BACKGROUND: Seizures in the neonate are relatively common and their clinical features are different from those in children and adults. The study aimed to provide the clinical profiles of neonatal seizure in our hospital. METHODS: A total of 41 newborns with seizures were enrolled in this study over a period of three years. They were evaluated with special reference to risk factors, neurologic examinations, laboratory data, neuroimaging studies, EEG findings, seizure types, response to treatment, and prognosis, etc. RESULTS: The average age at onset of seizures was 6.1+/-4.6 days and the majority of patients(42%) had multifocal clonic seizure and 24% had subtle seizure. Factors that are known to increase risk of neonatal seizures include abnormal delivery history, birth asphyxia, and electrolyte imbalance, etc. However, they remain obscure in about 20% of cases. More than 50 percent showed abnormal lesions on neuroimaging studies such as brain hemorrhage, periventricular leukomalacia, brain infarction, cortical dysplasia, hydrocephalus, etc. and 17 out of 32 patients showed abnormal electroencephalographic patterns. Phenobarbital was tried as a first line antiepileptic drug and phenytoin was added if it failed to control seizures. The treatments were terminated in the majority of patients during the hospital stay. The overall prognosis was relatively good except for those with abnormal EEG background or congenital central nervous system malformations. CONCLUSIONS: Neonatal seizures may permanently disrupt brain development. Better understanding of their clinical profiles and appropriate management may lead to a reduction in neurological disability in later childhood.
Adult ; Asphyxia ; Brain ; Brain Infarction ; Central Nervous System ; Child ; Electroencephalography ; Humans ; Hydrocephalus ; Infant, Newborn ; Intracranial Hemorrhages ; Length of Stay ; Leukomalacia, Periventricular ; Malformations of Cortical Development ; Neuroimaging ; Neurologic Examination ; Phenobarbital ; Phenytoin ; Prognosis* ; Reproductive History ; Risk Factors ; Seizures*

PURPOSE: microRNAs (miRNAs) are non-coding RNAs composed of 20 to 22 nucleotides that regulate development and differentiation in various organs by silencing specific RNAs and regulating gene expression. In the present study, we show that the microRNA (miR)-183 cluster is upregulated during hair cell regeneration and that its inhibition reduces hair cell regeneration following neomycin-induced ototoxicity in zebrafish. MATERIALS AND METHODS: miRNA expression patterns after neomycin exposure were analyzed using microarray chips. Quantitative polymerase chain reaction was performed to validate miR-183 cluster expression patterns following neomycin exposure (500 µM for 2 h). After injection of an antisense morpholino (MO) to miR-183 (MO-183) immediately after fertilization, hair cell regeneration after neomycin exposure in neuromast cells was evaluated by fluorescent staining (YO-PRO1). The MO-183 effect also was assessed in transgenic zebrafish larvae expressing green fluorescent protein (GFP) in inner ear hair cells. RESULTS: Microarray analysis clearly showed that the miR-183 cluster (miR-96, miR-182, and miR-183) was upregulated after neomycin treatment. We also confirmed upregulated expression of the miR-183 cluster during hair cell regeneration after neomycin-induced ototoxicity. miR-183 inhibition using MO-183 reduced hair cell regeneration in both wild-type and GFP transgenic zebrafish larvae. CONCLUSION: Our work demonstrates that the miR-183 cluster is essential for the regeneration of hair cells following ototoxic injury in zebrafish larvae. Therefore, regulation of the miR-183 cluster can be a novel target for stimulation of hair cell regeneration.
Animals ; Animals, Genetically Modified ; Cell Count ; Gene Expression Profiling ; Gene Expression Regulation/drug effects ; Gene Knockdown Techniques ; Green Fluorescent Proteins/metabolism ; Hair Cells, Auditory/drug effects ; Hair Cells, Auditory/physiology ; Larva/drug effects ; Larva/genetics ; MicroRNAs/genetics ; MicroRNAs/metabolism ; Morpholinos/pharmacology ; Neomycin/toxicity ; Regeneration/drug effects ; Regeneration/genetics ; Zebrafish/genetics

