No abstract available.
Chondrosarcoma* ; Nasal Cavity*

Early-onset epileptic encephalopathies are one of the most severe early onset epilepsies that can lead to progressive psychomotor impairment. These syndromes result from identifiable primary causes, such as structural, neurodegenerative, metabolic, or genetic defects, and an increasing number of novel genetic causes continue to be uncovered. A typical diagnostic approach includes documentation of anamnesis, determination of seizure semiology, electroencephalography, and neuroimaging. If primary biochemical investigations exclude precipitating conditions, a trial with the administration of a vitaminic compound (pyridoxine, pyridoxal-5-phosphate, or folinic acid) can then be initiated regardless of presumptive seizure causes. Patients with unclear etiologies should be considered for a further workup, which should include an evaluation for inherited metabolic defects and genetic analyses. Targeted next-generation sequencing panels showed a high diagnostic yield in patients with epileptic encephalopathy. Mutations associated with the emergence of epileptic encephalopathies can be identified in a targeted fashion by sequencing the most likely candidate genes. Next-generation sequencing technologies offer hope to a large number of patients with cryptogenic encephalopathies and will eventually lead to new therapeutic strategies and more favorable long-term outcomes.
Electroencephalography ; Epilepsy ; Genetics ; High-Throughput Nucleotide Sequencing ; Hope ; Humans ; Neuroimaging ; Psychomotor Disorders ; Seizures ; Vitamins

No abstract available.
Free Tissue Flaps*

Lithium is a commonly prescribed drug for bipolar disorder. Because of the narrow therapeutic range, lithium intoxication continues to be prevalent. Drugs that alter renal function such as ACEI, ARB, NSAIDS, and thiazide can increase the risk of chronic lithium toxicity even to stable patients. A 65-year old woman was admitted for hand tremor, cognitive impairment, and lethargy. A medical history included major depressive disorder, mitral stenosis, atrial fibrillation, and hypertension. Her prescription included lithium, quetiapine, digoxin, furosemide, and warfarin. She recently received Atacand Plus (candesartan plus thiazide) for hypertension. At the time of admission, the patient was drowsy and confused. The serum lithium level was 4.25 mEq/L. The patient received hydration. Due to neurologic complications and the degree of lithium toxicity, a total of three sessions of hemodialysis were performed, and the post-dialysis serum lithium level was 0.54 mEq/L. The neurologic symptoms recovered completely after a third dialysis session.
Aged ; Anti-Inflammatory Agents, Non-Steroidal ; Atrial Fibrillation ; Bipolar Disorder ; Depressive Disorder, Major ; Dialysis ; Digoxin ; Female ; Furosemide ; Hand ; Humans ; Hypertension ; Lethargy ; Lithium* ; Mitral Valve Stenosis ; Neurologic Manifestations ; Poisoning* ; Prescriptions ; Renal Dialysis* ; Tremor ; Warfarin ; Quetiapine Fumarate

PURPOSE: Reversible posterior leukoencephalopathy syndrome(RPLS) is a complex condition affecting gray/white matter of parieto-occipital lobes from multiple and different etiologies. This study was aimed to analyze clinical characteristics of this condition in Korean children. METHODS: A total of 36 patients from tertiary care medical centers were involved in the study. They were diagnosed as RPLS mainly by clinical and radiological manifestations. Their medical records and radiological features of brain MRI were retrospectively analyzed. RESULTS: Thirty six patients were involved in the study(21 males and 15 females, mean age: 9.8+/-4.1 years of age). Main underlying causes included drug induced(47%), renal diseases(28%), neurogenic tumors(8%) and so on. Initial manifestations were seizures, headache, visual disturbance, mental change, and nausea/vomiting. 13 patients(36%) had two or more symptoms. The brain MRI showed typical pattern of fairly symmetric, high T2 signal intensity in both parieto-occipital regions in 23 patients(64%) and various, atypical pattern in 13 patients(36%). 26 patients had the lesions affecting predominantly white matter, but 10 patients had the lesions affecting predominantly gray matter. 34 patients(94 %) had bilateral lesions, but 2 patients(6%) had unilateral lesion. This complex syndrome was associated with acute rise of blood pressure from a variety of conditions. A patient ended up a visual deficit despite the good prognosis. CONCLUSION: RPLS in Korean children seems to be complex, but a better understanding of this complex syndrome will lead to better clinical outcome by avoiding unnecessary investigations and appropriate management.
Blood Pressure ; Brain ; Child ; Female ; Headache ; Humans ; Leukoencephalopathies ; Male ; Medical Records ; Posterior Leukoencephalopathy Syndrome ; Retrospective Studies ; Seizures ; Tertiary Healthcare

