Severe myoclonic epilepsy of infancy(SMEI) is a condition beginning with recurrent, prolonged febrile convulsion in normal children, followed within months to 4 years by generalized tonic clonic seizures, partial seizures, atypical absences, myoclonic seizures and status epilepticus. The seizures are generally difficult to control. Carbamazepine which is appropriate for partial seizures, is not effective and may aggravate generalized seizures, but sodium valproate has been reported to be helpful. The evolution is always bad with persistent seizures and mental retardation. We experienced a severe myoclonic epilepsy of infancy in a 16-month-old male patient who had episodes of prolonged febrile convulsions followed by mixed type of seizures. We report a case of SMEI with a brief review of literatures.
Carbamazepine ; Child ; Epilepsies, Myoclonic* ; Humans ; Infant ; Intellectual Disability ; Male ; Seizures ; Seizures, Febrile ; Status Epilepticus ; Valproic Acid

We experienced a case of Rotor syndrome in an 8 year 6 month old boy who presented with icteric sclera and icteric skin on whole body. His clinical and laboratory characteristics were as follows; 1) Jaundice appeared from several months ago before admission without any clinical disturbance. 2) Direct bilirubin was more increased than indirect bilirubin. 3) Plasma indocyanine green (ICG) kinetics test revealed hepatic excretory in this patient that after intravenous injection of a standard dose of ICG (0.5mg/kg), its initial plasma disappearance is decreased, resultin in markedly increased retention of the dye 45 to 50 minutes after administration. 4) Oral cholangiography, ultrasonogram, and computed tomogram of abdomen were normal. 5) Liver biopsy specimen showed no dark brown pigmentations in the hepatic cells and any other pathologic abnormalities. 6) Nearly similar clinical and laboratory findings were observed 26 months later.
Abdomen ; Bilirubin ; Biopsy ; Cholangiography ; Hepatocytes ; Humans ; Hyperbilirubinemia, Hereditary* ; Indocyanine Green ; Infant ; Injections, Intravenous ; Jaundice ; Kinetics ; Liver ; Male ; Pigmentation ; Plasma ; Sclera ; Skin ; Ultrasonography

No abstract available.
Child* ; Humans ; Prognosis*

To elucidate the clinical characteristics-especially its clinical pattern and outcome-of children with encephalopathy, we carried out a retrospective review on medical records of 45 patients who were initially diagnosed as encephalopathy except mumps meningitis, Reye's syndome and neonatal hypoxic ischemic encephalopathy at the Department of pediatrics, Hanyang University children's hospital from Jaunary 1986 to February 1994. The results were summarized as follows: 1) Twenty five were male and twenty were female with male to female ratio 1.3:l. 2) There were no association with death rate and conscious level, abnormal liver function test, and sugar level in CSF on admission. 3) Death rate (33.3%) of group with seizure was higher than that (13.3%) of group without seizure on admission, but there was no statistical significance (p=0.28). 4) Death rate (7.5%) of group (l) which initial therapy started before 12hours was lower than that (43.4%) of group (ll) which initial therapy started after 12hours, but there was no statistical significance (p=0.09). However complete recovery rate (66.7%) of the former group was higher than that (26.7%) of the latter group with statistical significance (p<0.05). Therefore, early diagnosis and treatment before 12hours was essential to recovery and good prognosis in childern with acute encephalopathy.
Child* ; Early Diagnosis ; Female ; Humans ; Hypoxia-Ischemia, Brain ; Liver Function Tests ; Male ; Medical Records ; Meningitis ; Mortality ; Mumps ; Pediatrics ; Prognosis ; Retrospective Studies ; Seizures

No abstract available.

The finger extension reflex was performed to the 419 normal infants without CNS abnormalities that visited to the well baby clinic and out-patient department, and so to the patients with CNS abnormalities on the brain sonography and brain CT under the age of 2 months from May 1992 to September 1993 in the department of pediatrics, Hanyang university hospital. The results were summerized as follows 1) The positive rates of the finger extension reflex according to age in month on the normal infants from birth to 8 months of age were 100%, 100%, 100%, 72.3%, 57.1%, 28.6%, 17.5%, 6.3% and 0% respectively, and the reflex was almost lost after the age of 5 months. 2) Of 21 patients with brain pathology on the brain sonography and/or brain CT, the finger extension reflexes were absent on two patients with grade 3~4 intraventricular hemorrhage, one with hypoxic brain damage and one with severe brain parenchymal hemorrhage, partially responded on two patients with brain edema and one with moderate-sized brain parenchymal hemorrhage, and completely responded on 11 patients with mild intracranial hemorrhage, two with hydrocephalus and one with aneurysm of Galen vein. 3) Because there were no cases with localized brain pathology, we couldn't find the differences of the finger extension reflexes associated with the location of brain pathology. In conclusion, we found that the finger extension reflex may be lost from 3 months through 6 months of age. With further study of normal premature babies and more infants with brain pathology, we may use the finger extension reflex as one of the primitive reflexes and predict severe CNS abnormalities.
Aneurysm ; Brain ; Brain Diseases ; Brain Edema ; Cerebral Veins ; Fingers* ; Hemorrhage ; Humans ; Hydrocephalus ; Hypoxia, Brain ; Infant ; Intracranial Hemorrhages ; Outpatients ; Parturition ; Pediatrics ; Reflex*

No abstract available.
Meningitis*

No abstract available.
Child* ; Ductus Arteriosus, Patent* ; Humans

No abstract available.
Ependymoma*

No abstract available.
Brain Neoplasms* ; Brain* ; Child* ; Humans