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

No abstract available in English.
Congenital Abnormalities

Inflammatory pseudotumor of the liver is a relatively rare entity, and frequently misdiagnosed as a malignant tumor. We report a case of inflammatory pseudotumor involving the liver in a 53year-old man. The liver function test and serum alpha-fetoprotein level were within normal range. His preoperative diagnosis was as hepatocellular carcinoma by radiologic studies, and ultrasonography guided fine needle aspiration cytology and biopsy were done but confirmative diagnosis of malignancy or pseudotumor was not given. Grossly a relatively well marginated reddish brown soft mass with focal hemorrhage, measuring 5.0 cm in the largest diameter, was noted in the left lobe of liver. Surrounding hepatic parenchyma was yellowish brown in color without cirrhosis. Microscopically the mass showed typical findings of inflammatory pseudotumor and the ing liver tissue revealed diffuse fatty change and moderate chronic inflammatory cell on in the portal areas.
Biopsy ; Carcinoma, Hepatocellular

No Abstract Available.
Fas Ligand Protein* ; Keratoconus*

Authors measured the degree of posterior instability of the knee in 21 patitients who had posterior cruciate ligament(PCL) injury or have been reconstructed the PCL by using the manual maximal displacement test and quadriceps active test with KT-1000 arthrometer. To compare the reliability between the manual maximal displacement test and the quadriceps active test, two testers participated in the experiment. One tester was a novice and the other tester had substantial clinical experiences with the KT-1000 arthrometer. The quadriceps neutra! Angle was measured in the uninjured knee, and then quadriceps active test was done. Manual maximal displacement test and Quadriceps active test were done by each tester. In manual maximal displacement test, the Pearson correlation coefficient of the uninjured knee between two testers was 0.852 and that of PCL injured knee between two testers was 0.802. In quadriceps achive test, the Pearson correlation coefficient between two testers was 0.173. As a result, the manual maximal displacement test was more reliable for th measurement of the degree of the posterior instability in patients with posterior cruciate ligament tear or reccnstruction.
Humans ; Knee* ; Posterior Cruciate Ligament

Two cases of traumatic bilateral anterior and posterior dislocation of hip are reported. Dislocations were developed by a traffic accident as a passenger injury in truck. In both cases, the fractued femoral heads appeared posteriorly dislocated on x-ray examination and one of them was removed through posterior approach. Primary arthroplasty for operated hip was not attempted because the patient was young and the defect seemed to be unrelated to weight bearing surface. Extreme rarity of its incidence and curious uncertain mechanism of injury urge to report these cases.
Accidents, Traffic ; Arthroplasty ; Dislocations ; Head ; Hip ; Humans ; Incidence ; Motor Vehicles ; Weight-Bearing

Osteopoikilosis is a familial, rare condition only discovered on radiological examination for a coincident disease. It is characterized by numerous nodules of dense bone in the spongiosa of the metaphysis or in the epiphysial nucleus. They are particularly seen in the small bones of the hands and feet and at the ends of the large bones of the extremities. Most of the bones have been reported as being affected but the skull is rarely involved. This condition is usually symptomless and no specific treatment is necessary. A case of osteopoikilosis is presented with a brief review of literatures.
Extremities ; Foot ; Hand ; Osteopoikilosis ; Skull

The combined congenital malformation of partiaI or complete absence of the pectoralis main muscle and webbing of the fingers, on the same side, was first described by Poland in 1841. The syndrome is not hereditary and is of unknown origin and is affects male more frequently than female. The clinical features are variable but always include congenital aplasia of the stemorostal head of the pectoralls major muscle and syndactyly. The syndectely should be treated by the age of one year and complened before the preschool age. A case of Poland's syndrome is presented with a brief review of literatures.
Female ; Fingers ; Head ; Humans ; Male ; Poland Syndrome ; Poland ; Syndactyly

The authors have reviewed 16 cases of the fracture of the femoral neck in children which were treated at Pusan Gospel Hospital during the period of 5 years from March 1975 to January 1980. 12 of the 16 fractures of femoral neck had sustained the injury by automobile accidents. The ratio between boy and girl was 1.6:1. There were five Type-II (Transcervical), nine Type-III (Cervicotrochanteric) and two Type-IV (In-tertrochanteric) femoral fractures by Delbets classiflcation. 2. cases of undisplaced and displaced Type-IV were treated with abduction plaster spics cast. 14 cases, which were displced and had associated injury, were reduced by closed reduction and internal fixation with hagie pin, cortical screw and cancellous screw under general anesthesia. 11 of 16 cases were followed for 7 months to 29 months, average 14 months. The results were rated according to criterias of the Ratliff. Satisfactory results were obtained in 9 cases and those of 5 cases were treated by closed reduction and internal fixation wlth cancellous screw. Pain was developed in a Type-II and Type-II due to loosening of metal devices for internal fixation.
Anesthesia, General ; Automobiles ; Busan ; Child ; Female ; Femoral Fractures ; Femur Neck ; Humans ; Male ; Spine

The compartment syndrome was described by von Volkmann in 1872 and numerous reports have since been published. The anterior tibial syndrome is well known, but the peroneal compartment syndrome is very rare and have some differences in it's etiology, diagnosis and treatment. We experienced a case of the peroneal compartment syndrome developed after playing foot-ball, and treated by fasciotomy with some delay, but obtained a satisfactory functional result.
Anterior Compartment Syndrome ; Compartment Syndromes ; Diagnosis