Profile of Worster Drought Syndrome (WDS)

Abstract

Introduction: Worster Drought syndrome (WDS) is a type of cerebral palsy that affects the muscles around the mouth and throat. This causes problems with swallowing, feeding, talking, and dribbling. Although the impairments are predominantly motor, a range of cognitive, behavioral, and seizures also exist. These features point towards the involvement of the perisylvian area of the cerebral cortex. Purpose of the Study: In WDS, the predominant impairment is a profound expressive communication problem and feeding difficulties; hence, it is under-recognized and even under-reported. Besides, these children have a specific phenotype which when recognized early can make a significant difference in management. This study aims to know the etiology and profile of WDS. Materials and Methods: This is a prospective cohort study in a tertiary care hospital from January 2018 to December 2019. All patients who met the absolute criterion for the diagnosis of WDS were included. A detailed history, clinical examination findings, relevant investigations like CT/MRI brain, and EEG were recorded in a predesigned proforma, and results were analyzed. Results: A total of 83 children (M:F = 52:31) presented within the age group of 2 years to 18 years. Seventy (84.33%) of them were delivered by a normal vaginal route. All children had global developmental delay, expressive speech delay, and spasticity. Other features were undernutrition 75(90%), drooling 66(79.51%), seizures 44(52.60%), and prolonged feeding 42(50.60%). Birth asphyxia was present in 72 (86.70%) cases, but neuroimaging was suggestive of perisylvian gliosis in 80 (96.38%) cases. Conclusions: WDS should be considered in children with cerebral palsy if mild motor delay with a predominant expressive language delay is present without hearing impairment and autistic features. In India, birth asphyxia is the most common cause.