∙ The main pathogen for benign convulsions with mild gastroenteritis (CwG) was previously rotavirus; however, cases associated with norovirus are increasing. ∙ CwG is characterized by clustered generalized seizures. Electroencephalography and magnetic resonance imaging show transiently abnormal findings in the acute phase that eventually normalize with progression. Its prognosis is good, and long-term treatment is unnecessary. ∙ There are many reports on the pathophysiological mechanism of CwG, which remains unclear. |
Quadriplegic children with cerebral palsy are more susceptible to osteoporosis because of various risk factors that interfere with bone metabolism. Pamidronate is effective for pediatric osteoporosis, but there are no guidelines for optimal dosage or duration of treatment in quadriplegic children with osteoporosis. We aimed to evaluate the efficacy of low-dose pamidronate treatment in these patients. Ten quadriplegic patients on antiepileptic... |
We report a case of a 5-year-old girl who developed left hemiparesis and left facial palsy, 6 days after the initiation of fever and respiratory symptoms due to pneumonia. Chest radiography, conducted upon admission, showed pneumonic infiltration and pleural effusion in the left lung field. Brain magnetic resonance imaging showed acute ischemic infarction in the right middle cerebral artery territory.... |
Insulinoma, which arises from insulin-producing pancreatic beta cells, is a rare tumor in children. Only 5%-10% of insulinomas are malignant and undergo metastasis. We report a case of an 11-year-old girl who experienced hypoglycemia-related seizures induced by an insulinoma; after resection of the primary tumor, she developed hepatic focal nodular hyperplasia (FNH). Laboratory test results indicated marked hypoglycemia with hyperinsulinemia.... |
Benign convulsion with mild gastroenteritis (CwG) is a type of afebrile seizure that occurs in children. CwG is defined as a convulsion in a previously healthy child with no known central nervous system infection or encephalopathy, accompanying mild diarrhea without fever, electrolyte imbalance, or moderate to severe dehydration. Convulsions in CwG are characterized by multiple brief episodes of generalized or... |
Purpose : This study evaluated the clinical characteristics of brain tumors in children according to their location, the parental delay and the doctor's delay between the onset of symptoms and the diagnosis of a pediatric brain tumor. In addition, this study compared the relationship between the pre-diagnostic symptomatic interval and the tumor location. Methods : A retrospective study was undertaken of... |
Purpose : Among perinatal risk factors, neonatal seizures are one of the strongest independent discriminators of adverse outcome, representing high risks of mortality and neurologic morbidity. This study was undertaken to evaluate the neurologic outcome of neonatal status epilepticus according to underlying etiology, seizure pattern, onset time, and duration. Methods : We reviewed retrospectively 36 neonates(19 males, 17 females) with status... |
Atypical teratoid/rhabdoid tumor may arise at any central nervous system location, but it is most commonly located in the cerebellum(60 percent). The incidence of this tumor remains unclear but it occurs most commonly in children less than 2 years of age. This highly malignant tumor shows a rapid progression and nonspecific radiologic findings. We report a case of primary intracranial... |
Purpose : To evaluate the clinical characteristics of infants and young children who had developmental delay without delayed myelination and dysmyelination. Methods : We retrospectively reviewed 59 cases of developmental disability between July 1996 and June 2001 at Inha University Hospital. Twenty-eight patients showed normal myelination(Group I), while thirty-one patients showed delayed myelination(Group II) by brain MRI. The following clinical records... |
Krabbe disease is a rare autosomal recessive disorder clinically characterized by retardation in motor development, prominent spasticity, seizures, and optic atrophy. Pathologically, there are many globoid cells in the white matter, in addition to the lack of myelin and the presence of severe gliosis. Hence Krabbe disease is known as globoid cell leukodystrophy. Biochemically, the primary enzymatic deficiency in Krabbe... |
Rhizomelic chondrodysplasia punctata(RCDP) is a rare autosomal recessive disorder clinically characterized by symmetrical shortening of the proximal limbs, contractures of joints, a typical dysmorphic face, cataracts, and itchyosis. Patients with RCDP can be subdivided into three subgroups based on biochemical analysis and complementation studies. RCDP type I results from mutations in the PEX7 gene encoding the peroxisomal targeting signal type... |
Centrinuclear myopathy, an uncommon condition, is one of the congenital myopathies. It is characterized by the presence of central nuclei of muscle cells which can be detected on electronmicroscopy. It is believed to arise as a result of maturational arrest with persistence of microtubes postnatally. We report a boy with generalized hypotonia and muscle weakness who was diagnosed as centrinuclear... |
We experienced a case of partial DiGeorge syndrome in a 35+5 week premature female infant presented with micrognathia, fish-shaped mouth, beaked nose, nasal regurgitation, obstructive sleep apnea, velopharyngeal insufficiency and late onset hypocalcemic seizures. The chromosome 22q11 microdeletion was found by the FISH method. The lab findings showed serum calcium level of 4.4 mg/dL, ionized calcium level of 0.49 mg/dL,... |
Purpose : We are inclined to analyze the relationship between the intrapulmonary right-to-left shunt and the PaO2/PaCO2 after endotracheal single-dose surfactant instillation to premature neonates with respiratory distress syndrome within 6 hours after birth. Methods : From Jan. 1993 to Jun. 1995, we have conducted a clinical trial of surfactant replacement therapy to the premature neonates with respiratory distress syndrome at the neonatal intensive care unit... |