To evaluate the risk and factors associated with seizure recurrence in children with epilepsy while receiving the adequate anticonvulsant treament, we studied 58 patients with newly diagnosed epilepsy who were followed prospectively for a median of 26 months (range 7 to 54). The results were as follows: 1) Forty-four of the 58 patients (75.9%) had recurrence of seizure. 2) The rate of recurrence according to type of seizure was observed to be 22 patients (68.8%) in generalized tonic-clonic seizure, 6 patients (85.7%) in simple partial seizure, 5 patients (83.3%) in complex partial seizure, 3 patients (100%) in mixed seizure, 2 patients (100%) in absence, 3 patients (100%) in infantile spasm, 1 patient (100%) in atonic seizure, 2 patients (50%) in secondary generalized seizure. There was no significant difference in the risk of recurrence observed among these seizure types. 3) The risk of recurrence varied according to the history of seizure, seizure recurrence was observed in 100% of the cases with history of neonatal seizure, 72.7% of the cases with febrile convulsion, and 73.3% of the cases with non-specific history. No significant difference was observed among these past history of seizure. 4) The rate of seizure recurrence according to electroencephalographic abnormalities did not differ significantly. Seizure recurrence was noted in 13 of the 18 patients with mildly disordered tracings (72.2%), 15 of the 20 patients with moderate abnormality (75.0%), and 12 of the 16 patients with severe abnormality (75.0%). 5) Recurrence rate according to cause of seizure was more significantly frequent in those with symptomatic epilepsy than in those with idiopathic type (100% vs 70.2%, p<0.05). 6) The frequency percentage of seizure recurrence by age groups of below 1 year, 1 to 3 years, 4 to 6 years, and above 6 years at onset of seizure were 100, 66.7, 57.1, and 72.7, respectively. The rate of seizure recurrence was significantly highest in patients aged below 1 year at onset of seizure. 7) There was significant difference in seizure recurrence between those with and without abnormalities as shown by neurologic examination (100% vs 70.8%, p<0.05). 8) There was no consistent difference in valproic acid serum levels between those who had a recurrence and those who did not. The patients receiving phenobarbital had significantly high serum levels of the phenobarbital in recurrent groups than those who had no recurrence. In conclusion, factors associated with an increased risk of seizure recurrence were early age at onset of epilepsy, symptomatic epilepsy, and neurologic abnormalities. We found no associations between risks of recurrence and types of epilepsy, or electroencephalographic abnormalities.
Anticonvulsants* ; Child ; Epilepsy ; Humans ; Infant ; Infant, Newborn ; Neurologic Examination ; Phenobarbital ; Prospective Studies ; Recurrence* ; Risk Factors ; Seizures* ; Seizures, Febrile ; Spasms, Infantile ; Valproic Acid

Transient hyperammonemia in a newborn is an overwhelming disease manifested by hyperammonemic coma. The majority of affected newborns are premature and have mild respiratory syndrome. The diagnosis may be difficult to determine. This metabolic disorder is primarily characterized by severe hyperammonemia in the postnatal period, coma, absence of abnormal organic aciduria and normal activity of the enzymes of the urea cycle. Hyperammonemic coma may develop within 2-3 days of life, although its etiology is unknown. Laboratory studies reveal marked hyperammonemia (>4,000 micromol/L). The degree of neurologic impairment and developmental delay in this disorder depends on the duration of hyperammonemic coma. Moreover, the infant may succumb to the disease if treatment is not started immediately and continued vigorously. Hyperammonemic coma as a medical emergency requires dialysis therapy. Here, we report a case of severe transient hyperammonemia in a preterm infant (35 week of gestation) presented with respiratory distress, seizure, and deep coma within 48 hours and required ventilatory assistance and marked elevated plasma ammonia levels. He survived with aggressive therapy including peritoneal dialysis, and was followed 2 years later without sequelae.
Ammonia ; Coma ; Dialysis ; Emergencies ; Humans ; Hyperammonemia ; Infant ; Infant, Newborn ; Infant, Premature ; Peritoneal Dialysis ; Plasma ; Seizures ; Urea

