Guillain-Barré syndrome: perspectives with infants and children.
An acute flaccid paraparesis or ascending quadriparesis in an infant or child constitutes a very important pediatric neurology emergency. The Guillain-Barré syndrome (GBS) is the most frequent cause. This is primarily an autoimmune, post-infectious, demyelinating, peripheral nervous system process. A small percentage of children develop a primary axonal process not unlike that identified more commonly in China. Because of the potential for acute respiratory compromise, any child suspected of having GBS needs immediate hospitalization. The major considerations in differential diagnosis include transverse myelitis, toxic neuropathies, tick paralysis, infantile botulism, myasthenia gravis, and dermatomyositis. On occasion, some younger children present with an acute severe pain syndrome that may mask as a pseudo-encephalopathy. Another clinical variant is the Miller-Fisher syndrome characterized by ataxia, ophthalmoparesis, and areflexia. This is associated with a high frequency of the anti-GQ-1-b antibodies. Although most children with GBS have a relatively benign clinical course, some become very ill and require intubation with intensive care monitoring. Immunomodulating treatment should be used for any child who loses the ability to walk. To date, no well-controlled study has been completed analyzing the relative merits of the two most commonly used therapies, namely plasmapheresis or intravenously administered immunoglobulin.
Jones
H R
HR
Department of Neurology, Lahey Clinic, Burlington, MA 01805, USA.
D000208:Acute Disease; D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D002648:Child; D002675:Child, Preschool; D003131:Combined Modality Therapy; D003937:Diagnosis, Differential; D004576:Electromyography; D005260:Female; D020275:Guillain-Barre Syndrome / Q000150:complications* / Q000628:therapy; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D007231:Infant, Newborn; D008297:Male; D008475:Median Nerve / Q000503:physiopathology; D019846:Miller Fisher Syndrome / Q000175:diagnosis; D020335:Paraparesis / Q000175:diagnosis / Q000209:etiology* / Q000503:physiopathology; D010956:Plasmapheresis / Q000379:methods; D011241:Prednisone / Q000627:therapeutic use; D013497:Sural Nerve / Q000503:physiopathology
D000893:Anti-Inflammatory Agents; D016756:Immunoglobulins, Intravenous; D011241:Prednisone