AI Summary & Key Takeaways
AI InsightTopiramate and valproate were administered, by which convulsions were partially controlled.
📌 3 Key Takeaways
Abstract
Although it is known that hypoglycemia could cause severe negative effects on brain development and also infantile spasm, it has not been reported that transient hyperinsulinemic hypoglycemia, which spontaneously improves over a short time, may cause infantile spasms. Infantile spasm is a disorder of early childhood typically seen in the first year of life, characterized by the occurence of sudden, brief, generally bilateral and symetric motor spasms of the muscles of the trunk, neck and limbs. Infantile spasms are classified as idiopathic or symptomatic. The most common form symptomatic infantile spasms is due to prenatal, perinatal or postnatal insults. A 3140 g, full-term baby was admitted with poor sucking and feeding difficulty on the postnatal second day. The patient was followed-up with the diagnosis of hyperinsulinemic hypoglycemia and intravenous glucose infusion (15 mg/kg/min) was administered, but due to the persistence of hypoglycemia, diazoxide treatment was initiated. Hypoglycemia was not observed under diazoxide treatment and the drug was gradually decreased; treatment was terminated on the 21st day. The patient was continuously normoglycemic during follow-up and admitted with flexor spasms on the 45th day. A modified hypsarrhythmia pattern was detected in the electroencephalography. On cranial magnetic resonance imaging, diffuse cystic encephalomalacia areas were observed in the temporoparietal white and gray matter. The convulsions were not completely controlled with adrenocorticotropic hormone and vigabatrin treatments. Topiramate and valproate were administered, by which convulsions were partially controlled.
Özet
Hipogliseminin beyin gelişimi üzerine olan olumsuz etkileri ve infantil spazma neden olabileceği iyi bilinmekle birlikte, kısa sürede ve spontan olarak iyileşen hiperinsülinemik hipogliseminin infantil spazma neden olabileceği bildirilmemiştir. İnfantil spazm genellikle yaşamın ilk yılında ortaya çıkan, gövde, boyun ve bacak kaslarının ani, kısa süreli ve genellikle bilateral simetrik motor spazmları olarak tanımlanır. En sık görülen formu olan semptomatik infantil spazm prenatal, perinatal ve postnatal olayalara bağlı ortaya çıkar. Zamanında 3140 gram olarak doğan bebek postnatal ikinci gününde zayıf emme ve beslenme güçlüğü yakınmaları ile getirildi. Hiperinsülinemik hipoglisemi tanısı konan ve intravenöz glukoz infüzyonuna (15 mg/kg/dk) rağmen hipogisemisi devam eden olguya diazoksit başlandı. Bu tedavi ile hipoglisemisi gözlenmeyen olgunun diazoksit dozu kademeli olarak azaltılarak 21. günde kesildi. İzlemde sürekli normoglisemik olan bebek 45. günde fleksör spazmlar nedeni ile başvurdu. Elektroensefalografide modifiye hipsartimi paterni saptandı. Kraniyal manyetik rezonans görüntülemede temperopariyetal beyaz ve gri cevherde kistik ensefalomalazik alanlar gözlendi. Adrenokortikotropik hormon ve vigabatrin tedavileri ile nöbetleri kontrol altına alınamayan olguda topiramat ve valproat tedavilerinden kısmi fayda görüldü.