Harlequin Syndrome, Apnea, and Acute on Chronic Hemiparesis: An Atypical Pediatric Case of Chiari I With Holocord Syringohydromyelia

Abstract

A four-year-old, right-handed, term female, with mild global developmental delay, presented to the emergency department for evaluation of progressive right hemiparesis. Her development was significant for walking at 18 months and expressive speech delay. At age 3.5 years, she was diagnosed with Harlequin syndrome (Fig 1) after multiple episodes of left hemifacial flushing with physical exertion. Concurrently, she began dragging her right leg and was diagnosed with hemiplegic cerebral palsy by an outside hospital.