Since the introduction of shoulder arthroplasty by Neer in 1974, the design of not only the glenoid component but also the humeral component used in shoulder arthroplasty has continually evolved. Changes to the design of the humeral component include a gradually disappearing proximal fin; diversified surface finishes (such as smooth, grit-blasted, and porous coating); a more contoured stem from the originally straight and cylindrical shape; and the use of press-fit uncemented fixation as opposed to cemented fixation. Despite the evolution of the humeral component for shoulder arthroplasty, however, stem-related complications are not uncommon. Examples of stem-related complications include intraoperative humeral fractures, stem loosening, periprosthetic fractures, and stress shielding. These become much more common in revision arthroplasty, where patients are associated with further complications such as surgical difficulty in extracting the humeral component, proximal metaphyseal bone loss due to stress shielding, intraoperative humeral shaft fractures, and incomplete cement removal. Physicians have made many attempts to reduce these complications by shortening the stem of the humeral component. In this review, we will discuss some of the limitations of long-stem humeral components, the feasibility of replacing them with short-stem humeral components, and the clinical outcomes associated with short-stemmed humeral components in shoulder arthroplasty.
Arthroplasty ; Humans ; Humeral Fractures ; Periprosthetic Fractures ; Shoulder

Intermittent exotropia occurs commonly in Korea and its clinical characteristics are relatively well known. We analyzed the clinical characteristics of intermittent exotropia and studied the relationship among them. We examined 229 patients(92 males and 137 females( who first visited three strabismic clinics. The age of onset, frequency, family history and symptoms of intermittent exotropia were examined. The angle and type of deviation were studied and sensory tests were done. The mean age of onset was 32.4 months and frequency ws 39.1% and family history was 8.7%. Photophobia was the most common symptom of intermittent exotropia. The distant angle of deviation was 26.7 prism diopters in average and the near angle was 25.3 prism diopters in average. The most common type of intermittent exotropia was basic type. followed by simulated divergence excess type, convergence insufficiency type, and divergence excess type. In statistical study, there was no significant relationship among clinical characteristics of intermittent exotroia.
Age of Onset ; Exotropia* ; Humans ; Korea ; Male ; Ocular Motility Disorders ; Photophobia ; Statistics as Topic ; Strabismus

STUDY DESIGN: Level III, retrospective studies. OBJECTIVES: This study is conducted to evaluate the effect and usefulness of ultrasound guided facet block for the outpatients who complained of chronic lower back pain. SUMMARY OF THE LITERATURE REVIEW: Facet joint syndrome was introduced in 1976, by Mooney V. It was considered to be one of the major causes of low back pain. MATERIALS AND METHODS: Between October 2009 to March 2011, 98 cases were selected from the outpatients who complained of chronic lower back pain for more than 3 months. The patients had no surgery history and did not complain of neuromuscular symptoms, and they had more than three times outpatient care at least and could be followed up for 1 year. The patients were divided into three groups; first was the ultrasound guided facet block group (27 cases), second was the Fluoroscopy guided facet block group (39cases) and third was the conservative group (32 cases).The clinical results were analyzed using Kim's criteria and the Visual Analog Scale score, ODI score, Physician's global assessment (subjective), Patient's global assessment (Objective). RESULTS: The VAS score was improved from an average of 7.75+/-1.5 to 4.47+/-2.4 in the ultrasound guided facet block group, and from 7.81+/-1.4 to 4.39+/-2.6 in the Fluoroscopy guided facet block group, and from 7.87+/-1.3 to 6.24+/-2.1 in the conservative group. The VAS score, Kim's criteria, ODI score, Physician's global assessment (subjective), and Patient's global assessment (Objective) showed statistically significant improvement in the ultrasound guided facet block group and Fluoroscopy guided facet block group. CONCLUSION: In the outpatient clinics, comparing with the group that underwent conservative treatment with medication, the group treated with ultrasound for block could have better results in clinical improvement.
Ambulatory Care ; Ambulatory Care Facilities ; Fluoroscopy ; Humans ; Low Back Pain ; Outpatients ; Retrospective Studies ; Zygapophyseal Joint

