Ossification of the Triradiate Cartilage and Posterior Acetabulum

PURPOSE@#This study attempts to evaluate the skeletal maturation patterns of the triradiate cartilage (TRC) and the posterior acetabular wall (PA), which can be easily assessed on body computerized tomography (CT). It also examines the effect of gender and age on ossification of both TRC and PA.@*MATERIALS AND METHODS@#This retrospective study included a total of 1324 CT scans for children between the ages of 6 and 16 years. Depending on the extent of ossification in each right or left aspect, determined by the consensus of two observers, the TRC and PA scans were categorized into Grades 4 and 3, respectively.@*RESULTS@#The TRC for boys began to ossify at age 10 and closed completely at 14, while the PA for boys started ossification at age 11 and entirely fused at 13. The ages of ossification center appearance and complete fusion in both TRC and PA for girls were exactly two years earlier than boys. The TRC fused within one year after the closure of the PA.@*CONCLUSION@#The appearance and closure of the TRC and PA ossification centers show predictable patterns of development, appearance and merger earlier in females than in males. The suggestion is that development and morphogenesis of both TRC and PA ossification centers can be adequately assessed by using 3-dimensional body CT.

Giant hypothalamic hamartoma associated with an intracranial cyst in a newborn

We report the case of a giant hypothalamic hamartoma with a large intracranial cyst in a neonate. On ultrasonography, the lesion presented as a lobulated, mass-like lesion with similar echogenicity to the adjacent brain parenchyma, located anterior to the underdeveloped and compressed left temporal lobe, and presenting as an intracranial cyst in the left cerebral convexity without definite internal echogenicity or septa. The presence of a hypothalamic hamartoma and intracranial neurenteric cyst were confirmed by surgical biopsy. The association of a giant hypothalamic hamartoma and a neurenteric cyst is rare. Due to the rarity of this association, the large size of the intracranial cyst, and the resulting distortion in the regional anatomy, the diagnosis of the solid mass was not made correctly on prenatal high-resolution ultrasonography.

Characteristics of Pediatric Pancreatitis on Magnetic Resonance Cholangiopancreatography / 대한소아소화기영양학회지

Pediatric pancreatitis is not uncommon and results in considerable morbidity and mortality in the affected children. Unlike adults, pediatric pancreatitis is more frequently associated with underlying structural abnormalities, trauma, and drugs rather than an idiopathic etiology. Magnetic resonance cholangiopancreatography (MRCP) is a good imaging modality for evaluating pancreatitis and determining etiology without exposure to radiation. This article focuses on MRCP findings associated with various causes of pancreatitis in children, particularly structural abnormalities of the pancreaticobiliary system, as well as describing the feasibility, limitations, and solutions associated with pediatric MRCP.

Characteristics of Pediatric Pancreatitis on Magnetic Resonance Cholangiopancreatography / 대한소아소화기영양학회지

Pediatric pancreatitis is not uncommon and results in considerable morbidity and mortality in the affected children. Unlike adults, pediatric pancreatitis is more frequently associated with underlying structural abnormalities, trauma, and drugs rather than an idiopathic etiology. Magnetic resonance cholangiopancreatography (MRCP) is a good imaging modality for evaluating pancreatitis and determining etiology without exposure to radiation. This article focuses on MRCP findings associated with various causes of pancreatitis in children, particularly structural abnormalities of the pancreaticobiliary system, as well as describing the feasibility, limitations, and solutions associated with pediatric MRCP.

Correction: Characteristics of Pediatric Pancreatitis on Magnetic Resonance Cholangiopancreatography / 대한소아소화기영양학회지

The errors were discovered after publication: missing references and missing words.

