To know the efficacy of intravenous gammaglobulin(IVGG) treatment and the resulting outcome at 18 months for acute Guillain-Barr syndrome (GBS) in children, clinical courses of 27 GBS patients who had been treated with IVGG in a dose of 1g/kg/day oyer 2 consecutive days were investigated. One died with respiratory failure and 8 patients received assisted ventilatory care with improvement. All consecutively treated patients responded well to IVGG. The mean plateau period in 26 recovered patients was 8.5 days. At the 6th month evaluation, only one failed to reach grade 2 on GBSSG scales. At the 18th month evaluation, minor motor symptoms such as easy fatigability or mild tremor on writing were remained in 9 patients. 9 patients showed mild to moderate febrile response to IVGG infusion, but no significant side effects were noted. IVGG can be chosen as an initial treatment for acute GBS in children because of its rapid effect, relative safety even in unstable patients and convenience of administration.
Child* ; Humans ; Respiratory Insufficiency ; Tremor ; Weights and Measures ; Writing

PURPOSE: Cerebral cortical dysplasias are one of the important causes for epileptic seizures and developmental disabilities in children, particularly in diffuse or bilateral cases. These developmental malformations are generally regarded as a group of neuronal migration disorders, however, the classification system and pathogenetic mechanisms of cortical dysplasias are not yet entirely clear. Even a novel entity, congenital bilateral perisylvian syndrome, characterized by speech delay, pseudobulbar Palsy, intractable seizures, and bilateral perisylvian abnormalities on imaging studies, have rather diverse figures on morphology and symptomatology than initially considered. We have studied the clinical features and correlations of clinical outcomes 3nd magnetic resonance imaging(MRI) findings of bilaterally involved cortical dysplasias. METHODS: 20 cases of bilateral cortical dyspalsias were grouped into three categories on the basis of MRI findings; centroparietal dysplasias(6 cases), diffuse dysplasias with (5) or without white matter lesions(4), and schizencephaly(5). EEGs, motor and language development, epilepsy, and outcomes were reviewed in each groups. RESULTS: Language delay(100%), motor developmental delay(94.7%), motor deficit(65%), epilepsy(40%) were the main reasons for their initial hospital visits. A meaningful word expression was possible at the mean age of 2 years and 2 months(1 to 4 years of age). Hypotonia and spastic motor paralysis were evident in all the cases of diffuse dysplasia with white matter lesions and the schizencephaly groups. All but one case of centroparietal dysplasia showed motor developmental delay. Epilepsies were developed in 8 cases at the mean age of 5 years and 5 months(2 months to 12 years of age) and the seizures were relatively well controlled with anticonvulsants. EEG findings were variable; normal, focal or diffuse abnormalities. High amplitude diffuse fast activities were only noted in the diffuse dysplasia group. CONCLUSION: The severity of neurological deficits and developmental delay had correlated to the size of cortical abnormalities. The epilepsies were relatively well controlled during childhood and the EEG finding of high amplitude diffuse fast activities was specific for the diffuse cortical dysplasias. Bilateral centroparietal dysplasias should be included on consideration of the causes for developmental aphasia.
Anticonvulsants ; Aphasia ; Child ; Classification ; Developmental Disabilities ; Electroencephalography ; Epilepsy ; Humans ; Language Development ; Language Development Disorders ; Magnetic Resonance Imaging* ; Malformations of Cortical Development* ; Muscle Hypotonia ; Muscle Spasticity ; Neuronal Migration Disorders ; Paralysis ; Pseudobulbar Palsy ; Seizures

Hypertensive encephalopathy is characterized by an acute, severe rise in blood pressure associated with headache, nausea, vomiting, altered mental status, and focal neurologic deficits, and rapid improvement after control of blood pressure. An eight-year old boy had been admitted with symptoms of red urine, fever, headache, convulsion, and visual blurring of vision. Blood pressure was 140/90mmHg, and CSF exam revealed WBC 8/mm3, glucose 83mg/dL, protein 106mg/dL. Serum C3 was 8mg/dL, C4 17mg/dL, ASO 1,024 Todd units. Brain CT showed non specific findings, however, MRI revealed symmetric high signal intensity lesions on T2WI over the parieto-occipital areas bilaterally. These lesions had been completely resolved on the following MRI 4 weeks later.
Blood Pressure ; Brain* ; Fever ; Glomerulonephritis* ; Glucose ; Headache ; Humans ; Hypertensive Encephalopathy* ; Magnetic Resonance Imaging* ; Male ; Nausea ; Neurologic Manifestations ; Seizures ; Vomiting