Hemangioblastomas are benign tumors that most commonly occur in the posterior fossa around the 4th ventricle. Recurrent mutifocal hemangioblastomas are often found as a part of a systemic autosomal dominant disease, the Von Hippel-Lindau syndrome. Surgical removal of recurrent multifocal tumors are technically more difficult and challenging than solitary ones due to their proximity to critical structures, disturbed anatomical landmark and adehesion to the surrounding tissue, all of which may lead to high postoperative morbidity and mortality. Authors have experienced 2 cases of the multiple and recurrent hemangioblastomas with Von Hippel-Lindau syndrome. The patients of each of the cases underwent surgery for tumor removal at least 2 times. In the first patient, a 42-year-old male, a solitary cerebellar hemangioblastoma had been removed 6 years prior to recurrence. On followup MRI, more than three solid, homogenously enhanced nodules were found in the cerebellum. These tumors were operated on through the previous craniectomy site, but only two of them could be removed. This patient was discharged without additional neurologic deficit. The 2nd patient, a 40-year-old-male, had undergone three prior operations for cerebellar hemangioblastomas. On followup examination, he presented with progressive ataxia and confusion. Brain MRI showed multiple recurrent lesions of homogenously strongenhanced masses in the 4th ventricle and cerebellar vermis surrounded by cystic lesions, accompanied by obstructive hydrocephalus. No additional surgery of tumors were attempted, and only ventriculoperitoneal shunt was performed. He was discharged with improved neurological symtomes and sign.
Adult ; Ataxia ; Brain ; Cerebellum ; Follow-Up Studies ; Hemangioblastoma* ; Humans ; Hydrocephalus ; Magnetic Resonance Imaging ; Male ; Mortality ; Neurologic Manifestations ; Radiosurgery ; Recurrence ; Ventriculoperitoneal Shunt ; von Hippel-Lindau Disease*

Duplication cysts are rare congenital malformations, that may be detected anywhere along the alimentary tract, and they may communicate with the intestinal tract. Cystic duplication of the cecum is especially rare. About 80% of these cases are detected in the first 2 years of life as a result of an acute intestinal obstruction, which manifests as vomiting, recurrent abdominal pain, recurrent gastrointestinal bleeding and constipation. We report a case of intestinal obstruction secondary to a duplication cyst of the cecum in a neonate. The patient underwent surgery and was diagnosed subsequently, and is presently healthy.
Abdominal Pain ; Cecum ; Constipation ; Hemorrhage ; Humans ; Infant, Newborn ; Intestinal Obstruction ; Vomiting