Transperineal ultrasonography for evaluation of the perianal fistula and abscess in pediatric Crohn disease: preliminary study

PURPOSE: To assess the feasibility and effectiveness of transperineal ultrasonography (TPUS) for the evaluation of perianal Crohn disease (PCD) in pediatric patients. METHODS: Between September 2010 and August 2013, 64 TPUS examinations were performed in 43 patients (34 males and 9 females; mean age+/-standard deviation, 13.3+/-2.4 years; age range, 6 to 17 years) to evaluate PCD. The pain severity, location, and activity of perianal fistula, the presence of an abscess, and anal canal hyperemia were retrospectively evaluated. Spearman rank correlation analysis was performed to assess the relationship between the severity of the pain and the fistula activity, the presence of an abscess, and anal canal hyperemia. RESULTS: All examinations were successfully performed. Thirty-nine examinations (60.9%) were performed without any pain experienced by the patient, 19 examinations (29.7%) with mild pain, five examinations (7.8%) with moderate pain, and one examination (1.6%) with severe pain. The pain severity was correlated with the fistula activity (P<0.01). An anterior fistula location was more common than a posterior location. Active fistulas and abscesses were identified during 30 examinations (46.9%) and 12 examinations (18.8%), respectively. Anal canal hyperemia was identified in 31 examinations (48.4%). CONCLUSION: TPUS with a color Doppler study is useful for visualizing a perianal fistula or abscess and for assessing its inflammatory activity in pediatric Crohn patients.

Radiological Spectrum of Hepatic Mesenchymal Hamartoma in Children

OBJECTIVE: A hepatic mesenchymal hamartoma is an uncommon benign tumor in children and little is known about the spectrum of its radiological features. The purpose of this study is to describe the spectrum of radiological features of a hepatic mesenchymal hamartoma in children. MATERIALS AND METHODS: Thirteen children with a pathologically confirmed hepatic mesenchymal hamartoma (M:F = 7:6; mean age, 3 years 2 months) were included in our study. Ultrasonography (US) was performed in nine patients including color and power Doppler US (n = 7). CT scans were performed in all patients. We evaluated the imaging findings of the hepatic mesenchymal hamartomas and the corresponding pathological features. RESULTS: Each patient had a single tumor (mean diameter: 13 cm [1.8-20 cm]). On CT and/or US, four patients (31%) had a "multiseptated cystic tumor", five patients (38%) had a " mixed solid and cystic tumor", and four patients (31%) had a "solid tumor." The septa of the cystic portion were thin in the multiseptated cystic tumors and irregularly thick in the mixed solid and cystic tumors as seen on US. On a post-contrast CT scan, solid portions or thick septa of the tumors showed heterogeneous enhancement. The amount of hepatocytes was significantly different among the three tumor groups according to the imaging spectrum (p = 0.042). CONCLUSION: A hepatic mesenchymal hamartoma in children can show a wide spectrum of radiological features, from a multiseptated cystic tumor to a mixed solid and cystic tumor, and even a solid tumor.

Delayed Bronchostenosis After Blunt Chest Trauma in Children: CT and Pathologic Findings

Tracheobronchial disruption is an uncommon injury associated with blunt chest trauma. We report CT features and pathologic findings of two pediatric cases in which a bronchial injury was unnoticed initially but was diagnosed later by appearance of delayed bronchostenosis with distal atelectasis after blunt chest trauma in recent motor vehicle accidents. Pathologically, obliteration of the bronchial lumen was caused by dense fibrous overgrowth and granulation tissue.

Expression of MUC Gene Proteins in Cholecystitis, Adenoma and Adenocarcinoma of the Gallbladder

