Guillain-Barré in Children
Abstract & Commentary
By Michael Rubin, MD
Professor of Clinical Neurology, Weill Cornell Medical College
Dr. Rubin reports no financial relationships relevant to this field of study.
Synopsis: Guillain-Barré syndrome in children presents in a very similar way as in adults, and is treated with either intravenous immunoglobulin or plama exchange, even though there are no large randomized treatment trials of children.
Source: Devos D, et al. Guillain-Barré syndrome during childhood: Particular clinical and electrophysiological features. Muscle Nerve 2013:48;247-251.
With an incidence of up to 2.4 cases per 100,000 in the general population, and up to 1.34 cases per 100,000 in children under 15 years of age, Guillain-Barré syndrome (GBS) is the most common cause of acute flaccid paralysis in previously healthy infants and children. Males are affected more often than females in all age groups, with approximately two-thirds of patients giving a history of antecedent respiratory or gastrointestinal tract infection, most frequently, campylobacter. Does childhood GBS present any specific characteristics that may be identified?
Medical records, including clinical, laboratory, and electrophysiological data, of all 19 children diagnosed with GBS between January 2000 and June 2011, at Nantes University Hospital, Nantes Cedex, France, were reviewed. All subjects fulfilled GBS diagnostic criteria. Electrodiagnostic studies were performed using standard techniques and, for children ≥ 3 years of age, compared to healthy adult values. For those < 3 years of age, normal nerve conduction velocities and motor amplitudes were set at 80%, and distal latencies at 120%, of adult values, to allow for the incomplete myelination of nerves in this age group. Based on electrodiagnostic findings, patients were grouped into either acute inflammatory demyelinating polyradiculoneuropathy (AIDP) or acute motor axonal neuropathy (AMAN). None presented with Miller-Fisher syndrome or acute motor and sensory axonal neuropathy (AMSAN).
Among the 19 children (13 boys and 6 girls), 12 were < 5 years of age, the youngest presenting at 18 months of age. Five children were between 5-10 years of age, and two were older, the oldest presenting at 13 years of age. Most (80%) developed GBS in the winter (n = 7) or spring (n = 8), with 68% (n = 13) reporting an antecedent infection within the prior 4 weeks, respiratory in 8, and gastrointestinal in 2. None reported receiving a vaccination in the prior 4 weeks, and all but one presented as AIDP, while one presented as AMAN. Limb weakness was reported in all patients, and 74% complained of limb pain. Walking ability was lost in 10, and another eight had ataxia. Facial weakness was present in only a single child, three had some cranial nerve involvement, and eight were found to have autonomic abnormalities, with persistent hypertension in five and urinary dysfunction in three. Areflexia or hyporeflexia was found in all. Cerebrospinal fluid cytoalbuminogenic dissociation was found in 17, and MRI scans of brain and spinal cord were normal in the four children so studied. Nerve conduction studies were never normal, even when performed within 3-6 days (n = 9), with decreased motor amplitudes, and prolonged distal and F wave latencies being the most frequent early abnormalities. Leg pain and gait abnormalities in children suggest GBS and electrodiagnostic studies are essential to confirm the diagnosis. Prognosis is generally excellent.
There are no large clinical therapeutic trials for childhood GBS, and current recommendations, including plasmapheresis or intravenous immune globulin, are based on studies with adults. As in adults, and even prior to initiating specific therapy, a decision must be made regarding admission to an intensive care unit and when to consider mechanical ventilation. Patients require close monitoring of motor strength, blood pressure, heart rate, and sphincteric and respiratory function, and any patient admitted with GBS should be evaluated frequently. Children with reduced vital capacity (< 20 ml/kg), bulbar palsy, or rapidly progressive weakness should be admitted to a pediatric intensive care unit.
