Brain damage in glycogen storage disease type I
Copyright policy )Brain damage in glycogen storage disease type I
To investigate brain morphology and function in patients with glycogen storage disease type I (GSDI).Nineteen patients (13 females and 6 males, aged 0.9-22.6 years) and 38 sex- and age-matched controls entered the study. Neurological examinations, psychometric tests (IQ, tests of performance and verbal abilities), standard electroencephalogram (EEG), somatosensory (SEPs), visual (VEPs), and brain-stem auditory evoked potentials (BAEPs), and brain magnetic resonance imaging (MRI) were performed.The results of tests of performance ability were lower in patients than in controls (P <.05). The prevalence of abnormal EEG findings (26.3% versus 2.6%), VEPs (38.4% versus 7.7%), SEPs (23.0% versus 0%), and BAEPs abnormalities (15.7% versus 0%) was higher in patients than in controls (P <.05). MRI pattern was altered in 57.1% of patients and was normal in all controls (P <.05). Both results of tests of performance ability and BAEPs abnormalities significantly correlated with the frequency of admissions for hypoglycemia, whereas EEG abnormalities correlated with dietary compliance (P <.05).Brain damage, probably caused by recurrent severe hypoglycemia, may be present in patients with GSDI.
Adult, Male, Neurologic Examination, Adolescent, Brain Diseases, Metabolic, Infant, Electroencephalography, Glycogen Storage Disease Type I, Magnetic Resonance Imaging, Hypoglycemia, Italy, Adolescent, Adult, Brain Diseases; Metabolic; epidemiology/etiology/pathology, Case-Control Studies, Child, Child; Preschool, Electroencephalography, Evoked Potentials, Female, Glycogen Storage Disease Type I; complications, Humans, Hypoglycemia; complications, Infant, Italy; epidemiology, Magnetic Resonance Imaging, Male, Neurologic Examination, Case-Control Studies, Child, Preschool, Humans, Female, Child, Evoked Potentials
Adult, Male, Neurologic Examination, Adolescent, Brain Diseases, Metabolic, Infant, Electroencephalography, Glycogen Storage Disease Type I, Magnetic Resonance Imaging, Hypoglycemia, Italy, Adolescent, Adult, Brain Diseases; Metabolic; epidemiology/etiology/pathology, Case-Control Studies, Child, Child; Preschool, Electroencephalography, Evoked Potentials, Female, Glycogen Storage Disease Type I; complications, Humans, Hypoglycemia; complications, Infant, Italy; epidemiology, Magnetic Resonance Imaging, Male, Neurologic Examination, Case-Control Studies, Child, Preschool, Humans, Female, Child, Evoked Potentials
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