The Journal of Pediatric Research

Cystic encephalomalacia and infantile spasm as a complication of transient and mild hyperinsulinemic hypoglycemia []
. 2016; 3(2): 0-0

Cystic encephalomalacia and infantile spasm as a complication of transient and mild hyperinsulinemic hypoglycemia

Ahmet Anık1, Ayşe Anık2, Tolga Ünüvar1, Ayşe Fahriye Tosun3, Şiar Dursun3, Abdullah Barış Akcan2, Yasemin Durum4, Münevver Kaynak Türkmen2
1Adnan Menderes University Faculty Of Medicine, Department Of Pediatric Endocrinology, Aydın, Turkey
2Adnan Menderes University Faculty Of Medicine,department Of Neonatology, Aydın, Turkey
3Adnan Menderes University Faculty Of Medicine,department Of Pediatric Neurology,
4Adnan Menderes University Faculty Of Medicine,department Of Radiology, Aydın, Turkey

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 first year of life characterized by the occurence of sudden, brief, generally bilateral and symetric motor spasms of muscles of the trunck, 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 (ACTH) and vigabatrin treatments, and topiramate and valproate were administered, by which convulsions were partially controlled.

Keywords: infantile spasm, transient hypoglycemia, hyperinsulinemia


Geçici ve hafif hiperinsülinemik hipogliseminin komplikasyonu olarak kistik ensefalomalazi ve infantil spazm

Ahmet Anık1, Ayşe Anık2, Tolga Ünüvar1, Ayşe Fahriye Tosun3, Şiar Dursun3, Abdullah Barış Akcan2, Yasemin Durum4, Münevver Kaynak Türkmen2
1Adnan Menderes Üniversitesi Tıp Fakültesi, Çocuk Endokrinolojisi Bilim Dalı, Aydın, Türkiye
2Adnan Menderes Üniversitesi Tıp Fakültesi, Neonatoloji Bilim Dalı, Aydın, Türkiye
3Adnan Menderes Üniversitesi Tıp Fakültesi, Çocuk Nörolojisi Bilim Dalı, Aydın, Türkiye
4Adnan Menderes Üniversitesi Tıp Fakültesi, Radyoloji Anabilim Dalı, Aydın, Türkiye

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 (ACTH) ve vigabatrin tedavileri ile nöbetleri kontrol altına alınamayan olguda topiramat ve valproat tedavilerinden kısmi fayda görüldü.

Anahtar Kelimeler: infantil spazm, geçici hipoglisemi, hiperinsülinemi


Ahmet Anık, Ayşe Anık, Tolga Ünüvar, Ayşe Fahriye Tosun, Şiar Dursun, Abdullah Barış Akcan, Yasemin Durum, Münevver Kaynak Türkmen. Cystic encephalomalacia and infantile spasm as a complication of transient and mild hyperinsulinemic hypoglycemia. . 2016; 3(2): 0-0

Corresponding Author: Ahmet Anık, Türkiye


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