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Case Report
Two Cases of Leigh Disease
Seong Hun Kim, Soo En Park, Ju Seok Lee, Sang Ook Nam, Yeong Tak Lim
Clin Exp Pediatr. 1998;41(3):405-409.   Published online March 15, 1998
Leigh Disease, or subacute necrotizing encephalopathy(SNE), is a degenerative disorder characterized by lesions of the gray and white matter in the bran and spinal cord. The pathogenesis was known as mitochondrial enzyme defect of the respiratory chain system. We experienced 2 cases of Leigh disease. The first case, a seven-month old girl who was presented with weak respiration and failure...
Original Article
Longitudinal Change of Cerebral Blood Flow Velocity in Neonates with Perinatal Asphyxia and Hypoxic-Ischemic Encephalopathy with Doppler Technique
Kook In Park, Dong Kwan Han, Joon Soo Lee, Ran Namgung, Chul Lee
Clin Exp Pediatr. 1994;37(9):1196-1204.   Published online September 15, 1994
Fifty-three full-term, preterm, term-SGA newborn infants with perinatal asphyxia and 16 full-term infants with hypoxic-ischemic encephalopathy were studied with color Doppler songraphy to assess the postnatal change of the cerebral blood flow velocity(CBFV) longitudinally. A control group of 81 healthy infants also had CBFV recordings during the 1st week of life. Pourcelot Resistance index(PI) and area under the velocity curve(AUVC)...
Occult Mediastinal Ganglioneuroblastoma Presenting with Myoclonic Encephalopathy as Paraneoplastic Syndrome
Hahng Lee, Dong Ki Han, Jae Won Oh, In Joon Seol, Eun Kyung Hong, Seok Chol Jeon
Clin Exp Pediatr. 1994;37(5):695-700.   Published online May 15, 1994
Ganglioneruroblastoma and neuroblastoma are among commonest types of childhood malignancy and a number of unique paraneoplastic syndromes have been associated with both localized and disseminated neuroblastoma. The coincidence of neuroblastoma and myoclonic encephalopathy or other paraneoplastic syndromes occurs relatively rare, and therefore, failure to recognize this association could result in delays in both diagnosis and treatment, and the result...
Case Report
A Case of Early Infantile Epileptic Encephalopathy
Sang Mi Ha, Hee Jung Jung
Clin Exp Pediatr. 1992;35(11):1590-1596.   Published online November 15, 1992
Early infantile epileptic encephalopathy is characterized by its early onset (less than 3 months of age), frequent tonic spasms, suppression-burst on EEG, extremely poor response to treatment and grave outcome. The authors experienced a case of early infantile epileptic encephalopathy. The patient was a 1-month-old girl with a complaint of generalized tonic convulsion since 2 days of age, and her EEG...
Original Article
Predictors of neurologic handicap in hypoxic ischemic encephalopathy.
Seung Tae Kim, Gui Ran Kim, Byung Hak Lim, Sang Geel Lee, Im Ju Kang
Clin Exp Pediatr. 1991;34(4):473-479.   Published online April 30, 1991
Retrospective follow up studies were done in 52 term infants who were treated at the nursery in Taegu Fatima Hospital, for clinical evidence of Hypoxic ischemic enecphalopathy (stage II). All children were followed for a minimum of 6 months. Factors that significantly correlated with prognosis included 5-minute Apgar scores, the age at the onset of the seizures, the EEG and the initial brain CT. (1) Of...
Changes in Auditory Brainstem Reponses (ABR) in Severe Hyperbilirubinemic Neonates with Transient Bilirubin Encephalopathy.
Chong Woo Bae, Sa Jun Chung, Chang Il Ahn
Clin Exp Pediatr. 1990;33(9):1216-1222.   Published online September 30, 1990
Many authors have reported alterations in the ABR in severe hyperbilirubinemia and ABR is an effective non-invasive method of assessing the functional status of the auditory nerve and brainstem sensory pathway in the management of neonatal hyperbilirubinemia. Perlman et al reported that jaundice in the early neonatal period is associated with significant transient aberration of ABR, suggestive of a transient bilirubin encephalopathy (TBE). And...
A Case of Early Infantile Epileptic Encephalopathy with Suppression-burst.
Sa Jun Chung, Chang Il Ahn
Clin Exp Pediatr. 1989;32(9):1320-1323.   Published online September 30, 1989
The authors experienced a case of 45-day-old female newborn infant who showed the intractable tonic spasms with series formation and hypotonia. We diagnosed it through the evaluation of the clinical, radiological features including brain CT and electroencephalogram. The suppresion-burst pattern in EEG and clinical features aid to the confirmation of the diagnosis. A brief review of the related literature were made.
