Atypical Clinical Presentations of Pediatric Acute Immune-Mediated Polyneuropathy

Guillain-Barré syndrome (GBS) is the most common cause of acute flaccid paralysis in children. During the acute phase, the disorder can be life-threatening by involving the respiratory muscles and the autonomic nervous system. Nevertheless, the prognosis is good, and most children achieve full recovery. The aim of this study was to characterize the clinical and electrophysiologic findings in children with Guillain-Barré syndrome referred to a tertiary center in Israel. A retrospective database review from 2009 to 2015 identified 39 children. Data on clinical presentation, respiratory complications, and long-term neurologic outcomes were collected. Atypical clinical findings at admission included asymmetric weakness in 23%, nonascending weakness in 30%, and normal deep tendon reflexes in 28%. Eight children were later diagnosed with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). Electrophysiologic findings, available in 12 patients with Guillain-Barré syndrome, revealed acute inflammatory demyelinating polyradiculoneuropathy (AIDP) in 4 (33.5%), AIDP with secondary axonal changes in 3 (25%), and acute motor axonal neuropathy (AMAN) subtype in 4 (33.5%); 8% had no abnormal findings. On follow-up, 71% of the children with Guillain-Barré syndrome fully recovered compared to 14% of the children with CIDP. Corresponding rates of neurologic sequelae were 29% and 86%. Clinicians should be alert to the atypical presenting symptoms of Guillain-Barré syndrome, which occur in a significant proportion of children.