We performed an immunohistochemical study on the estrogen receptor alpha (ER-alpha) distribution in the cerebellum of a human neonate with multiple congenital anomalies, that had been acquired during autopsy. Although the exact pathology in the brain was not clearly elucidated in this study, an unidentified stressful condition might have induced the astrocytes into reactive states. In this immunohistochemical study on the neonatal cerebellum with multiple congenital anomalies, intense ER-alpha immunoreactivities (IRs) were localized mainly within the white matter even though ER-alpha IRs were known to be mainly localized in neurons. Double immunohistochemical staining showed that ER-alpha IR cells were reactive astrocytes, but not neurons. Interestingly, there were differences in the process length among the reactive astrocytes showing ER-alpha IRs. Our quantitative data confirmed that among the glial fibrillary acidic protein (GFAP)-expressing reactive astrocytes, the cells exhibiting intense ER-alpha IRs have much longer cytoplasmic processes and relatively weaker GFAP IRs. Taken together, the elongated processes of reactive astrocytes might be due to decreased expression of GFAP, which might be induced by elevated expression of ER-alpha even though the elucidation of the exact mechanism needs further studies.
Abnormalities, Multiple/*pathology ; Astrocytes/*metabolism ; Autopsy ; Brain/pathology ; Cerebellum/*metabolism ; Cytoplasm/metabolism ; Estrogen Receptor alpha/*metabolism ; Female ; Gastrointestinal Diseases/congenital/*pathology ; *Gene Expression Regulation ; Glial Fibrillary Acidic Protein/metabolism ; Humans ; Immunohistochemistry/methods ; Infant, Newborn ; Urogenital Abnormalities/*pathology

PURPOSE: To report on the changes in the patterns of care and survival over time for esthesioneuroblastoma. MATERIALS AND METHODS: We retrospectively analyzed 42 previously untreated and histologically confirmed esthesioneuroblastoma patients seen between March 1989 and June 2007. According to Kadish's classification, 3 patients (7%) were stage A, 6 (14%) at stage B, and 33 (79%) at stage C. Of the 33 Kadish C patients, 19 and 14 patients were treated from 1989 through 2000 and from 2001 through 2007, respectively. Treatment included surgical resection, radiotherapy, chemotherapy, or a combination of these methods. Chemotherapy was administered to 8 of 19 patients (42%) seen from 1989 through 2000, whereas all of the 14 patients seen from 2001 through 2007 received chemotherapy (p<0.001). No patient was treated by three-dimensional conformal radiotherapy (3D-CRT) from 1989 through 2000, however 8 of 14 patients (67%) seen from 2001 through 2007 underwent 3D-CRT (p<0.001). The median follow-up time for surviving patients was 6.5 years (range, 2.2~15.8 years). RESULTS: The 5-year overall survival (OS) and progression-free survival (PFS) rates for the entire cohort were 53% and 39%, respectively. The 5-year OS was 100% for Kadish stages A or B and 39% for stage C (p=0.007). For patients with stage C disease who were treated from 1989 to 2000 and from 2001 to 2007, the 5-year OS rate was 26% and 59% (p=0.029), respectively and the corresponding 5-year PFS rate was 16% and 46% (p=0.001), respectively. Intraorbital extension and treatment era (1989~2000 vs. 2001~2007) were found as independent factors for OS and PFS in a multivariate analyses. CONCLUSION: The results of this study suggest that treatment era, which features a distinction in treatment modality and technique with the introduction of 3D-CRT, may be the cause of improved OS and PFS in Kadish stage C patients. To achieve better outcomes for patients with Kadish stage C, combined chemoradiotherapy, especially 3D-CRT, is recommended in addition to surgery.
Chemoradiotherapy ; Cohort Studies ; Disease-Free Survival ; Esthesioneuroblastoma, Olfactory ; Follow-Up Studies ; Humans ; Multivariate Analysis ; Prognosis ; Radiotherapy, Conformal ; Retrospective Studies

