Guillain-Barré Syndrome
Brighton Collaboration Case Definition (Level 1)
  • Acute onset of bilateral and relatively symmetric flaccid weakness/paralysis of the limbs with or without involvement of respiratory or cranial nerve-innervated muscles.
  • Decreased or absent deep tendon reflexes at least in affected limbs.
  • Monophasic illness pattern, with weakness nadir reached between 12 hours and 28 days, followed by clinical plateau and subsequent improvement, or death
  • Cerebrospinal fluid (CSF) with a total white cell count <50 cells/mm3 (with or without CSF protein elevation above laboratory normal value)
  • Electrophysiologic (NCS/EMG) findings consistent with GBS
Guillain-Barré Syndrome Discussion

Guillain-Barré syndrome (GBS) is an acute polyneuropathy often triggered by inflammatory and probably autoimmune mechanisms. The illness is typically characterized by the subacute onset of progressive, symmetrical distal paralysis and lower limb weakness. Weakness may progress to upper limbs and there is often pain in the long lower limb and dorsal region muscles. There are decreased or absent deep tendon reflexes in affected limbs. Sensory abnormalities, involvement of cranial nerves, and paralysis of respiratory muscles also can occur. GBS may as well cause dysphagia and autonomic nervous system dysfunction resulting in tachycardia, and arrhythmias. When severe, the patient is almost totally paralyzed, and GBS can interfere with breathing and, at times, with blood pressure or heart rate. In these cases the disorder is life threatening and is considered a medical emergency. Most patients, however, recover from even the most severe forms of Guillain-Barré syndrome, although some continue to have a certain degree of weakness. Both sexes are equally prone to Guillain-Barré syndrome and its variants. It can strike all ages, but with peaks in young adulthood (15-35 yrs) and in elderly persons (50-75 yrs). While the syndrome is rare, afflicting between 1and 3 persons in 100,000, GBS is the most common cause of acute flaccid paralysis in the United States. In about 2/3 of cases, Guillain-Barré is preceded by a few days or weeks (up to two months) by symptoms of a respiratory or gastrointestinal viral infection. Infectious agents related to GBS include cytomegalovirus, Epstein-Barr virus, Campylobacter jejuni, Mycoplasma pneumoniae and Haemophilus influenzae. An increased risk of GBS may be also related to vaccination, but with presently used vaccines this increase remains below one case of GBS per one hundred thousand doses for most populations. Despite its association with the 1976 Swine Influenza vaccine, there is evidence that, in general, influenza vaccination decreases the risk of acquiring Guillain-Barré syndrome.

After the first clinical manifestations of the disease, the symptoms can progress over the course of hours, days, or weeks. Untreated, the illness has a mean time to the nadir of clinical function of 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. Most patients (up to 85%) with GBS achieve a full and functional recovery within 6-12 months. Recovery is maximal by 18 months after onset. Patients may have persistent weakness, areflexia, imbalance, or sensory loss. The reported incidence of some form of permanent neurologic sequelae ranges between 10% and 40%. Approximately 7-15% of patients have permanent bilateral foot-drop, intrinsic hand muscle wasting, sensory ataxia, and dysesthesia. Recent studies report that patients with Guillain-Barré syndrome may exhibit long-term differences in pain intensity, fatigability, and functional impairment compared with healthy controls.

Despite intensive care in tertiary care centers with a team of medical professionals who are familiar with GBS management, there may be up to a 20% incidence of prolonged morbidity and an approximately 8% mortality. Most cases of mortality are due to adult respiratory distress syndrome, sepsis, pneumonia, pulmonary emboli, cardiac arrest, or severe autonomic instability.

Guillain-Barré Syndrome Treatment

Only intravenous immune serum globulin (IVIG) and plasma exchange (PE) therapy have proven effective for Guillain-Barré syndrome. Both therapies have been shown to shorten recovery time by as much as 50%. IVIG is easier to administer and has fewer complications than plasma exchange. The cost and efficacy of the two treatments are comparable.

Randomized trials in severe disease show that IVIG started within 4 weeks from onset hastens recovery as much as plasma exchange. Combining plasma exchange and IVIG neither improved outcomes nor shortened the duration of illness. Additionally, IVIG is the preferential treatment in hemodynamically unstable patients and in those unable to ambulate independently.

Corticosteroids are ineffective as monotherapy. Limited evidence shows that oral corticosteroids significantly slow recovery from GBS. Substantial evidence shows that intravenous methylprednisolone alone does not produce significant benefit or harm. In combination with IVIG, intravenous methylprednisolone may hasten recovery but does not significantly affect the long-term outcome. The dose of IVIG is 2gm/kg infused over 3 days.

To reduce the risk of potential anaphylaxis, it is recommended to check serum IgA level before infusing IVIG. Infusions may increase risk of thromboembolic events, migraine attacks, aseptic meningitis (10%), urticaria, pruritus, or petechiae (2-30 days postinfusion). There is increased risk of renal tubular necrosis in elderly patients and in patients with diabetes, volume depletion, and preexisting kidney disease.

Guillain-Barré Syndrome Case Example

An 18-year-old white male with no chronic medical problems, 7 days post vaccination, noted numbness of his toes bilaterally. One week later, both feet felt numb. Three days later, his legs felt numb. He went to the Military Treatment Facility for evaluation and was told his extremity numbness was due to his boots. Three days later, he developed tingling of both his legs that was associated with painful cramps in the calves and tingling of his fingertips. The pain in the calves was at a level of 7/10 and worsened with walking and improved with rest. He could not walk up stairs. He also noticed difficulty getting on and off his bunk due to weakness in the arms.

He again presented to sick call for evaluation for his numbness of the feet and upper extremity weakness and tingling of the finger tips. He denied headache, nausea /vomiting, abdominal pain or generalized weakness. He denied fever, chills, and insect bites, and had no history of STDs. On physical exam his sensory exam was abnormal, motor strength was reduced (3/5 w/ SLR, 3/5 heel to shin, unable to toe raise), gait and stance were abnormal, and the deep tendon reflexes were absent or diminished. His EMG/NCV showed "electrophysiologic evidence of generalized sensorimotor polyradiculopathy with mixed axonal and acquired demyelinating features."

Cerebral spinal fluid was clear. Protein was elevated at 179.0 (12-60). There were 3 (0-8) WBCs and 2 (0) RBCs noted.