PURPOSE: Normal gallbladder mucosa shows unique and diverse patterns of mucin genes, and altered mucin expressions have been noted in the gallbladders with stones, dysplasia or carcinomas. The aim of this study was to characterize the expressions of MUC gene proteins in cholecystitis, adenoma and adenocarcinoma of the gallbladder. Differences of MUCs expressions according to the histopathologic parameters in gallbladder carcinomas were also studied. METHODS: Three tissue microarray blocks were made from 23 cases of cholecystitis, 40 cases of gallbladder adenoma, and 66 cases of gallbladder adenocarcinoma. Immunohistochemical stains for MUC1, MUC2, MUC4, MUC5AC and MUC6 were performed, and staining intensity and patterns were evaluated. RESULTS: MUC1, MUC2, MUC4, MUC5AC and MUC6 were overexpressed in 86 (66.7%), 20 (15.5%), 68 (52.7%), 74 (57.4%) and 47 (36.4%) of gallbladder lesions, respectively. MUC1 and MUC2 overexpression rates were higher in gallbladder carcinoma than in cholecystitis and gallbladder adenoma (P<0.05). MUC6 overexpression rate was higher in gallbladder adenoma than in cholecystitis and gallbladder carcinoma (P<0.05). Overexpression of MUC4 and MUC5AC showed no significant differences in cholecystitis, gallbladder adenoma and gallbladder carcinoma. In gallbladder carcinomas MUC1 overexpression rate was high in cases with deeper tumor invasion (P<0.05). MUC6 overexpression rate decreased in cases with larger tumor (P<0.05), higher histologic grade (P=NS), and deeper invasion (P=NS). MUC2, MUC4 and MUC5AC overexpression rates had no relations to the histopathologic parameters. CONCLUSION: Carcinomatous change of gallbladder may be related to MUC1 and MUC2 overexpressions. MUC1 overexpression seems to be related to aggressive biologic behavior of gallbladder carcinoma. MUC6 overexpression acts as a good prognostic factor.

Characteristic MR Findings of Growing Skull Fracture in Children

PURPOSE: Leptomeningeal cyst or growing skull fracture can occur in young infants or children following head trauma. We present MR imaging findings in five children with growing skull fracture. MATERIALS AND METHODS: We reviewed the MR images of five children (M: F=2:3) with growing skull fracture. The mean age was 7.5 years. The time interval between the occurrence of head trauma and the presentation of growing skull fracture varied from three months to 12 years. We reviewed the precontrast CT scans and/or the plain skull radiographs in those patients for whom these studies were available. RESULTS: The most common location of the growing skull fracture was the parietal bone (n=3). On the MR images, there were bone defects with posttraumatic cystic encephalomalacia or porencephalic cysts. Marginal bony thickening and diploic space widening were noted in four patients. MR imaging was excellent for visualizing the parenchymal changes and pericranial lesions. CONCLUSION: In children with growing skull fracture, MR imaging can clearly depict trauma-related parenchymal changes, pericerebral lesions as well as bony edge thickening with remodeling.

Acute Necrotizing Encephalopathy in Korean Infants and Children: Imaging Findings and Diverse Clinical Outcome

OBJECTIVE: The purpose of our study was to describe acute necrotizing encephalopathy in Korean infants and children, and we sought to evaluate the prognostic factors. MATERIALS AND METHODS: Acute necrotizing encephalopathy was diagnosed in 14 Korean infants and children. We retrospectively analyzed the neuroimaging findings including the follow-up changes. The clinical course of the disease was graded, and we evaluated prognostic factors including age, serum level of the aminotransferase, hemorrhage, and localized atrophy of the brain. RESULTS: This encephalopathy predominantly affected the bilateral thalami (n=14), pons (n=12), and midbrain (n=10) in a symmetrical pattern. Hemorrhage was observed in eight patients (57%). On the follow-up images (n=12), the brain lesions were reduced in extent for all patients, and generalized atrophy was seen in six patients. Localized tissue loss was observed in five patients and a complete resolution occurred for one patient. All the patients survived and two recovered completely; mild (n=6) to severe (n=6) neurological deficits persisted in the remaining 12 patients. The significant prognostic factors identified in this study were the presence of hemorrhage (p = 0.009) and localized atrophy (p = 0.015). CONCLUSION: Acute necrotizing encephalopathy in Korean patients showed the characteristic patterns of the post-infectious encephalopathy as described in the literature. The high survival rate and the relatively favorable clinical course observed for the present study suggest a more diverse spectrum of disease severity than was previously described. The presence of hemorrhage and localized tissue loss on MR images may suggest a poor prognosis.