Pulmonary embolism is a fatal complication of major operation. But early detection of pulmonary thromboembolism during general anesthesia is not easy because clinical manifestations of pulmonary thromboembolism are nonspecific during general anesthesia. So the prevention of pulmonary thromboembolism is important for the high risk patients. We experienced a case of pulmonary thromboembolism which occurred in an old female patient with femur fracture immediately after leg elevation following induction of general anesthesia.
Anesthesia, General ; Female ; Femur ; Humans ; Leg ; Orthopedics ; Pulmonary Embolism

This study was conducted to investigate the etiology, the clinical characteristics and prognosis of acute necrotizing encephalopathy (ANE) in Korean children. Six children (1 yr to 7 yr) patients with ANE were enrolled. They were diagnosed by clinical and radiological characteristics and their clinical data were retrospectively analyzed. In a search of clinically plausible causes, brain MRI in all patients, mitochondrial DNA studies for mitochondrial encephalomyopathy, lactic acidosis, and strokelike episodes (MELAS) and myoclonus epilepsy and ragged red fibers (MERRF) in four patients, and genomic typing on HLA DRB/HLA DQB genes in three patients were performed. All had precedent illnesses and the main initial symptoms included mental change (83%), seizures (50%), and focal deficits (50%). MRI revealed increased T2 signal density in the bilateral thalami and/or the brainstem in all patients. Mitochodrial DNA studies for MELAS and MERRF were negative in those children and HLA-DRB1*1401, HLA-DRB3*0202, and HLA-DQB1*0502 seemed to be significant. A high dose steroid was given to all patients, which seemed to be partly effective except for 2 patients. In conclusion, ANE is relatively rare, but can result in serious neurological complication in children. Early detection and appropriate treatment may lead to a better neurological outcome.
Child ; Child, Preschool ; Female ; HLA-DQ Antigens/metabolism ; HLA-DQ beta-Chains ; HLA-DR Antigens/metabolism ; HLA-DRB1 Chains ; HLA-DRB3 Chains ; Humans ; Infant ; Korea ; Leukoencephalitis, Acute Hemorrhagic/diagnosis/etiology/*pathology/*physiopathology ; MELAS Syndrome/pathology/physiopathology ; MERRF Syndrome/pathology/physiopathology ; Magnetic Resonance Imaging ; Male ; Prognosis ; Retrospective Studies

PURPOSE: This study was aimed to compare the effectiveness and tolerability of topiramate add-on therapy versus lidocaine add-on therapy in children with refractory status epilepticus. METHODS: We performed a retrospective analysis of children with refractory status epilepticus who were hospitalized at Kyungpook National University Hospital during the period 2005 to 2012. We divided them into two groups, Group A (midazolam, MDZ, and Topiramate, TPM:11 patients) and Group B (midazolam and lidocaine, LDC:7 patients). Furthermore, they were sub-categorized according to children's sex, age, seizure duration, and seizure type. To comepare the effectiveness and tolerability between the two groups, we evaluated seizure freedom, >50% seizure reduction, adverse events and mortality rates, respectively. RESULTS: In MDZ/TPM group, average dosage of midazolam infusion was 10.8+/-6.8 mcg/kg/min, seizure freedom was achieved in 1 out of 11 patients (9%) and >50% seizure reduction was in 2 out of 11 patients (18%). On the other hand, in MDC/LDC group, average dosage of midazolam infusion is 11.3+/-5.2 mcg/kg/min, seizure freedom was achieved in 2 out of 7 patients (29%) and >50% seizure reduction was 3 out of 7 patients (43%). Aside from the promising effects, there was no significant difference between the two groups. CONCLUSION: In children with refractory status epilepticus, topiramate or lidocaine add-on therapy seemed to be effective and fairly tolerated and showed no serious adverse effects. Therefore they can be an alternative treatment option. However, further studies are required.
Child* ; Freedom ; Gyeongsangbuk-do ; Hand ; Humans ; Lidocaine* ; Midazolam ; Mortality ; Retrospective Studies ; Seizures ; Status Epilepticus*

