We present two patients with a dural arteriovenous fistula (dAVF) of the ophthalmic sheath who developed progressive exophthalmos, conjunctival chemosis, and visual loss. These symptoms mimic those of cavernous sinus dAVFs. Dural AVFs of the ophthalmic sheath are extremely rare and their clinical management is controversial. We successfully treated these two patients by transvenous coil embolization. Transvenous embolization appears to be a safe and effective method to treat dAVFs of the ophthalmic sheath.
Cavernous Sinus ; Central Nervous System Vascular Malformations* ; Embolization, Therapeutic* ; Exophthalmos ; Humans ; Methods

With focused ultrasound (FUS) and microbubbles, BBB can be transiently disrupted with a localized and non-invasive approach. BBB disruption induced by FUS has made progressions to move forward on delivery of therapeutic agents into a brain in a specific area of brain for better treatment of neurological diseases. In addition to be used as an improvement of drug delivery, BBB disruption has been found to induce biological effects such as a clearance of protein aggregation which cause Alzheimer's disease, regulation of proteins which facilitate drug uptake, and modulation of neuronal function and neurogenesis. In this review, we discuss overview about the principles of BBB opening with FUS and milestones in these biological effects of FUS-induced BBB disruption.
Alzheimer Disease ; Brain ; Microbubbles ; Neurogenesis ; Neurons ; Ultrasonography*

Purpose: Despite enzyme replacement therapy (ERT) and/or allogeneic hematopoietic stem cell transplantation, individuals with mucopolysaccharidosis (MPS) I or II often experience significant growth deficiencies. This study aimed to assess the safety and efficacy of recombinant human growth hormone (hGH) treatment in children diagnosed with MPS I or II. Materials and Methods: A total of nine pediatric patients—four with MPS I and five with MPS II—underwent treatment with ERT and hGH at Samsung Medical Center. Results: The mean hGH dose administered was 0.26±0.03 mg/kg/week. In the MPS I group, three patients showed an increase in height Z-score from –4.09±0.83 to –3.68±0.43 after 1 year of hGH treatment, and to –3.10±0.72 by the end of the hGH regimen. In the MPS II group, while the height Z-score of four patients decreased according to standard growth charts, it improved from 1.61±1.79 to 2.71±1.68 based on the disease-specific growth chart through hGH treatment. Two patients discontinued hGH treatment due to lack of efficacy after 22 and 6 months each of treatment, respectively. No new-onset neurological symptoms or necessity for prosthetic or orthopedic surgery were reported during hGH treatment. Conclusion This study provides insights into the impact of hGH on MPS patients, demonstrating its potential to reverse growth deceleration in some cases. Further research is needed to explore the long-term effects of hGH on changes in body composition, muscle strength, and bone health in this population.

PURPOSE: We evaluate respiratory morbidities in infants beyond 35 weeks of gestation born via elective cesarean section by gestational age. METHODS: This is a retrospective study of 443 infants who were born at Seoul National University Hospital by elective cesarean section beyond 35 weeks of gestation from January 2011 to December 2012. We compared respiratory morbidities in four groups classified by gestational age (35(+0)-36(+6) weeks, 37(+0)-37(+6) weeks, 38(+0)-38(+6) weeks, 39(+0)-40(+6) weeks). RESULTS: There were significantly lower Apgar scores in the late-preterm infant group (35-36 weeks) compared to other term infant groups and the proportion of infants born from mothers with preeclampsia gradually decreased as gestational age increased. There were significant differences in O2 supplement, duration of O2 (>24 hours), checked chest radiography, transient tachypnea of newborn (TTN), transfer to neonatal intensive care unit (NICU), endotracheal intubation, and ventilator uses including nasal continuous positive airway pressure in four groups (P<0.05). By logistic regression analysis, compared to births at 38 weeks, births at 35-36 weeks and at 37 weeks were associated with an increased risk of respiratory morbidities [odds ratios (OR) and 95% confidence intervals (CI) for births at 35-36 weeks, 122.5 (17.4-863.4) for TTN; 54.0 (10.1-289.4) for transfer to NICU; 99.5 (14.9-666.2) for ventilator apply; OR and 95% CI for births at 37 weeks, 8.8 (1.6-50.1) for TTN; 5.3 (1.1-24.7) for transfer to NICU; 8.4 (1.5-47.7) for ventilator apply; P<0.05]. There were no significant differences in respiratory morbidities between births at 38 weeks and births at 39-40 weeks. CONCLUSION: Postponing the timing of elective cesarean section to beyond 38 weeks of gestation would be helpful in reducing the neonatal respiratory morbidities.
Cesarean Section* ; Continuous Positive Airway Pressure ; Female ; Gestational Age* ; Humans ; Infant ; Infant, Newborn* ; Intensive Care, Neonatal ; Intubation, Intratracheal ; Logistic Models ; Mothers ; Parturition ; Pre-Eclampsia ; Pregnancy ; Pregnancy* ; Radiography ; Respiration Disorders ; Retrospective Studies ; Seoul ; Term Birth ; Thorax ; Transient Tachypnea of the Newborn ; Ventilators, Mechanical