The Comparison of Severity according to Preceding Causes of Bronchopulmonary Dysplasia in Very Low Birth Weight Infants

PURPOSE: This report attempts to reveal the incidence and prevalence of bronchopulmonary dysplasia (BPD) and compare the severity according to preceding causes of BPD in very low birth weight (VLBW) infants. METHOD: Retrospective study was done on 293 VLBW infants who were born and admitted to neonatal intensive care unit in Samsung medical center between October, 1995 and December, 2001. Classical BPD was defined as oxygen dependency at 36 week's postmenstrual age (PMA). Ogawa BPD was defined as oxygen dependency at 28 days after birth, with respiratory distress symptoms and the change on chest X-ray finding. This classification further classified as BPD into 5 subtypes by the presence of respiratory distress syndrome (RDS), pathologic chorioamnionitis and the type of chest X-ray finding. BPD by Jobe and Bancalari was defined as oxygen dependency at 28 days after birth and classified as 3 subtypes (severe, moderate, mild) by the severity of oxygen dependency. Comparisons were made among classifications. RESULTS: Classical BPD infants were 56 (19.1%), Ogawa BPD infants were 76 (25.9 %), BPD by Jobe and Bancalari infants were 124 (42.3%). In Ogawa classification, Infants with RDS and the change on chest X-ray were 58 infants (76.4%). There was no statistical difference of mortality between each type of Ogawa BPD. In classification by Jobe and Bancalari, 35 infants (28.2%) belonged to severe BPD and 75 infants (60.5%) belonged to mild BPD. The mortality was highest in severe BPD infants but there was no statistical difference after correction by birth weight. There was no statistical correlation between Ogawa classification and classification by Jobe and Bancalari. CONCLUSION: There was no statistical difference in mortality or severity between each subtype of classifications according to the severity or preceding cause of BPD in very low birth weight infants.

Mucopolysaccharidosis: Abnormal Findings on Abdomen and Chest Excluding Musculoskeletal System

PURPOSE: Mucopolysaccharidosis (MPS) is a lysosomal storage disease that causes tissue distortion and dysfunction due to the infiltration of mucopolysaccharide in connective tissue. The purpose of this study was to evaluate the characteristic findings of abdominal CT and plain chest radiography in patients with MPS. MATERIALS AND METHODS: Sixty-two children with MPS diagnosed by urine analysis were involved in this study; 24 of these underwent abdominal CT and the findings were reviewed by two radiologists, who reached a consensus. Organomegaly was classified as severe, moderate or mild. On chest PA radiographs of 42 of the children, the transverse diameter of the trachea was measured and compared with that of 42 normal controls. Student's t test was used for statistical analysis. RESUTLS: At abdominal CT, hepatomegaly was observed in 22 patients (92%; 2 severe, 15 moderate and 5 mild); and splenomegaly was present in 18 (75%; 2 severe, 4 moderate and 12 mild). Among eight patients (33%) with pancreatic enlargement, one had a severly enlarged pancreas, while in the remaining seven, enlargement was mild. Also present were inguinal hernia (n=15), umbilical hernia (n=12), undulation with thickening of the diaphragmatic crura (n=10), abnormalities related to the male genitalia (n=5) and vascular anomaly (n=3). In MPS patients, the mid-point diameter of the trachea (range, 5.6-9 mm; mean, 6.9 mm) was significantly less than in normal controls (range, 8-14 mm; mean, 10.8 mm) (p<0.001). CONCLUSION: An awareness of the characteristic abnormalities observed at abdominal CT and chest PA radiography can lead to a better understanding of MPS in children.

