West syndrome is a rare, severe form of epilepsy with onset in infancy and early childhood. It combines episodes of epileptic spasms that occur in a cluster, an abnormal pattern of interictal electroencephalogram termed as hypsarrhythmia and neuropsychomotor delay. The syndrome mainly results from brain dysfunction in the prenatal, perinatal, or postnatal period and focal lesions early in life may secondarily affect other sites in the brain presenting with some degree of developmental delay and mental retardation. The oral manifestations vary to a large extent presenting as generalized tooth wear, gingival enlargement, multiple white spot lesions, and a high-arched palate. This case report highlights the importance of early diagnosis, various clinical features, and management in a pediatric patient with West syndrome.
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