No abstract available.
Lipodystrophy*

Moyamoya is a chronic cerebrovascular disease characterized by progressive stenosis or occlusion of the terminal parts of both intermal carotid arteries with telangiectatic vascular network of collateral circulation at the base of the brain and leptomeningeal arteries. The etiology and pathophysiology of this disease are still unknown. Although the idiopathic presentattion is the commonest, moyamoya disease has also been reported in several hereditary or acquired clinical conditions including neurofibromatosis, sickle cell anemia, tuberculous meningitis, atherosclerosis, and following radiation therapy to the head. The term moyamoya disease should be reserved for those cases in which the characteristic angiogrphic pattern is idiopathic; moyamoya syndrome is used when the underlying condition is known. We have experienced a case of coexistence of moyamoya syndrome and hereditary spherocytosis in a 6-year-8-month-old girl who presented with right-sided hemiparesis and pallor. A cerebral angiogram revealed occlusion of proximal portion of left middle cerebral artery and abnormal collateral network. The peripheral blood smear and osmotic fragility test disclosed hereditary spherocytosis. To our knowledge, the coexistence of moyamoya syndrome and hereditary spherocytosis has not been documented. We report here the case and the brief review of related literatures. Further studies are needed to clarify the intimate relationship between the two diseases.
Anemia, Sickle Cell ; Arteries ; Atherosclerosis ; Brain ; Carotid Arteries ; Collateral Circulation ; Constriction, Pathologic ; Female ; Head ; Humans ; Middle Cerebral Artery ; Moyamoya Disease* ; Neurofibromatoses ; Osmotic Fragility ; Pallor ; Paresis ; Tuberculosis, Meningeal

Intravenous immunoglobulin (IVIG) is widely used to treat various neurologic or autoimmune disorders in childhood. Aseptic meningitis has been reported as a rare complication of IVIG therapy. We describe two girls, aged 2 and 8 years, with myasthenia gravis who suffered from aseptic meningitis after an administration of high-dose IVIG. Any other etiologic agent were identified in neither case. Both patients recovered completely within a few days and left no sequelae. To our knowledge, these are the first such reported cases in children with myasthenia gravis.
Child ; Female ; Humans ; Immunization, Passive* ; Immunoglobulins* ; Immunoglobulins, Intravenous ; Meningitis ; Meningitis, Aseptic* ; Myasthenia Gravis*

Congenital obstuction of pulmonary vein without anomalous drainage can cause long-standing pulmonary congestion and pulmonary arterial hypertension, and it may include stenosis of individual pulmonary veinsor total pulmonary vein atresia. We reviewed seven cases of pulmonary vein obstruction, five of which accompanied other cardiac anomalies. Right pulmonary veins were involved in all seven cases including one bilateral case. Pulmonary veins were occluded totally in five and partially in three lungs. Pumonary catheterization and angiography were done for diagnosis. Chest radiographs of total occlusion cases showed decreased lung volume, features of pulmonary edema, interstitial lesions, and pleural thickening, which were quite specific, whereas pulmonry venous dilatation was dominant findings in partial obstruction cases. Pulmonary perfusion scans (n=3) showed total perfusion defects in the cases of total occlusion of veins. MR imaging (n=2) demonstrated total occlusion of pulmonary veins in the venoatrial junction in two, and membranous focal obstruction in one lung. Two patients had pneumonectomy and histological confirmation, Although catheterization and angiography are essential for the diagnosis, MR imaging is thought to be useful for the diagnosis of pulmonary vein obstruction.
Angiography ; Catheterization ; Catheters ; Constriction, Pathologic ; Diagnosis ; Dilatation ; Drainage ; Estrogens, Conjugated (USP) ; Humans ; Hypertension ; Lung ; Magnetic Resonance Imaging ; Perfusion ; Pneumonectomy ; Pulmonary Edema ; Pulmonary Veins* ; Radiography, Thoracic ; Veins