Clinical Manifestations and Surgical Outcome of Medically Refractory Epilepsy in Childhood

PURPOSE: The cognitive and psychosocial morbidity of medically refractory epilepsy is considerable. Epilepsy surgery can play a important role in these patients. We investigated the clinical manifestations and the surgical outcome of our patients with medically refractory epilepsy. METHODS: We studied 27 patients under 15 years old who had epilepsy surgery at Samsung Medical Center between March 1995 and December 2001 retrospectively. RESULTS: The median age at first unprovoked seizure was 6 years, the median age at surgery was 11.02 years and duration of follow-up was 14 months to 7 years 2 months. Complex partial seizure was the most common type of seizures. Interictal and ictal discharges were lateralized in 66.7% and 81.5%, respectively. MRI showed abnormal findings in 23 patients and the cortical dysplasia was the most common pathologic finding. As per surgical outcomes, 77.8% of the patients became seizure free. Among patients with temporal lobe epilepsy(TLE), seizure free rate was 92.3% whereas patients with extratemporal lobe epilepsy(ETLE) showed 64.3% seizure free rate. All patients with TLE with focal lesions became seizure free. Patients with focal lesions which were concordant with findings of EEG, SPECT, and PET showed excellent surgical outcomes. However, patients with no focal lesion in MRI and discordant preoperative examinations showed poor outcomes. CONCLUSION: The results of epilepsy surgery in our patients were quite satisfactory. Good surgical outcome can be expected when the decision of surgery is made based on a good correlation among clinical, neuroradiological, and other preoperative examinations.

Clinical Relevance of Clinicopathologic Parameters and Tumor Markers in Ductal Carcinoma in Site of the Breast

PURPOSE: Ductal carcinoma in situ (DCIS) of the breasts is a heterogeneous group of lesions with diverse malignant potentials and controversial treatment options. This study was planned to investigate the patterns of clinicopathologic parameters and tumor markers related to biological aggressiveness and to make treatment decisions available based on a variety of these parameters. METHODS: We reviewed forty cases of DCIS treated at Pusan Paik Hospital from March 1992 to July 2002. Clinicopathologic features such as age, chief complaint, mammographic finding, tumor size, histologic subtype, and operation type were analysed, and the expression of ER, PR, p53, C- erbB-2, cathepsin D, bcl-2, MIB-1 and CD34 were evaluated using immunohistochemical methods. RESULTS: The size of the tumor was less than 1.5 cm in 16 (44.4%) cases, 1.5 cm to 4 cm in 17 (47.2%) cases, and more than 4 cm in 3 (8.3%) cases. There were 11 (27.5%) cases of the comedo subtype and 29 (72.5%) cases of the noncomedo subtype. Nuclear grade was divided into low (8 cases, 20.0%), intermediate (20 cases, 50.0%), and high (12 cases, 30.0%). According to Van Nuys' classification, there were 25 (62.5%) cases, 4 (10.0%) cases, and 11 (27.5%) cases of group I, II, and III, respectively. The groups presenting as mass on mammogram had no significant relationship with those presenting as microcalcification in terms of tumor size, histologic subtype, nuclear grade, and Van Nuys classification. The expression rates of PR, p53, C-erbB-2, cathepsin D, and bcl-2 were 32.4%, 67.6%, 35.1%, 29.7%, 67.6%, and 45.9%, respectively. High MIB-1 labelling index (LI) and high microvessel density were observed in 8.1% and 32.4%, respectively. The group presenting as mass on mammogram showed higher ER (P=0.0276) and PR (P=0.102) expression, compared with the microcalcification group. Positive ER and PR were associated with low nuclear grade (P=0.0233, 0.1727), while positive p53 and C-erbB-2 and high MIB-1 LI correlated with Van Nuys' group III (P=0.0637, 0.0532). Positive ER correlated with positive PR (P=0.0581) and negative C-erbB-2 (P=0.0642). In addition, there were positive associations between PR and bcl-2 expression (P=0.0939), between p53, C-erbB-2 (P<0.0001) and high MIB-1 labelling index (P= 0.0785), and between cathepsin D and high microvessel density (P= 0.0151). CONCLUSION: Clinico-pathologic evaluation of tumor size, histologic subtype, nuclear grade, and Van Nuys classification can help predict more aggressive immunophenotypes of DCIS. Positive p53 and C-erbB-2 and high MIB-1 is associated not only with more aggressive clinical behavior and more advanced histologic features of DCIS, but also with negative ER, PR, and bcl-2. Our results support the clinical relevance of combining both clinico-pathologic factors and biologic tumor markers for determining the treatment modality in DCIS patients.