OBJECTIVES: Laryngomicrosurgery (LMS) is used to manage most vocal fold lesions. However, the functional voice outcome of the LMS might be diverse due to the influence of various factors. We intend to evaluate the incidence and etiologic factors of persistent dysphonia after LMS for benign vocal fold disease (BVFD). METHODS: We performed a retrospective review of 755 patients who underwent LMS for BVFD. We analyzed the clinical characteristics, preoperative and postoperative two onths voice studies. Postsurgical dysphonia was defined as grade 1 or above in GRBAS (grade, roughness, breathiness, asthenia, and strain) scale. Thirty nine patients (5.2%; 25 males and 14 females; average, 42.9 years; range, 21 to 70 years) were diagnosed with postsurgical dysphonia. RESULTS: There was no correlation between the diagnosis, coexistence with laryngopharyngeal reflux disease, habit of smoking, or occupational voice abuse and voice outcome. The patients with a worse preoperative acoustic parameter had aworse voice outcome. Stroboscopic findings showed excessive scarring or bowing in 21 cases, presence of lesion remnant in eight cases, prolonged laryngeal edema in five and no abnormal findings in three. CONCLUSION: Great care should be taken in patients with worse preoperative jitter. With a few exceptions, postoperative dysphonia can be avoided by the use of an ppropriate surgical technique.
Acoustics ; Asthenia ; Cicatrix ; Dysphonia ; Humans ; Incidence ; Laryngeal Edema ; Laryngopharyngeal Reflux ; Male ; Retrospective Studies ; Smoke ; Smoking ; Vocal Cords ; Voice

Bilateral pneumothorax is very rare in primary spontaneous pneumothorax patients. This condition can cause chest pain, dyspnea, and even lead to tension pneumothorax. Spontaneous hemopneumothorax can be lethal due to massive bleeding and hypovolemic shock. This condition requires precise decision making and emergency management. We report on a case of simultaneous bilateral spontaneous pneumothorax combined with hemopneumothorax in a healthy 21-year-old male patient with chest discomfort. In the emergency department, closed thoracostomy was performed for decompression of hemopneumothorax, with drainage of 850 mL of blood. Then bilateral video-assisted thoracoscopic surgery was performed successfully.
Chest Pain ; Decision Making ; Decompression ; Drainage ; Dyspnea ; Emergencies ; Emergency Service, Hospital ; Hemopneumothorax* ; Hemorrhage ; Humans ; Male ; Pneumothorax* ; Shock ; Thoracic Surgery, Video-Assisted ; Thoracostomy ; Thorax ; Young Adult

Leiomyoma is the most common benign tumor of the esophagus, but it still occurs rarely, as compared with the incidence of cacinoma. There are no geographic or racial differences and manifestations are unusual and inconsistent. About 97% of the esophageal leiomyoma may oecur in intramural type and 1 of the tumor may be polypoid type. Considerable diagnostic problems may arise as well as problems of proper surgical management. We experienced a case of a 47-year old female with esophageal leiomyoma in the mid- point of the esophagus. The patient complained of substernal discomfort for 1 month and routine examinatian and gastrofiberscope were performed. The gastrofiberscopic finding was asmoothly protruded, round bean-sized polypoid mass in the midpoint of the esophagus which was removed by polypectomy. There were no other complications. So we reported this case with review of literature.
Esophagus ; Female ; Humans ; Incidence ; Leiomyoma* ; Middle Aged

Purpose: Nasopharyngeal cancer (NPC) has a higher prevalence of regional nodal metastasis than other head and neck cancers; however, level IB lymph node involvement is rare. We evaluated the safety and feasibility of level IB-sparing radiotherapy (RT) for NPC patients. Materials and Methods: We retrospectively reviewed 236 patients with NPC who underwent definitive intensity-modulated RT with or without chemotherapy between 2004 and 2018. Of them, 212 received IB-sparing RT, and 24 received non-IB-sparing RT. We conducted a propensity score matching analysis to compare treatment outcomes according to IB-sparing status. In addition, dosimetric analysis of the salivary glands was performed to identify the relationship between xerostomia and the IB-sparing RT. Results: The median follow-up duration was 78 months (range, 7 to 194 months). Local, regional, and distant recurrences were observed in 11.9%, 6.8%, and 16.1% of patients, respectively. Of the 16 patients with regional recurrence, 14 underwent IB-sparing RT. The most common site categorization of regional recurrence was level II (75%), followed by retropharyngeal lymph nodes (43.8%); however, there was no recurrence at level IB. In the matched cohorts, IB-sparing RT was not significantly related to treatment outcomes. However, IB-sparing RT patients received a significantly lower mean ipsilateral and contralateral submandibular glands doses (all, p < 0.001) and had a lower incidence of chronic xerostomia compared with non-IB-sparing RT patients (p = 0.006). Conclusion Our results demonstrated that IB-sparing RT is sufficiently safe and feasible for treating NPC. To reduce the occurrence of xerostomia, IB-sparing RT should be considered without compromising target coverage.