The immature teratoma is extremely rare tumor consisting of mature tissues and immature embryonal tissues. The immature teratoma in children occurs more frequently at the sacrococcygeal bone, testis and ovary. Only 9.5 percent of immature teratomas occur in retroperitoneum. Often the presenting manifestation is asymptomatic with increasing abdominal distention or mass discovered during physical examination. We report a case of congenital retroperitoneal immature teratoma in 3-week-old boy who was treated by surgical excision.
Child ; Female ; Humans ; Male ; Ovary ; Physical Examination ; Teratoma* ; Testis

Disseminated Fusarium infection has rarely been encountered in neutropenic cancer patients. The clinical features include fever, positive blood cultures, severe myalgias, disseminated ecthyma gangrenosum-like skin lesions, ocular symptoms and multi-organ-system involvement. The mortality rate reported so far exceeded 70% despite antifungal treatments. We report here the first two cases of disseminated fusarial infections in Korea. The first patient contracted pneumonia during severe neutropenic period following consolidation chemotherapy for acute myelogenous leukemia(AML). Fusarium oxyshrum was cultured from bronchoalveolar lavage fluid. The patient remains free of fungemia after successful antifungal treatment of high-dose amphotericin B and terbinafine. The second patient had relapsed AML and suffered from disseminated fusarial infection involving lungs with characteristic skin lesions. The patient succumbed to refractory leukemia and the fungal infection. A brief review of the literature ensues with the case report.
Amphotericin B ; Bronchoalveolar Lavage Fluid ; Child* ; Consolidation Chemotherapy ; Ecthyma ; Fever ; Fungemia ; Fusariosis ; Fusarium ; Humans ; Korea ; Leukemia* ; Lung ; Mortality ; Myalgia ; Pneumonia ; Skin

BACKGROUND: Fanconi's anemia(FA) is an autosomal recessive disease characterized by aplastic anemia and congenital malformations. As up to 30% of patients have no physical stigmata, the modern diagnosis of FA rests on chromosomal breakage of patient's cells induced by chemical clastogens such as diepoxybutane(DEB) or mitomycin-C(MMC). METHODS: We reviewed the clinical manifestations, laboratory findings, diagnostic methods, treatment and outcome of 6 patients diagnosed to have a FA at the Chonnam University Hospital for the last 6 years. RESULTS: Six cases(16.2 %) were found to have FA among 37 aplastic children who were diagnosed during the same period. The mean age at diagnosis was 6.3 years which was the usual onset of hematologic findings. All patients had features of aplastic anemia, and had one or more anomalies, such as low birth weight, hyperpigmentation, cafeau-lait spots, mental retardation, developmental delay, peculiar face(broad nasal bases, epicanthal folds, micrognathia), polydactyly, microcephaly, short stature, and dislocation of hip. We found increased breaks in cultured cells with DEB and MMC in 5 cases tested. The median duration of follow-up was 30 months. Oxymetholone and prednisolone treatment was partially beneficial in three cases. Immunosuppressive treatment with ALG/ATG was not successful in two cases tried. Four cases are living now, without transfusion in three. Two patients were died of disseminated fungal infection and transplant-related problems, respectively. CONCLUSIONS: Fanconi's anemia should be sought carefully in any patients with aplastic anemia because the prognosis, treatment modality, and the approach to bone marrow transplantation are quite different when the hematologic disorder is inherited rather than acquired.
Anemia, Aplastic ; Bone Marrow Transplantation ; Cells, Cultured ; Child ; Christianity ; Chromosome Breakage ; Diagnosis ; Dislocations ; Fanconi Anemia* ; Follow-Up Studies ; Hip ; Humans ; Hyperpigmentation ; Infant, Low Birth Weight ; Infant, Newborn ; Intellectual Disability ; Jeollanam-do ; Microcephaly ; Mitomycin ; Mutagens ; Oxymetholone ; Polydactyly ; Prednisolone ; Prognosis