A retrospective review of 36 patients with infective endocarditis by strict case definition was performed from 1972 through April of 1984. Male to female ratio was 1:1.25, with mean age of 19.1. Thirty of the 36 patients(83.3%) had the history of predisposing heart diseases: congenital heart disease was most frequent one below the age of 20, rheumatic heart disease between the age of 20-39 and degenerative heart disease above the age of 40. The commonest presenting symptoms were fever, dyspnea, and malaise, and the commonest signs were fever, murmur, and hepatomegaly. Average hemoglobinand hematocrit were 10.1g/dl and 30.8% respectively, showing moderate degree of anemia. White cell count was 11,600+/-5400/mm3 and erythrocyte sedimentation rate was 46+/-18mm/hr. Over four fifths of the patients demonstrated proteinuria and elevated C-reactive protein. Two thirds of the patients showed microscopic hematuria and one third showed positive rheumatoid factor. Eight patients(22.2%) showed arrhythmia and 4(11.1%) showed conduction abnormalities on electrocardiography. The commonest etiologic microorganisms were alpha-hemolytic streptococcus(30.6%) and staphylococcus aureus(22.2%). Abacteremic cases were found in 38.9% of the patients. To compared with alpha-hemolytic streptoccus endocarditis the cases caused by staphylococcus aureus showed the shorter duration of symptoms before admission, the less frequent complication, the higher fever, and the more elevated white cell counts. To penicillin, alpha-hemolytic streptococcus was sensitive in most cultures and staphylococcus arueus was resistant in half of them, and to aminoglycosides vice versa. To cefazolin, both microorganisms were sensitive in most cultures. In 18 of 24(75%) patients vegetation was visualized by echocardiography. The most frequent site of involvement was the mitral valve. And in vegetation detected group complication rate by embolism was found to be higher. The common complications were congestive heart gailure in 24(66.75%), embolism in 8(22.2%), and metastatic infection in 6(16.7%). The mortality rate of infective endocarditis was 25%, and the causes of death were cerebral embolism in 3(8.3%), septic septic shock in 3(8.3%), congestive heart failure in 2(5.6%), and mycotic aneurysmal rupture in 1(2.8%). Conclusively in infective endocarditis the author observed the increasing prevalence of degenerative heart disease as a prdisposing heart disease and increasing incidence of staphylococcus aureus as a causative microorganism. Adn the differences of clinical characteristics between staphylococcus ureus and alpha-hemolytic streptococcus endocarditis would be helpful in diagnosis and treatment of infective endocarditis as well as recognition of prognosis.
Aminoglycosides ; Anemia ; Aneurysm, Infected ; Arrhythmias, Cardiac ; Blood Sedimentation ; C-Reactive Protein ; Cause of Death ; Cefazolin ; Cell Count ; Diagnosis ; Dyspnea ; Echocardiography ; Electrocardiography ; Embolism ; Endocarditis* ; Estrogens, Conjugated (USP) ; Female ; Fever ; Heart ; Heart Defects, Congenital ; Heart Diseases ; Heart Failure ; Hematocrit ; Hematuria ; Hepatomegaly ; Humans ; Incidence ; Intracranial Embolism ; Male ; Mitral Valve ; Mortality ; Penicillins ; Prevalence ; Prognosis ; Proteinuria ; Retrospective Studies ; Rheumatic Heart Disease ; Rheumatoid Factor ; Rupture ; Shock, Septic ; Staphylococcus ; Staphylococcus aureus ; Streptococcus

Canavan's disease is a hereditary disease that causes development delay by demyelinization of white matter in brain. The cardinal symptoms of Canavan's disease are head-lag, macrocephaly, developmental delay, blindness, epilepsy and hypotonia. Seven-month old baby delivered by Caesarean section at gestational age 40 weeks was complaining of an inability to keep head up. In past history, he was treated for congenital nystagmus. Chromosomal study was normal. Brain MRI showed delay of myelination of 5 months old. During neurodevelopment treatment in our hospital about development delay, macrocephaly was observed with head circumference 46 cm (90~97 percentile). He couldn't control his head yet. Brain MRI was done when he was 12-month old. There was no myelination in whole brain compared with that of same age group. The peak elevation of N-acetylaspartic acid (NAA) was showed in magnetic resonance spectroscopy (MRS). NAA was detected as high as 29.7 mmol/molCr, we diagnosed him as Canavan's disease. So we reported this case with a brief review of related literatures.
Aspartic Acid ; Blindness ; Brain ; Cesarean Section ; Epilepsy ; Female ; Genetic Diseases, Inborn ; Gestational Age ; Head ; Humans ; Macrocephaly ; Magnetic Resonance Spectroscopy ; Muscle Hypotonia ; Myelin Sheath ; Nystagmus, Congenital ; Pregnancy

Evans syndrome is the combination of direct Coombs' positive hemolytic anemia and immune thrombocytopenic purpura, in the absence of a known underlying etiology. Being reported rarely in pediatric patients, the syndrome is characterized by periods of remission and exacerbation with viable responses to therapy. Management of the disease remains a challenge despite a variety of therapeutic trials. We experienced a 11-years old male patient of Evans syndrome who was initially presented as having an autoimmune hemolytic anemia 17 months before. Over the 5 years of follow-up, he had a chronic, relapsing courses, showing partial responses to a variety of therapeutic trials, including IVIG, oral prednisolone, methylprednisolone pulse therapy, cyclosporine A and vincristine. A brief review of the literature ensues with the case report.
Anemia, Hemolytic ; Anemia, Hemolytic, Autoimmune ; Child ; Cyclosporine ; Follow-Up Studies* ; Humans ; Immunoglobulins, Intravenous ; Male ; Methylprednisolone ; Prednisolone ; Purpura, Thrombocytopenic, Idiopathic ; Vincristine