Guillain-Barré syndrome in children: Treatment and prognosis
- Monique M Ryan, FRACP
Monique M Ryan, FRACP
- University of Melbourne
- Head, Department of Neurology
- Royal Children's Hospital Melbourne
- Section Editors
- Douglas R Nordli, Jr, MD
Douglas R Nordli, Jr, MD
- Section Editor — Pediatric Neurology
- Chief of Neurology
- Children’s Hospital Los Angeles
- Vice Chair of Neurology
- USC Keck School of Medicine
- Adrienne G Randolph, MD, MSc
Adrienne G Randolph, MD, MSc
- Section Editor — Pediatric Critical Care Medicine
- Professor of Anaesthesia and Pediatrics
- Harvard Medical School
The acute immune-mediated polyneuropathies are classified under the eponym Guillain-Barré syndrome (GBS). Typically, GBS presents as an acute monophasic paralyzing illness provoked by a preceding infection. The main modalities of therapy for GBS are intravenous immune globulin and plasma exchange. Even before initiating therapy, common questions that arise include whether the patient needs intensive care, assisted ventilation, or other supportive measures.
This topic will discuss the management and prognosis of GBS in children. Other aspects of GBS in children are discussed separately. (See "Guillain-Barré syndrome: Pathogenesis" and "Guillain-Barré syndrome in children: Epidemiology, clinical features, and diagnosis".)
CLINICAL FEATURES AND DIAGNOSIS
The clinical features and diagnosis of GBS are reviewed here briefly and discussed in detail separately. (See "Guillain-Barré syndrome in children: Epidemiology, clinical features, and diagnosis".)
Acute inflammatory demyelinating polyneuropathy (AIDP) is the prototype of GBS, and is the most common form in North America, Europe and most of the developed world. Acute motor axonal neuropathy (AMAN) is a pure motor form of GBS. It occurs mainly in northern China, but is also a common variant of GBS in other locations, including Japan, Mexico, and South America. The clinical presentation is similar in the two types. In most cases, neurologic symptoms develop two to four weeks after what initially appears to be a benign febrile respiratory or gastrointestinal infection. The predominant symptoms of GBS at presentation in children are pain and gait difficulty. Lower extremity symmetric weakness may ascend over hours to days to involve the arms, and the muscles of respiration in severe cases. In those with cranial neuropathy, the facial nerve is most commonly affected, resulting in bilateral facial weakness. Autonomic dysfunction occurs in approximately one-half of children with GBS. Physical examination reveals symmetric weakness with diminished or absent reflexes. Most patients reach the nadir of their function within two to four weeks, followed by a slow return of function over the course of weeks to months. (See "Guillain-Barré syndrome in children: Epidemiology, clinical features, and diagnosis", section on 'Clinical features'.)
Electrophysiologic studies are the most specific and sensitive tests for diagnosis of GBS. Characteristic findings from lumbar puncture include a normal opening pressure and cerebrospinal fluid with fewer than 10 cells (typically mononuclear) and an elevated protein concentration (>45 mg/dL). Spinal MRI with administration of gadolinium frequently shows enhancement of the spinal nerve roots and cauda equina during the first weeks after the onset of symptoms. (See "Guillain-Barré syndrome in children: Epidemiology, clinical features, and diagnosis", section on 'Diagnosis'.)
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- CLINICAL FEATURES AND DIAGNOSIS
- MONITORING AND SUPPORTIVE CARE
- Need for intensive care
- Need for assisted ventilation
- Autonomic dysfunction
- Other supportive measures
- Intravenous immune globulin
- Plasma exchange
- IVIG versus plasma exchange
- SUMMARY AND RECOMMENDATIONS