Worster-Drought syndrome with progressive symptomatic improvement in early infancy

Abstract

Worster-Drought syndrome (WDS), or congenital suprabulbar paresis, is characterised by congenital dysarthria, dysphagia and other pseudobulbar paresis without structural abnormalities around the Sylvian fissure on imaging. This rare syndrome is challenging to diagnose, particularly in preterm infants. This report describes a low-birth-weight female infant with WDS who had no sucking reflex from birth, airway obstruction due to saliva retention and muscle rigidity, who was diagnosed with the syndrome at a postconceptional age (PCA) of 1 month. She was discharged with only home oxygen therapy as respiratory support at a PCA of 3 months after gradual improvement in her clinical symptoms. Diagnosis of WDS is difficult in the early postnatal period in preterm cases owing to prematurity but should be suspected when bulbar palsy, including absence of the sucking reflex, persistent dysphagia and obstructed breathing, persists beyond a PCA of 40 weeks and when muscle stiffness is present.