TY  - JOUR
TI  - Evaluation of central nervous system in patients with glycogen storage disease type 1a
AB  - Aydemir Y, Gürakan F, Saltık-Temizel İN, Demir H, Karlı-Oğuz K, Yalnızoğlu D, Topçu M, Özen H, Yüce A. Evaluation of central nervous system in patients with glycogen storage disease type 1a. Turk J Pediatr 2016; 58: 12-18. We aimed to evaluate structure and functions of central nervous system (CNS) in children with glycogen storage disease (GSD) type 1a.Neurological examination, psychometric tests, electroencephalography (EEG), magnetic resonance imaging (MRI), visual evoked potentials(VEP) and brainstem auditory evoked potentials (BAEP) were performed. The results were compared between patients with good and poor metabolic control and healthy children.Twenty-three patients with GSD type 1a were studied. Twelve patients were in poor metabolic control group and 11 patients in good metabolic control group. Five patients had intellectual disability, 10 had EEG abnormalities, seven had abnormal VEP and two had abnormal BAEP results. MRI was abnormal in five patients. There was significant correlation between the number of hypoglycemic attacks and MRI abnormalities. Central nervous system may be affected in GSD type 1a even in patients with normal neurologic examination. Accumulation of abnormal results in patients with poor metabolic control supports the importance of metabolic control in GSD type 1a
AU  - Yalnizoglu, Dilek
AU  - DEMİR, Hülya
AU  - Yüce, Aysel
AU  - Gürakan, Figen
AU  - OĞUZ, Kader Karlı
AU  - AYDEMİR, Yusuf
AU  - Topçu, Meral
AU  - saltik temizel, inci nur
AU  - Özen, Hasan
PY  - 2016
JO  - Turkish Journal of Pediatrics
VL  - 58
IS  - 1
SN  - 0041-4301
SP  - 12
EP  - 18
DB  - TRDizin
UR  - http://search/yayin/detay/260902
ER  -