BACKGROUND: Persistent hyperinsulinemic hypoglycemia of infancy (PHHI) is characterized by disproportional secretion of insulin from pancreatic beta-cells. Although one of the manifestations of hypoglycemia is West syndrome, it is rarely reported in PHHI. PATIENT REPORT: A 6 month-old girl who was followed up with the diagnosis of PHHI was admitted to hospital with the complaint of jerky movements at her extremities. EEG revealed the typical pattern of hypsarrhythmia leading to the diagnosis of West syndrome. CONCLUSION: To our knowledge, there is only one report in the literature of West syndrome as a manifestation of PHHI, and that was the hyperammoniemic form of the disease. The present report is the first of normoammoniemic PHHI leading to West syndrome. We wish to highlight the potential risks of PHHI, especially in inadequately treated patients, and to emphasize that close neurological follow-up is very important in children who suffer from PHHI.
BACKGROUND: Persistent hyperinsulinemic hypoglycemia of infancy (PHHI) is characterized by disproportional secretion of insulin from pancreatic beta-cells. Although one of the manifestations of hypoglycemia is West syndrome, it is rarely reported in PHHI. PATIENT REPORT: A 6 month-old girl who was followed up with the diagnosis of PHHI was admitted to hospital with the complaint of jerky movements at her extremities. EEG revealed the typical pattern of hypsarrhythmia leading to the diagnosis of West syndrome. CONCLUSION: To our knowledge, there is only one report in the literature of West syndrome as a manifestation of PHHI, and that was the hyperammoniemic form of the disease. The present report is the first of normoammoniemic PHHI leading to West syndrome. We wish to highlight the potential risks of PHHI, especially in inadequately treated patients, and to emphasize that close neurological follow-up is very important in children who suffer from PHHI.