Background: and Purpose The natural course of adult-onset moyamoya disease (MMD) is unknown, and there is no medical treatment that halts its progression. We hypothesized that progressive shrinkage of large intracranial arteries occurs in adult-onset MMD, and that cilostazol inhibits this process. Methods: Serial high-resolution magnetic resonance imaging (HR-MRI) was performed on 66 patients with MMD: 30 patients received cilostazol, 21 received other antiplatelets, and 15 received no antiplatelets or had poor compliance to them. Serial HR-MRI was performed (interval between MRI scans: 29.67±18.02 months, mean±SD), and changes in outer diameter, luminal stenosis, and vascular enhancement were measured. Factors affecting HR-MRI changes were evaluated, including vascular risk factors and the ring finger protein 213 gene variant. Results: The progression of stenosis to occlusion, recurrent ischemic stroke, and the development of new stenotic segments were observed in seven, seven, and three patients, respectively. Serial HR-MRI indicated that the degree of stenosis increased with negative remodeling (outer diameter shrinkage). Patients who received cilostazol presented significantly larger outer diameters and lower degrees of stenosis compared with other groups (p=0.005 and p=0.031, respectively). After adjusting for clinical and genetic factors, only cilostazol use was independently associated with negative remodeling (odds ratio=0.29, 95% confidence interval=0.10–0.84, p=0.023). While vascular enhancement was observed in most patients (61 patients), the progression of enhancement or the occurrence of new vascular enhancement was rarely observed on follow-up HR-MRI (6 and 1 patients, respectively). Conclusions Adult-onset MMD induces progressive shrinkage of large intracranial arteries, which cilostazol treatment may prevent. Further randomized clinical trials are warranted.

Background: and Purpose Previous studies have assessed the relationship between cerebral vessel tortuosity and intracranial aneurysm (IA) based on two-dimensional brain image analysis. We evaluated the relationship between cerebral vessel tortuosity and IA according to the hemodynamic location using three-dimensional (3D) analysis and studied the effect of tortuosity on the recurrence of treated IA. Methods: We collected clinical and imaging data from patients with IA and disease-free controls. IAs were categorized into outer curvature and bifurcation types. Computerized analysis of the images provided information on the length of the arterial segment and tortuosity of the cerebral arteries in 3D space. Results: Data from 95 patients with IA and 95 controls were analyzed. Regarding parent vessel tortuosity index (TI; P<0.01), average TI (P<0.01), basilar artery (BA; P=0.02), left posterior cerebral artery (P=0.03), both vertebral arteries (VAs; P<0.01), and right internal carotid artery (P<0.01), there was a significant difference only in the outer curvature type compared with the control group. The outer curvature type was analyzed, and the occurrence of an IA was associated with increased TI of the parent vessel, average, BA, right middle cerebral artery, and both VAs in the logistic regression analysis. However, in all aneurysm cases, recanalization of the treated aneurysm was inversely associated with increased TI of the parent vessels. Conclusions TIs of intracranial arteries are associated with the occurrence of IA, especially in the outer curvature type. IAs with a high TI in the parent vessel showed good outcomes with endovascular treatment.

Background: and Purpose Previous studies have assessed the relationship between cerebral vessel tortuosity and intracranial aneurysm (IA) based on two-dimensional brain image analysis. We evaluated the relationship between cerebral vessel tortuosity and IA according to the hemodynamic location using three-dimensional (3D) analysis and studied the effect of tortuosity on the recurrence of treated IA. Methods: We collected clinical and imaging data from patients with IA and disease-free controls. IAs were categorized into outer curvature and bifurcation types. Computerized analysis of the images provided information on the length of the arterial segment and tortuosity of the cerebral arteries in 3D space. Results: Data from 95 patients with IA and 95 controls were analyzed. Regarding parent vessel tortuosity index (TI; P<0.01), average TI (P<0.01), basilar artery (BA; P=0.02), left posterior cerebral artery (P=0.03), both vertebral arteries (VAs; P<0.01), and right internal carotid artery (P<0.01), there was a significant difference only in the outer curvature type compared with the control group. The outer curvature type was analyzed, and the occurrence of an IA was associated with increased TI of the parent vessel, average, BA, right middle cerebral artery, and both VAs in the logistic regression analysis. However, in all aneurysm cases, recanalization of the treated aneurysm was inversely associated with increased TI of the parent vessels. Conclusions TIs of intracranial arteries are associated with the occurrence of IA, especially in the outer curvature type. IAs with a high TI in the parent vessel showed good outcomes with endovascular treatment.

