International Journal of Hospital Pharmacy

  • Case report of west syndrome: a rare severe epilepsy in infants

    West syndrome (WS) also known as infantile spasms, is severe form of epilepsy of early childhood was first describe in 1840s. WS presents with myoclonic-tonic seizures (spasms) characterized by flexor, extensor or mixed movements with distinct electroencephalogram (EEG) pattern of hypsarrthmia and psychomotor arrest. Prevalence rate is ~ 4.0 cases per 10,000 children. Peak age of beginning is between 3 and 7 months; onset after 18 months is rare. Recent guidelines from the American Academy of Neurology and the Child Neurology Society for medical treatment of WS, recommends that ACTH is probably effective and vigabatrin is possibly effective in cessation of spasms of hypsarrhythmia. Although discovered 160 years ago, still its diagnosis, assessment and management continue to create many challenges to health care professionals and affected families. In this case of a 3 yr old male child was admitted for long period of almost 2 months before being discharged. This case was confirmed early but due to its complexity it took long time to get complete resolve of situation. ACTH with Valproate was found to effective; though ACTH showed some reactions still it was considered the best available medicine for WS. In conclusion, early detection and referral to a pediatric neurologist for clinical evaluation and prompt effective treatment is strongly recommended as it may improve prognosis.