Guillain-Barre Syndrome
GUILLIAN BARRE SYNDROME
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Last Updated : 4/3/2007
Shawn Aylward
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Presentation
Incidence is estimated at 0.5-1.5 per 100,000 children <16 years.(1) It demonstrates a slight male predominance and a bimodal distribution with peaks in young adults and the very old.(2)
It is typically considered a monophasic illness, though about 5% of patients have recurrence. Outbreaks have been reported following immunization campaigns involving the oral polio vaccine.(3, 4) Another outbreak was seen in 1976 associated with the H1N1 influenza vaccine; fortunately this was not seen with the most recent outbreak and vaccine campaign in 2009.(5, 6) Up to 2/3 of patients report an infection 3-6 weeks prior to symptom onset, often a viral upper respiratory infection. Given the temporal relation to onset of symptoms, work up for infections causes is often negative. Causative infections that have been substantiated in the literature include Campylobacter jejuni, Cytomegalovirus, Epstein-Barr virus, Mycoplasma pneumonia.(7) C. jejuni has been associated with cases in endemic areas such as China.
Patients often complain of distal weakness that ascends over time. In younger children, balance difficulties sometimes are the initial symptoms reported by parents, leading to the discovery of muscle weakness. As the weakness progresses, they may find the inability to walk, become easily fatigued or complain of difficulties with breathing or swallowing. In children, pain is a common symptom seen in up to 79% at the time of presentation.(8) Reports can vary from leg/back pain, paresthesias, dysesthesias, meningismus, myalgia, visceral discomfort, joint and radicular pain. Fortunately its presence has a poor correlation with presenting severity and eventual recovery.
Despite the wide clinical presentation, there is a stepwise fashion to the clinical course. It begins with onset and progression of symptoms until reaching their nadir. This is typically fairly rapid, with 50-75% reaching nadir by 2 weeks, and 90-98% by 4 weeks. Repeat clinical exams note loss of deep tendon reflexes that begin distally before ascending as the clinical course progresses. Some patients develop hypo-reflexia of the upper extremities without progressing to true areflexia. The plateau phase lasts days to 4 weeks followed by the recovery phase. The duration of recovery varies largely dependent on the severity of neuronal injury.(1)
Autonomic dysfunction is reported in up to 25% of cases.(1) Symptoms are usually intermittent and manifest as postural hypotension, variability in blood pressure, supraventricular tachycardia, and bradycardia. Gastrointestinal and urinary complications in terms of constipation, pseduo-obstruction, swallowing difficulties, and urinary retention can be seen. Rarely the autonomic instability can result in cardiac arrest.(9)

Variants
Miller Fisher Syndrome
This is often thought of as a proximal to distal progression. Symptoms include ataxia, ophthalmoparesis and areflexia.(10) In children it is estimated to encompass 2-4% of cases. Antibodies to the GQ1b ganglioside have been found in some cases. This ganglioside is found in cranial and other peripheral nerves which explains its limited region of involvement.

Acute Motor and Sensory Axonal Neuropathy (AMSAN)
This variant is often thought of as a continuum with AIDP. Presentation is clinically identical for the two. Unfortunately those with a severe amount of axonal degeneration often have incomplete recovery. Diagnostic electromyographic feature of this variant is absence or decrement of motor and sensory action potential amplitudes with only minor slowing of nerve conduction velocities.

Acute Motor Axonal Neuropathy (AMAN)
The first clear reports of this condition came from outbreaks in rural northern China. The presentation is similar to AIDP and AMSAN but has involvement of the motor axons and sparing of the sensory axons. EMG hallmarks include normal sensory nerve conduction velocities with reduced compound motor action potential amplitudes, and relatively normal motor conduction velocities. Needle EMG shows diffuse denervation injury. There is a stronger association with C. jejuni infections than with the other forms, especially in cases originating from northern China.

Polyneuritis Cranialis
This variant is denoted by acute onset of multiple cranial nerve palsies, typically bilateral VII with sparing of II. Clinically patients will have bilateral facial weakness, dysphonia and dysphagia. Studies have shown an association with CMV infection. Postcontrast MRI shows enhancement of multiple cranial nerves; the degree of involvement on imaging is more extensive than is seen on clinical exam.

GBS with Central nervous System Manifestations.
CNS manifestations range from encephalopathy, transverse myelitis, optic neuritis, brainstem/ cerebellar syndromes or acute disseminated encephalomyelitis (ADEM). Pathologic findings are largely due to injury of the spinal nerve roots and cranial nerves. Evidence of inflammation in the medulla, pons and spinal cord can be seen.
In North America and Europe, AIPD accounts for approximately 90% of cases. In China, Japan, Bangladesh, and Mexico, AMAN accounts for 30% to 65% of cases and AIDP is reported at 22% to 46%.(11)

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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