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

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

No abstract available.
Anesthesia, Local* ; Lipectomy* ; Male ; Penis*

PURPOSE: To correlate the CT findings of renal cell carcinomas with nuclear grading in histopathology. MATERIALS AND METHODS: Preoperative CT scans and pathologic nuclear grading of 60 surgically resected renal cell carcinomas in 55 patients were retrospectively and independently reviewed. RESULTS: As nuclear grade increased, renal cell carcinomas were more likely to be of higher stage(92% of nuclear grade III renal cell carcinomas was of stage Ill, all Grade IV tumors were of stage IIIb or higher) and greater size(84% of grade III tumors and 100% of grade IV tumors>5cm in size) at presentation, and appeared more heterogeneous(84% of grade III tumors and 88% of grade IV tumors showed moderate or severe heterogeneity) and less well marginated(84% of grade Ill tumors and 100% of grade IV tumors had irregular or imperceptible margins). CONCLUSION: Small well-marginated homogeneous renal cell carcinomas were either nuclear grade I or II, and were distinguishable from the more aggressive nuclear grade III or IV lesions, which generally displayed irregular margins and greater inhomogeneity.
Carcinoma, Renal Cell* ; Humans ; Retrospective Studies ; Tomography, X-Ray Computed

PURPOSE: To describe the characteristic CT findings of ameloblastomas in the mandibleand maxilla. MATERIALS AND METHODS: CT findings of 11 patients with ameloblastoma (9 cases in the mandible and 2 cases in the maxilla) proved by excisional biopsy were evaluated retrospectively with regard to the location, size, multilocularity, solid and cystic component, cortical destruction, soft tissue invasion, and contrast enhancement RESULTS: These were 8 multilculer expansile and I unicystic (developed in a dentigerous cyst) mandibler ameloblasftomas, and 2 unilocular maxillary ameloblasftomas. All cases showed inhomogenously enhancing solid component, nine of which were larger than cystic component. Nine cases, larger than 5 cm in diameter, revealed either a focal or extensive cortical destruction with various degree of invasion into the adjacent structures. One maxillary ameloblastoma contained a thick calcification along the margin of the lesion. CONCLUSION: Maxillomandibular ameloblastomas appeared as expansile lesion containing enhancing solid component relatively larger than cystic portion and having cortical destruction in large lesions(5cm>). Maxillary ameloblastomas were unilocular in appearance in spite of multilocularity in mandibular counterparts.
Ameloblastoma* ; Biopsy ; Humans ; Mandible* ; Maxilla* ; Retrospective Studies

No abstract available.
Jaw*

No abstract available.
Bone Density* ; Child* ; Glucocorticoids* ; Humans ; Nephrotic Syndrome*

Duplication of the urethra within a single penile shaft is a rare anomaly. These can be divided into those that are in the sagittal plane, which is most common, and those that occur side by side. In some cases, the accessory channel is complete, having a separate bladder opening and no communication with the more normal ventral urethra. In other cases, the accessory urethra is incomplete, either ending blindly or communicating with the urethra distal to the bladder neck. We report on a 20-year-old male with incomplete double urethra.
Humans ; Male ; Neck ; Urethra* ; Urinary Bladder ; Young Adult

No abstract available.

PURPOSE: lntra-arterial cisplatin infusion chemotherapy delivers higher concentration of this therapeutic agent to the limited area of cervical carcinoma. We evaluated the role of endorectal ultrasonography (ERUS) in the analysis of the tumor response to this chemotherapy. MATERIALS AND METHODS: ERUS was done in 20 patients of cervical carcinomas limited to the pelvis by rotating the 5 UHz linear arraytransducer along the reference sagittal plane in the middle of uterine cervix and vagina. We examined and compared the change of tumor size and morphology on ER US, before, during and after intra-arterial cisplatin (50 mg/m2) chemotherapy through internal lilac artery near uterine arterial branching. Colposcopy and radical hysterectomy with pelvic lymphadenectomy were performed in all patients, and ERUS findingswere compared with those findings. RESULTS: ERUS correlated well with surgery in the estimation of preoperative tumor size and morphology. Tumor reduction rate on ERUS (100-the largest area of the preoperative tumor/the largest area of the initial tumor) was as follows: more than 75% decrease of the original size in 3 patients, 75% -50% in 6 patients, 50%-25% in 8 patients, and less than 25% in 3 patients. Hyperechoic reflecting areas were noted in 4 patients, which corresponded to gaseous necrosis on postoperative pathology. CONCLUSION: ERUS is helpful to evaluate the morphologic change of cervical carcinoma limited tothe pelvis in intra-arterial cisplatin chemotherapy.
Arteries ; Cervix Uteri ; Cisplatin* ; Colposcopy ; Drug Therapy* ; Female ; Follow-Up Studies* ; Humans ; Hysterectomy ; Lymph Node Excision ; Necrosis ; Pathology ; Pelvis ; Ultrasonography* ; Vagina