The Role of Neoadjuvant Chemotherapy for Advanced Stage Wilms Tumor

PURPOSE: This study was designed to exclude radiation in advanced(stage 3, 4) Wilms tumor (WT) by increasing the chance of complete surgical removal with preceding neoadjuvant chemotherapy, thereby reducing the incidence of late effects. METHODS: Between December 1998 and July 2002, we conducted neoadjuvant chemotherapy after needle aspiration biopsy on patients who had advanced WT. If needle biopsy was accessible, we conducted neoadjuvant chemotherapy(vincristine, adriamycin, dactinomycin) for 12 weeks and then performed surgical removal, excluded radiation therapy and conducted postoperative chemotherapy (vincristine, dactinomycin+/-adriamycin). In other cases, we firstly conducted the operation and then performed radiation and postoperative chemotherapy. RESULTS: Of the 17 patients diagnosed as WT, 12 patients had an advanced stage of disease. In two of the 12 patients, initial surgical removal was conducted. The median age of patients was 21 months(5-103 months). Of the 10 the patients who received neoadjuvant chemotherapy, eight patients were stage 1, one patient was stage 2, and the other was stage 3 at operation. In nine patients except one with stage 3 disease, we could perform complete surgical resection and there fore could omit radiation. In four cases we could also exclude adriamycin after operation. All but one patient was alive, disease-free, for a median follow-up of 21 months(9-43 months). CONCLUSIONS: After neoadjuvant chemotherapy, we could increase the chance of complete tumor resection, exclude radiation and decrease the intensity of postoperative chemotherapy in selected cases. Long term follow-up is needed to determine whether our method would significantly decrease late effects.

Radiological Changes Following High Frequency Oscillatory Ventilation Therapy in Very Low Birth Weight Infants with Respiratory Distress Syndrome

PURPOSE: High frequency oscillatory ventilation (HFOV) is used to support infants with severe respiratory failure unresponsive to conventional ventilation (CV). We reviewed chest radiographs before and after HFOV with clinical correlation in infants with respiratory distress syndrome (RDS). METHODS: Eighteen very low birth weight infants with RDS who had HFOV were included in this study. All patients were diagnosed as having RDS clinically and radiologically. Mean gestational age of infants was 27 weeks (range : 24-31 weeks). The mean duration of HFOV was 3 days (range : 14 hours-9 days). The chest radiographs of these infants within 3 hours before and after application of HFOV were retrospectively reviewed. Radiological findings based on aeration and parenchymal densities were classified into improved, no change, and progressed. Medical records were reviewed for FiO2 levels, clinical outcomes, complications, and causes of death. RESULTS: In 15 of 18 infants, aeration and parenchymal densities were improved and FiO2 levels were also improved after HFOV. Four of these 15 infants who showed improvement of radiological findings developed pneumothorax, sepsis, pulmonary or intestinal bleeding, and subsequently died. In remaining 3 infants in whom chest radiographs after HFOV showed no interval change or progression, oxygenation was also worsened and all died. CONCLUSION: Chest radiographs of HFOV-treated, very low birth weight infants showed improvement of aeration and parenchymal densities in most cases. Clinical outcome was good in infants who showed improvement on chest radiographs compared to those of progression group as far as there was no associated complication. Knowledge of radiological changes after HFOV will help in interpretation of chest radiographs in those HFOV-treated infants.

