(Kiosk 6) Functional Recovery of Presumed Pharyngeal-cervical-brachial Variant Guillain Barre Syndrome Secondary to COVID-19 in a Pediatric Patient: A Case Report

Case Diagnosis: Pharyngeal-Cervical-Brachial Variant Guillain Barre Syndrome

Case Description or Program Description: A 9-year-old, previously healthy male with recent COVID, presented with progressive bilateral upper extremity weakness, absent upper limb proprioception, poor head control, hoarse voice, ataxia, cervical adenopathy, shuffling gait and hypercarbic respiratory failure secondary to neuromuscular weakness. He was admitted with the diagnosis of pharyngeal-cervical-brachial variant Guillain Barre Syndrome requiring tracheostomy with ventilatory support, gastrostomy, plasma exchange therapy and a five day course of IVIg. Electrodiagnostic study 6 weeks after symptoms onset and 2 weeks after last dose of IVIg revealed severe, non-uniform, chronic and ongoing, sensorimotor polyneuropathy predominately demyelinating with secondary axonal features.

Rehabilitation medicine assisted with neuropathic pain management, pulmonary rehabilitation, weaning sedatives, sleep management, bowel regimen, co-management with psychiatry, dysphagia management, and coordination of goals of care with the therapy team.

Setting: Inpatient Rehabilitation

Assessment/Results: By discharge, he gained upper extremity range of motion and strength, decannulated to room air, advanced to regular diet with removal of gastrostomy tube, improved from dependency to modified independence in all ADLs, and ambulated moderate distances with walker and close supervision.

Discussion (relevance): Pharyngeal-Cervical-Brachial (PCB) variant Guillain Barre syndrome is thought to be a focal form of acute motor axonal neuropathy, separate from acute inflammatory demyelinating polyneuropathy with axonal neurophysiological findings rather than demyelinating findings.
PCB is defined as rapidly progressive oropharyngeal and cervicobrachial weakness associated with hyporeflexia or areflexia in the absence of ophthalmoplegia. It is very rare in children and there is wide variation in neurorecovery outcomes. Previous reports describe everything from full recovery to poor recovery, even after many months.

Conclusions: This case represents an excellent rehabilitative outcome in a child with severe PCB. Through this multidisciplinary effort, our patient was able to be at a supervisory/contact guard level for most tasks and was able to ambulate out of the hospital. While this syndrome is very rare - especially in children - it elucidates the appreciation for basic rehabilitation principles in order to restore functionality in a complex condition.