PURPOSE: This retrospective study was conducted to assess outcome and to determine the prognostic factors in patients with a desmoid tumor treated with postoperative radiotherapy. MATERIALS AND METHODS: Twenty-seven patients with a desmoid tumor who were treated with postoperative radiotherapy between June 1984 and October 2005 were analyzed. There were 13 male and 14 female patients. The age of the patients ranged from 3 to 79 years (median age, 28 years). Tumors were located in an extra-abdominal area (21 cases), and in the abdominal walls (6 cases). The tumor size ranged from 2.5 to 25 cm (median size, 7.5 cm) in the largest linear dimension. Thirteen cases received radiotherapy after initial surgery, and 14 recurrent cases received radiotherapy after additional surgery. The total radiation dose given was 45~66 Gy (median dose, 59.4 Gy), and the fraction size was 1.8~2.0 Gy. RESULTS: The median follow-up period was 61 months (range, 12~203 months). Two patients developed local progression and six patients experienced local recurrence. The 5-year disease-free survival rate and the 5-year progression-free survival rate were 61% and 70%, respectively. Wide local excision was associated with better disease free survival with statistical significance (p=0.028). Radiotherapy after initial surgery (p=0.046) and a higher radiation dose of more than 60 Gy (p=0.049) were associated with better progression free survival with statistical significance. At the time of the last follow-up, the number of additional surgeries was higher in patients that received radiotherapy after reoperation (p<0.001). CONCLUSION: Radiotherapy after the initial operation improved local control and decreased the number of subsequent operations. Thus, postoperative radiotherapy after an initial operation is recommended in patients with a high risk of recurrence for a desmoid tumor.
Disease-Free Survival ; Female ; Fibromatosis, Aggressive* ; Follow-Up Studies ; Humans ; Male ; Radiotherapy ; Radiotherapy, Adjuvant* ; Recurrence ; Reoperation ; Retrospective Studies

The authors report below a clinical study of 23 patients bearing 31 primary central nervous system lymphomas diagnosed between January 1985 and December 1994. The cohort included 13 men and 10 women whose mean age was 46 years, ranging from 28 to 61 years. No patient had antecedent of human immunodeficiency virus positivity but one had a past history of rheumatoid arthritis. The duration of symptom was less than 8 weeks in 52% of the patients. Symptom groups included increased intracranial pressure(78%), focal neurological decificit(52%), neuropsychiatric symptoms(43%), and seizures(13%). The histopathologcal diagnosies were done in 19 cases(10 cases by resective surgery, 9 cases by open or stereotactic biopsy). The others were diagnosed by the typical clinical course such as rapid disappearance of lesions after steroid therapy, and/or radiological findings. Histological subtypes(National Cancer Institute Working Formulation) was confirmed in 8 patients including 3 cases of diffuse larger cell type. Phenotype was determined in 7 patients: 4 were B-cell type and 3 were T-cell type. One patient committed suicide during the radiation therapy and was therefore excluded from the survival analysis. All but two patients received radiation therapy. Five patients received chemotherapy. The over-all Kaplan-Meier survival rate was 46% at 2 years and 15.5% at 5 years. On univariate analysis, statistically significant prognostic factor associated with survival was not found but the higher Karnofsky score and single lesion were found to be favorable to the long-term survival. In the statistical analysis of the patients who received radiation therapy, surgical resection did not significantly influence the survival.
Arthritis, Rheumatoid ; B-Lymphocytes ; Brain Neoplasms ; Central Nervous System* ; Cohort Studies ; Drug Therapy ; Female ; HIV ; Humans ; Lymphoma ; Male ; Phenotype ; Suicide ; Survival Rate ; T-Lymphocytes