BACKGROUND: Angiomatous meningioma is a rare histological subtype of meningioma. Therefore, this specific medical condition is rarely reviewed in the literature. In the present work, we report the clinical and radiological features with postoperative outcomes of angiomatous meningioma. METHODS: This retrospective study included the patients who were pathologically diagnosed with angiomatous meningioma after surgical resection between February 2010 and September 2015 in our institute. We analyzed the clinical data, radiological manifestation, treatment and prognosis of all patients. RESULTS: The 15 patients (5 males and 10 females) were diagnosed with angiomatous meningioma during the study period. The median age of patients at the time of surgery was 63 years (range: 40 to 80 years). According to Simpson classification, 7, 5, and 3 patients achieved Simpson grade I, II, and IV resection, respectively. In the follow-up period, recurrence was noted in one patient. Ten out of the 15 patients showed homogeneous enhancement. Two patients demonstrated cystic changes. There was no occurrence of calcification or hemorrhage in our patients. Characteristically, 14 out of 15 patients showed signal voids of vessels. Significant peritumoral edema was observed in the majority of tumors (67%). CONCLUSION: Angiomatous meningiomas are rare benign meningioma. Brain images of angiomatous meningioma usually demonstrate signal void signs and peritumoral edema. In the present study, angiomatous meningiomas showed good prognosis after surgical resection.
Brain ; Brain Edema ; Classification ; Edema ; Follow-Up Studies ; Hemorrhage ; Humans ; Magnetic Resonance Imaging ; Male ; Meningioma* ; Prognosis ; Recurrence ; Retrospective Studies

BACKGROUND: Non-functioning pituitary adenomas (NFPA) invading into the cavernous sinus are surgically challenging. To decrease recurrence rate, surgeon makes a strong endeavor to resect tumor gross totally. However, gross total resection (GTR) is difficult to achieve with cavernous sinus invasion. Recently, a new classification system for cavernous invasion of pituitary adenomas was suggested. The aim of this study is to validate this new classification system and to identify limitations and considerations in designing treatment strategies for patients with NFPA involving the cavernous sinus. METHODS: Between January 2000 and January 2012, 275 patients who underwent operation for NFPA were enrolled in the study. Median age was 50 years (15–79 years). There were 145 males and 130 females. The median follow-up duration was 4 years (range 1–12.5 years). RESULTS: Related to extent of tumor removal, GTR was obtained in 184 patients (66.9%), near total resection (NTR) was obtained in 45 patients (16.3%), and sub-total resection (STR) was obtained in 46 patients (16.7%) of a total 275 patients. There were statistically significant differences between the extent of resection and the new Knosp classification (p<0.001). In the high-grade group of the new Knosp classification, there was no difference in recurrence between patients who underwent GTR or NTR only and those who underwent STR with adjuvant radiation therapy (p=0.515). CONCLUSION: In case of high risk of surgical complications, STR with adjuvant radiation therapy can be considered as an alternative strategy for safe treatment of cavernous-invading adenomas.
Adenoma ; Cavernous Sinus ; Classification ; Female ; Follow-Up Studies ; Humans ; Male ; Pituitary Neoplasms* ; Recurrence

Osteoarthritis (OA) is the most common degenerative joint disorder in the elderly population. To identify OA-associated genetic variants and candidate genes, we conducted a genome-wide association study (GWAS). A total 3,793 samples (476 cases: wrist + knee and 3317 controls) from a community-based epidemiological study were genotyped using the Affymetrix SNP 5.0. An intronic SNP (rs4789934) in the TIMP2 (tissue inhibitor of metalloproteinase-2) showed the most significance with OA (odd ratio [OR] = 2.06, 95% confidence interval [CI] = 1.52-2.81, p = 4.01 x 10(-6)). Furthermore, a polymorphism (rs1352677) in the NKAIN2 (Na+/K+ transporting ATPase interacting 2) was suggestively associated with OA (OR = 1.43, CI = 1.22-1.66, p = 7.01 x 10(-6)). The present study provides new insights into the identification of genetic predisposing factors for OA.
Adenosine Triphosphatases ; Aged ; Epidemiologic Studies ; Genome-Wide Association Study ; Humans ; Introns ; Joints ; Knee ; Osteoarthritis ; Wrist

OBJECTIVE: To evaluate the efficacy of temozolomide (TMZ) chemotherapy for recurrent anaplastic oligodendroglioma (AO) and anaplastic oligoastrocytoma (AOA). METHODS: A multi-center retrospective trial enrolled seventy-two patients with histologically proven AO/AOA who underwent TMZ chemotherapy for their recurrent tumors from 2006 to 2010. TMZ was administered orally (150 to 200 mg/m2/day) for 5 days per 28 days until unacceptable toxicity occurred or tumor progression was observed. RESULTS: TMZ chemotherapy cycles administered was median 5.3 (range, 1-41). The objective response rate was 24% including 8 cases (11%) of complete response and another 23 patients (32%) were remained as stable disease. Severe side effects (> or =grade 3) occurred only in 9 patients (13%). Progression-free survival (PFS) of all patients was a median 8.0 months (95% confidence interval, 6.0-10.0). The time to recurrence of a year or after was a favorable prognostic factor for PFS (p<0.05). Overall survival (OS) was apparently differed by the patient's histology, as AOA patients survived a median OS of 18.0 months while AO patients did not reach median OS at median follow-up of 11.5 months (range 2.7-65 months). Good performance status of Eastern Cooperative Oncology Group 0 and 1 showed prolonged OS (p<0.01). CONCLUSION: For recurrent AO/AOA after surgery followed by radiation therapy, TMZ could be recommended as a salvage therapy at the estimated efficacy equal to procarbazine, lomustine, and vincristine (PCV) chemotherapy at first relapse. For patients previously treated with PCV, TMZ is a favorable therapeutic option as 2nd line salvage chemotherapy with an acceptable toxicity rate.
Disease-Free Survival ; Drug Therapy* ; Follow-Up Studies ; Humans ; Lomustine ; Oligodendroglioma* ; Procarbazine ; Recurrence ; Retrospective Studies ; Salvage Therapy ; Vincristine