PURPOSE: Although benign rolandic epilepsy (BRE) is a benign condition, it may be associated with a spectrum of behavioral, psychiatric, and cognitive disorders. This study aimed to assess the cognitive and other neuropsychological profiles of children with BRE. METHODS: In total, 23 children with BRE were consecutively recruited. All children underwent sleep electroencephalography (EEG) and were assessed on a battery of comprehensive neuropsychological tests including the Korean versions of the Wechsler intelligence scale for children III, frontal executive neuropsychological test, rey complex figure test, Wisconsin card sorting test, attention deficit diagnostic scale, and child behavior checklist scale. RESULTS: The study subjects included 13 boys and 10 girls aged 9.0+/-1.6 years. Our subjects showed an average monthly seizure frequency of 0.9+/-0.7, and a majority of them had focal seizures (70%). The spike index (frequency/min) was 4.1+/-5.3 (right) and 13.1+/-15.9 (left). Of the 23 subjects, 9 showed frequent spikes (>10/min) on the EEG. The subjects had normal cognitive and frontal executive functions, memory, and other neuropsychological sub-domain scores, even though 8 children (35%) showed some evidence of learning difficulties, attention deficits, and aggressive behavior. CONCLUSION: Our data have limited predictive value; however, these data demonstrate that although BRE appears to be benign at the onset, children with BRE might develop cognitive, behavioral, and other psychiatric disorders during the active phase of epilepsy, and these problems may even outlast the BRE. Therefore, we recommend scrupulous follow-up for children with BRE.
Aged ; Attention Deficit Disorder with Hyperactivity ; Checklist ; Child ; Child Behavior ; Cognition ; Electroencephalography ; Epilepsy ; Epilepsy, Rolandic ; Executive Function ; Humans ; Intelligence ; Intelligence Tests ; Learning ; Memory ; Neuropsychological Tests ; Seizures ; Wisconsin

PURPOSE: This study was aimed to evaluate the effectiveness and safety of midazolam infusion and topiramate add-on therapy for refractory status epilepticus (RSE) in children. METHODS: Nineteen children were involved in the study and their clinical data were retrospectively analyzed. After failure of initial conventional treatment, all of the children were given midazolam (MDZ) infusion, titrated until a response is achieved or maximum dose of 20 mcg/kg/min. Eleven children were given additional topiramate (TPM) at 1-2mg/kg/day by nasogastric and titrated to the favorable response every 1-3 days. We subdivided the children into 2 groups by whether TPM added (TPM group) or not (MDZ group) and compared them each other. RESULTS: The age of subjects ranged from 2 to 175 months (54.9+/-53.3 months; male 10, female 9). Seizure duration of initial presentation ranged from 30 to 1,440 minutes (218.4+/-432.9 minutes). Seizure types were convulsive in 16 children (84%) and 3 were non-convulsive (16%). In the MDZ group (4.0+/-2.9 mcg/kg/minutes), time to seizure control ranged from 1 day to 2 days (1.1+/-0.4 days), but seizures recurred in 50 %. In the TPM group (6.3+/-1.5 mg/kg/day), time to seizure control ranged from 1 day to 5 days (1.9+/-1.5 days), but seizures recurred in 36%. 1 child (5.3%) died of sepsis and 7 children (36.8%) experienced cardio-respiratoty compromise. 7 children (63.6%) in the TPM group recovered completely to baseline neurological function. CONCLUSIONS: The results indicate that midazolam infusion is still the mainstay of treatment, but TPM add-on therapy seems to be a useful option in RSE. However, further studies are needed to establish its precise role and safety.
Child ; Female ; Fructose ; Humans ; Male ; Midazolam ; Retrospective Studies ; Seizures ; Sepsis ; Status Epilepticus