Subtalar Coalition: Usefulness of the C Sign on Lateral Radiographs of the Ankle

PURPOSE: To assess the usefulness of the talocalcaneal C sign in the diagnosis of subtalar coalition, as seen on lateral radiographs of the ankle. MATERIALS AND METHODS: Lateral radiographs of 12 ankles in 11 patients were included in this study. Twelve subtalar coalitions were confirmed by surgery (n=6), or by CT and/or MR (n=6). The presence of the talocal-caneal C sign on lateral ankle radiographs was determined. RESULTS: The C sign was continuous in six feet and interrupted in the remaining six. Subtalar coalition occurred simultaneously in the middle and posterior subtalar joints in two cases, the posterior subtalar joint only in six, and in the middle subtalar joint only in four. In six cases confirmed at surgery, subtalar coalitions consisted of both synostosis and non-osseous fusion (synchondrosis and/or syndesmosis) and in one case of middle subtalar coalition, there was a bony bridge. The remaining six cases, confirmed at CT or MRI, involved both synostosis and non-osseous fusion (n=1) or non-osseous fusion only (n=5). CONCLUSION: In the diagnosis of subtalar coalition, the talocalcaneal C sign, seen on lateral radiographs of the ankle, is a useful indicator.

Surgically Treated Intractable Child Epilepsy with Focal Cortical Dysplasia : Clinical and Electroencephalographic Findings

PURPOSE: Widespread use of MRI now gives us increased insights into the different expressions of cortical malformations. We carried out this study to characterize the clinical and EEG features of focal cortical dysplasia(FCD) which also caused intractable epilepsy requiring surgical treatment. METHODS: A retrospective analysis was conducted in 8 children. The history of seizures, imaging studies, electroencephalographic findings, pathologic results, and surgical outcomes were reviewed. RESULTS: The onset ages of clinical seizure were from 4 days after birth to 36 months. The fre quency of seizures were from multiple times a day to a few times a month. Five out of 8 FCD patients had global developmental delays with more degrees of delay in language. Only 2 out of 8 patients showed clinical partial seizures only. The scalp EEG disclosed localized interictal epileptiform activity in all 8 patients and localized continuous slow waves in 7 out of 8 patients. Ictal scalp EEG onset demonstrated a predominantly localized EEG seizure activity in 6 patients. Ictal invasive EEG findings were variable in each patient and the patterns of ictal EEG were demonstrated. Five out of 8 patients became seizure-free. One patient had one episode of seizure during the 16 months follow up period. Two patients did not have significant relief from seizures. CONCLUSION: The patients with FCD had varied spectrums of clinical manifestation, MRI and EEG findings. Five out of 8 FCD patients had complete relief from seizures, one patient has had one short, simple, partial seizure since surgery, but 2 patients with FCD in the frontal area did not experience significant relief from seizures.

The Clinical Features of Acute Disseminated Encephalomyelitis in Children

PURPOSE: Acute disseminated encephalomyelitis(ADEM) is an immunologically mediated demyelinating disease of the central nervous system. We investigated the clinical features and outcomes of children with ADEM presenting with different neurologic symptoms and clinical courses. METHODS: Fifteen cases(male 10 and female 5) of ADEM who were diagnosed in the department of pediatrics, Samsung Medical Center from July 1997 to April 2001 were reviewed. RESULTS: The ages at initial presentations were from 1 year 5 months to 14 year 8 months. The presence of preceding events was present in all patients. The most common presenting symptom was seizure(60%), followed by altered consciousness(40%), ataxia(33 %), cranial nerve palsy, headache, quadriparesis, visual disturbance, and tremor. Brain magnetic resonance imaging showed relatively symmetrical, multifocal hyperintense lesions on T2-weighted image. One out of 4 brain CT showed an abnormal finding and remaining 3 cases showed abnormal findings in MRI only. Two patients had normal MRI findings at the initial phase of the illness but later revealed MRI findings compatible with ADEM. Thirteen patients were managed on high dose methylprednisolone therapy and/or intravenous immunoglobulin. After treatments, all patients revealed rapid clinical recovery. Relapse occurred in four patients(26%) 3 weeks to 1 year 6 months after developing their first illness. Among them, two patients were diagnosed as multiple sclerosis later. CONCLUSION: We experienced 15 children with the initial diagnosis of ADEM. Even though immunosuppressive drugs were effective to eliminate their presenting symptoms in most patients, there was 26% of relapse rate with 2 patients with later diagnosis of multiple sclerosis during long-term follow-up suggesting heterogeneity existed with regard to etiology and clinical courses of ADEM.