Clinical Review of the of Seizures among Children / 대한소아신경학회지

PURPOSE: The purpose of this study was to evaluate childhood seizures to provide appropriate medical services. METHODS: We retrospectively reviewed the medical records of 221 chidren under 18 years of age with seizures (excluding febrile convulsion), who were admitted to the pediatric department of Sacred Heart Hospital, Hallym University from 2007 to 2009. RESULTS: The male to female was 1.3:1 and the peak age was 6 years or less, accounting for 63%. The most common causes of seizures according to age were listed as follows; hypocalcemia (41%) and hypoxic ischemic encephalopathies (41%) in the neonatal period, benign convulsion with mild gastroenteritis (BCwMG, 47%) in preschool children (1month and 5 years), and unprovoked seizure (80%) more than 6 years. Electroencephalogram and neuroimaging abnormalities were found in the ratio from 20% and 14% respectively. CONCLUSION: Seizure is the most common neurologic disease in the childhood. The above results reveal that the causes of childhood seizures in the different age group are different. Therefore, the exact diagnosis of disease according to age is needed. We hope that further clinical studies on this topic will be performed.

A Case of Infantile Alexander Disease / 대한소아신경학회지

Alexander disease(AD) is a rare fatal demyelinating disorder, caused by the mutation of glial fibrillary acidic protein(GFAP) gene. It is characterized by progressive demyelination of central nervous system, and the accumulation of Rosenthal fibers within astrocytes. It is divided into three group: infantile, juvenile, and adult. The infantile type is most common, has onset during the first 2 years of life. It shows macrocephaly and psychomotor delay, spastic paraparesis, seizure, and feeding problems, and usually dies within the first decade. The severity of the pathological changes depend on the age of onset. Radiological study revealed white matter loss, usually with frontal predominance. It is diagnosed by DNA analysis. We present case of a 10-month-old male patient with AD. He had focal seizures, demyelination in the frontal lobe in MRI, and the presence of a K86E mutaion in the GFAP gene, involving the replacement of adenosine with guanine.