The activation of neurotrophic signaling pathways following the upregulation of glial cell line-derived neurotrophic factor (GDNF), a member of the transforming growth factor-β family, has a potential neuroprotective effect in the adult brain. Herein, we report that hippocampal transduction of adeno-associated virus serotype 1 (AAV1) with a constitutively active form of ras homolog enriched in brain [Rheb(S16H)], which can stimulate the production of brain-derived neurotrophic factor (BDNF) in hippocampal neurons, induces the increases in expression of GDNF and GDNF family receptor α-1 (GFRα-1), in neurons and astrocytes in the hippocampus of rat brain in vivo . Moreover, upregulation of GDNF and GFRα-1 contributes to neuroprotection against thrombin-induced neurotoxicity in the hippocampus. These results suggest that AAV1-Rheb(S16H) transduction of hippocampal neurons, resulting in neurotrophic interactions between neurons and astrocytes, may be useful for neuroprotection in the adult hippocampus.

OBJECTIVES: To investigate longitudinal changes in behavior and quality-of-life (QoL) in children with sleep disordered breathing (SDB) after adenotonsillectomy (AT). METHODS: This study prospectively enrolled 148 children who underwent AT for SDB. Caregivers filled out Korean attention deficit hyperactivity disorder (ADHD) rating scale (K-ARS) and Korean version of the obstructive sleep apnea-18 (KOSA-18) at preoperative 1 day, postoperative 1 month, and postoperative 6 months. Longitudinal changes in K-ARS and KOSA-18 were evaluated and compared among subgroups. RESULTS: Both K-ARS and KOSA-18 scores improved immediately at postoperative 1 month and were maintained at postoperative 6 months. The mean preoperative K-ARS scores of the non-ADHD, ADHD-trait, and overt-ADHD groups showed uniformly immediate and sustained improvements. Also, the mean preoperative KOSA-18 scores of the small, moderate, and large impact groups were significantly improved both at postoperative 1 month and 6 months. The changes in behavior and QoL showed no significant differences with regard to the aforementioned factors. CONCLUSION: After AT, we may expect immediate and sustained improvements in behavior and QoL in children with SDB regardless of preoperative symptom severity. Moreover, by performing AT, improvements in behavior and QoL are expected regardless of sex, age, allergic trait, or concurrent coblation-assisted turbinoplasty. These findings may help our colleague physicians counselling pediatric SDB patients and their caregivers.
Adenoidectomy ; Attention Deficit Disorder with Hyperactivity ; Caregivers ; Child* ; Humans ; Prospective Studies ; Quality of Life* ; Sleep Apnea Syndromes* ; Tonsillectomy

Premature ovarian failure (POF) is a condition in which the ovarian functions of hormone production and oocyte development become impaired before the typical age for menopause. POF and early menopause are present in a broad spectrum of gonad dysgenesis, from a complete cessation of ovarian function to an intermittent follicle maturation failure. Actually POF has been identified as a genetic entity (especially chromosome X), but data on genetic factors of premature menopause are limited. Until now, several cases revealed that inactivation of X chromosomes has an effect on ages of premature menopause and females with balanced or unbalanced X-autosome translocations can have several reproductive problems. On the other hand, there have been a few data that was caused by autosome-autosome translocation can lead. Therefore we report a relevant case of POF with translocation between chromosomes 1 and 4. She had her first menstrual period at the age of 12, and after 7 years she stopped menstruation. Chromosomal analysis showed 46, XX, t (1;4) (p22.3;q31.3). While evaluating this rare case, we could review various causes (especially genetic factors) of POF. To remind clinicians about this disease, we report a case of POF caused by autosome-autosome translocation with a literature review.
Female ; Gonads ; Hand ; Humans ; Menopause ; Menopause, Premature* ; Menstruation ; Oocytes ; Primary Ovarian Insufficiency ; X Chromosome

Uterine serous adenocarcinoma (USC) is rare and invasive cancer. This cancer is more often reported in the ovary, the fallopian tube, and the endometrium than uterine cervix. No matter where the tumor is located, the tumor exhibits similar histological characteristics. So when uterine cancer is proven to be serous adenocarcinoma, it is necessary to see if the tumor originated from ovary or endometrium and invaded the cervix. We report a case of a 73-year-old postmenopausal woman with USC arising near the internal os of endocervical canal, clinically misdiagnosed as uterine cervix cancer.
Adenocarcinoma* ; Aged* ; Cervix Uteri* ; Endometrium ; Fallopian Tubes ; Female ; Humans ; Ovary ; Uterine Cervical Neoplasms ; Uterine Neoplasms ; Uterus

