Guillain-Barre Syndrome
GUILLIAN BARRE SYNDROME
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Last Updated : 4/3/2007
Shawn Aylward
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Treatment
The main goal of treatment for GBS is to avoid respiratory failure and potential death. Fortunately in industrialized countries death is relatively rare. However in countries with limited medical care, mortality is reported at around 10% of pediatric cases.(1)
One of the biggest problems with GBS is the relative insidious onset of respiratory failure without clear laboratory markers. Practitioners should not wait for there to be evidence of respiratory compromise on ABG or related testing before considering elective intubation and mechanical ventilation.
Patients should be followed with respiratory assessments at periodic intervals to monitor for changes or decline. These are done via two different tests, typically administered by a respiratory therapist; forced vital capacity (FVC) and negative inspiratory force (NIF). A normal FVC is 60 mL/kg and intubation should be considered once below 20 mL/kg. NIF’s have a wide range of normal values, with normal typically being -50cmH20. The more positive (i.e. closer to zero), the weaker the patient is and -30cm H20 suggests impending respiratory failure.(14)
With the risks of autonomic instability, continuous heart, respiratory monitoring and pulse oximetry are recommended until the patient has plateaued.
Whether to treat is often based on the severity of symptoms at the time of presentation and whether it is believed the patient has reached their nadir. There are two mainstays of treatment to help shorten the progression period of the disease. These are intravenous immune globulin (IVIG) and plasmapheresis. The exact mechanism by which these treatments work is not entirely understood. Plasmapheresis likely filters out the circulating antibodies that are directed against the peripheral nerves. IVIG is less well understood. It may bind anti-idiotypic antibodies, absorb complement, or down regulate B-cell mediated antibody production.
Plasmapheresis has been proven save in children and has been shown to reduce the time to independent ambulation in non-ambulating patients from 60 to 24 days.(15) Complications are rare and include hemorrhage, hypotension, transfusion reactions, septicemia, hypocalcemia, arrhythmia, cardiac arrest, transmitted infections and local tissue injury.
IVIG is a blood product and caries associated risks. Additional side effects include ‘flu-like’ symptoms (headache, fever, myalgias) and aseptic meningitis. It is given at a dose of 2 g/kg, however there is a wide variety in terms of the course. Treatment can be given over 1, 2 or 5 days with studies showing each is well tolerated in children. A study examined a single dose administration in 9 patients, none of which required mechanical ventilation though 2 patients did have cranial nerve involvement. Mean duration of symptoms prior to IVIG was 5.7 days. The mean time to improvement of one grade on the functional GBS scale (table 2) was 3.5 days and mean period to regain ambulation was 11.2 days.(16, 17) All patients regained full mobility without relapse except for one who had relapse at 5 months and subsequent full recovery after a second round of IVIG.(17) Shahar et al. studied 26 children who received infusions over 2 days. Twenty had evidence of cranial nerve involvement with oropharyngeal dysfunction, 22 also experienced sensory symptoms at the time of presentation. All 26 deteriorated over a mean of 6 days to the point they were unable to ambulate. Nineteen developed respiratory involvement, 2 to the point they required mechanical ventilation. Following the infusions, 25 noted improvement of 1-2 grades on the functional GBS scale in less than 14 days. Twenty walked independently by 1 week, and one ventilated patient was extubated after 3 days. Eighteen had full recovery by 2 weeks with the rest by 4 months, and none had evidence of relapse. The single patient with a protracted course did have initial improvement after the infusions but subsequently deteriorated over a period of 4 days. He developed complete paralysis requiring mechanical ventilation for 4 weeks and received a second dose of IVIG 2 weeks after worsening. Improvement was noted after 6 weeks, walking independently after 2 months, and full recovery by 4 months.(18)
Yata et al. used a 5 day course of IVIG with treatment mean of 10 days from onset of the disease. Mechanical ventilation was not required for any patient at the time of enrolment. Median time to improve at least one grade on the functional GBS scale was 10 days, time required to improve two or more grades was 27 days. They did note worsening during or after treatment in 3 patients. One patient had worsening respiratory status after the first administration requiring mechanical ventilation. One patient experienced relapse 41 days after initial treatment and one showed no improvement 20 days after completing the infusions. Both patients received alternative treatments. All three did eventually show improvement.(19)

Table 2. Motor Grading Scale for Acute GBS
Score Clinical Criteria
0 Healthy state
1 Minor signs/symptoms
2 Able to walk 5m without walker or support
3 Able to walk 5m with walker or support
4 Bed or chair bound, unable to walk 5m with walker or support
5 Requires assisted ventilation
6 Dead


Outcomes
It is reported that children are more likely to have a full recovery compared to adults. Korinthenberg and Monting found that following the progression phase, 30-40% of children remain able to walk with or without assistance. This is in contrast to the adult literature which reports only 19% of adults remain able to walk. Mechanical ventilation is needed in 15-20% of cases with 35-45% having some degree of impairment in cranial nerve function; these figures are comparable with that seen in adults.(20)

Roodbol et al. looked at the longterm outcomes in pediatric cases. All patients in their study were able to walk independently within 1 year of disease onset, regardless of disease severity. Two-thirds of patients reported residual issues, mainly severe fatigue and painful/paresthesias of the hands or feet. Neurological exam found signs of residual peripheral nerve damage including limb weakness, areflexia and sensory deficits. They found 14% in their cohort had significant residual disability. A large percentage of patients reported school or work problems. Several were held back a grade or dropped out of school following their GBS diagnosis despite good motor recovery. They found that older age at diagnosis resulted in higher negative effects on education.(21)

References

Contributor Information and Disclosures

Shawn Aylward
MD,
Assistant professor, Nationwide Children’s Hospital,
The Ohio State University. Columbus, OH. USA


First Created : 3/6/2001
Last Updated : 4/3/2007

References

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