Two Case of Transient Bilirubin Encephalopathy in Newborn.
Eun Kyoung Sohn, Chong Woo Bae, Sa Jun Chung, Chang Il Ahn
Clin Exp Pediatr. 1989;32(9):1295-1299.   Published online September 30, 1989
More recently, several studies have been repotted about the abnormalities of auditory brainstem response (ABR) in hyperbilirubinemia of newborn. By using of ABR, visual evoked potenital and nuclear magnetic resonance techniques, some investigators suggested that transient alteration in ABR in severely jaundiced neonates indicated reversible transient subclinical or clinical bilirubin induced neurotoxicity. They thought it would be the early stage of kernicterus. We experienced two...
Clinical Study of Encephalitis and Encephalopathy in Children.
Dong Hunb Lee, Soo Chun Kim, Sa Jun Chung, Chang Il Ahn
Clin Exp Pediatr. 1989;32(4):500-510.   Published online April 30, 1989
This study was undertaken to evaluate the electronencephalographic and clinical findings in 132 cases of encephalitis and encephalopathy in the Department of Pediatrics, Kyung Hee University Hospital for 9 years from Jan. 1979 to Dec. 1987. The results were summerized as follows; 1) The incidence of ecephalitis was 0.46%{86 cases) and the incidence of encephalopathy was 0.25 % (46 cases) among total in-patient (18,671). 2) Male...
Changes in Auditory Brainstem Resposes in Hyperbilirubinemic Neonates Before and after Exchange Transfussion and Phototherapy.
Chong Woo Bae, Young Ho Ra, Sa Jun Chung, Chang Il Ahn
Clin Exp Pediatr. 1988;31(5):547-554.   Published online May 31, 1988
More recently, several studies of newborns involving ABR testing have demonstrated abnormalities following an exposure to moderate or severe hyperbilirubinemia. Jaundice in the early neonatal period is associated with significant transient aberrations of ABR, which suggested transient brain- stem encephalopathy. So, many authors mentioned that ABR may be a useful tool for the monitoring of early biliubin toxicity in infants who were at risk...
Case Report
A case of Myoclonic Encephalopathy associated with Neuroblastoma.
Jae Seung Yang, Chang Jun Coe, Han Gu Mun, Chan Il Park
Clin Exp Pediatr. 1985;28(9):926-930.   Published online September 30, 1985
We experienced a case of myoclonic encephalopathy, associated with occult neuroblastoma. This 14 months old male child manifested opsoclonus, myoclonic seizure as well as delayed development. This patient also, had neuroblastoma, which has been removed. After the surgical removal of the tumor, the opsoclons was not disappeared.
Original Article
Reye's Syndrome in Children.
Dong Jin Lee, Sook Hee Lee, Ja Hoon Koo, Doo Hong Ahn, Yong Jin Kim
Clin Exp Pediatr. 1984;27(5):457-466.   Published online May 31, 1984
A clinical observation has been made on 44 children with Reys's syndrome who were admitted to pediatric department of KNU hospital during 6 year period from September 1977 to August 1983. Male to female ratio was 1.3 : 1. Cases under 6 years of age comprised 40(90.9%) and cases under 1 year 13(29.6%). The most common antecedent illness was URI...
Case Report
Pheochromocytoma in A Child, with Hypertensive Encephalopathy.
Sung Jee Nam, Oh Young Kwon, Ja Hoon Koo, Doo Hong Ahn, Ku Hong Cho, Dae Ki Han, Sae Kook Chang
Clin Exp Pediatr. 1982;25(1):72-79.   Published online January 31, 1982
A 12 years old boy was admitted with chief complaint of fever, vomiting, sweating and comatose state. Blood pressure ranged 200/150mmHg, and various tests, including 24 hour urinary VMA, regitine test, abdominal aortography and CT scan, showed pheochromocytoma on the left adrenal gland. After successful surgical removal of the tumor, he has been free of symptoms afterward. And diagnosis of...
A Case of Cushing's Syndrome Associatied with Hypertensive Encephalopathy.
Choong Rae Kim, In Soon Park, Do Seung Lee, Jae Sun Park, Kee Ryo Chang, Soon Yong Lee
Clin Exp Pediatr. 1981;24(1):80-84.   Published online January 15, 1981
A 8-yrs-old boy was admitted because of convulsion and coma. The diagnosis of Cushing's syndrome(key word) associated with hypertensive encephalopathy(key word) due to right adrenocortical carcinoma(key word) was made by clinical features, biochemical studies, radiological studies and pathological examination. The tumor was successfully resected by right adrenalectomy. But 22 months later after operation, he died of dyspnea and heart failure....
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