PURPOSE: This study evaluated the demographics and semen parameters of males with cancer who banked their sperm prior to chemotherapy. MATERIALS AND METHODS: This is a retrospective study of 66 cases referred for sperm banking prior to initiation of chemotherapy over a 15-year period (1999~2014). Patients who had previously received cancer treatment including chemotherapy or radiotherapy were not included in this study. RESULTS: We studied a total of 66 cancer patients referred for cryopreservation of sperm prior to chemotherapy. The mean age of the patients at the time of banking was 32.0+/-7.9 years (range, 19~58 years). The types of cancer were testicular cancer (31 cases, 47.0%), non-Hodgkin's disease (10 cases, 15.1%), Hodgkin's disease (5 cases, 7.6%), leukemia (8 cases, 12.1%), gastrointestinal malignancy (5 cases, 7.6%), and musculoskeletal malignancy (5 cases, 7.6%). There were significant differences in sperm concentration and viability among the various types of cancer, but no significant difference in semen volume or sperm motility and morphology. CONCLUSIONS: In this study we found that sperm quality could decrease even before chemotherapy. Because chemotherapy can also negatively affect spermatogenesis, sperm cryopreservation prior to treatment should be strongly recommended for cancer patients of reproductive age.
Cryopreservation* ; Demography ; Drug Therapy* ; Hodgkin Disease ; Humans ; Infertility, Male ; Korea ; Leukemia ; Male ; Radiotherapy ; Retrospective Studies ; Semen ; Semen Analysis* ; Sperm Banks ; Sperm Motility ; Spermatogenesis ; Spermatozoa* ; Testicular Neoplasms

PURPOSE: Infertile couples interested in nonspouse artificial insemination by donor (AID) not only require a thorough understanding of the medical procedure but also must scrutinize the effects it will have on family relationships, including those on the infant to be born. We conducted a series of surveys in couples with male infertility to collect information necessary for AID counseling. MATERIALS AND METHODS: A total of 384 cases with noncurable male infertility were enrolled in this inquiry survey. The questionnaire consisted of 18 items that assessed demographic characteristics, background information concerning the choice to use AID, subjective experiences, long-term effects, and an overall evaluation. RESULTS: A total of 126 surveys were returned (32.8%). AID was first suggested by the husband in about half of the cases. The major reason for considering the procedure was to form a complete family. Two-thirds of the couples were anxious about the procedure, most often about possible congenital or acquired deformities in the infant. After the birth of the child, most couples were positive about their decision to have used AID. About half of the couples felt that the child was their own and expected not to tell the child about AID. Overall, about 50% of the couples were satisfied with the procedure. CONCLUSIONS: Those who underwent AID experienced various psychological effects, including anxiety about the child to be born. To overcome these problems, sufficient medical information and consultation about the process of selecting the donor and about AID procedures should be provided before the procedure is used.
Anxiety ; Child ; Congenital Abnormalities ; Counseling ; Family Characteristics ; Family Relations ; Humans ; Infant ; Infertility, Male ; Insemination, Artificial* ; Male* ; Parturition ; Questionnaires ; Sperm Banks ; Spouses ; Tissue Donors*

Lymphangioma is a rare, benign mesenchymal neoplasm, which is characterized by numerous intercommunicating cystic spaces containing lymphatic fluid. It is considered a congenital disease resulting from the obstruction of regional lymph drainage during the developmental period. Lymphangioma may be focal/unilateral or diffuse/bilateral, and in the latter case, it is referred to as lymphangiomatosis. Here, we report a case of a 38-year-old man with perirenal lymphangiomatosis. The patient's chief complaint was left flank pain, and left pleural effusion was found on radiological examination. After radical nephrectomy, the pathological examinations revealed that the kidney was enclosed by a multicystic mass with intrarenal cystic dilatations. We report the case and discuss the management of perirenal lymphangiomatosis with a literature review.
Adult ; Dilatation ; Drainage ; Flank Pain ; Humans ; Kidney ; Lymphangioma ; Lymphatic Diseases ; Nephrectomy ; Pleural Effusion