy associated with cranial nerve VI. Ptosis from cranial nerve III (oculomotor) palsy also is often associated with limited eye movements. Pupillary abnormalities, especially those accompanying ophthalmoparesis, are relatively common as well. Papilledema secondary to elevated intracranial pressure is present in rare cases. Tonic pupils have been reported but only in severe cases.

Lower extremity weakness usually begins first and ascends symmetrically and progressively over the first several days. Upper extremity, trunk, facial, and oropharyngeal weakness is observed to a variable extent. Marked asymmetrical weakness calls the diagnosis of GBS into question.

Patients may be unable to stand or walk despite reasonable strength, especially when ophthalmoparesis or impaired proprioception is present.

Despite frequent complaints of paresthesias, objective sensory changes are minimal. A well-demarcated sensory level should not be observed in patients with GBS; such a finding calls the diagnosis of GBS into question.

Reflexes are absent or reduced early in the disease course. Hyporeflexia or areflexia of involved areas represents a major clinical finding on examination of the patient with GBS. Pathologic reflexes, such as the Babinski sign, are absent. Hypotonia can be observed with significant weakness.

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