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Item Case Report First pediatric case with primary familial brain calcification due to a novel variant on the MYORG gene and review of the literature(2021) Orgun, Leman Tekin; Besen, Seyda; Sangun, Ozlem; Bisgin, Atil; Alkan, Ozlem; Erol, IlknurVariants in the myogenesis-regulating glycosidase (MYORG) gene which is known as the first autosomal recessive gene that has been associated with primary familial brain calcification (AR-PFBC). Although adult patients have been reported, no pediatric case has been reported until now. Herein, we review the clinical and radiological features of all AR- PFBC patients with biallelic variants in the MYORG gene who were reported until now, and we report the youngest patient who has a novel homozygous variant. Since the first identification of the MYORG gene in 2018, 74cases of MYORG variants related to AR-PFBC were evaluated. The ages of symptom onset of the patients ranged between 7.5 and 87 years. The most frequent clinical courses were speech impairment, movement disorder and cerebellar signs. All patients showed basal ganglia calcification usually bilaterally with different severities. Conclusion; herein, we reported the first pediatric patient in the literature who had a novel homozygous variant in the MYORG gene with mild clinic findings. (c) 2021 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved.Item EVALUATION OF EPICARDIAL FAT AND CAROTID AND FEMORAL INTIMA-MEDIA THICKNESSES IN GERIATRIC PATIENTS WITH END-STAGE RENAL DISEASE(2020) Gunesli, Aylin; Yilmaz, Mustafa; Yalcin, Cigdem; Tekkarismaz, Nihan; Alkan, Ozlem; 0000-0002-2557-9579; 0000-0001-5483-8253; 0000-0001-7631-7395; S-6973-2016; AAM-4284-2021; AAD-9088-2021Introduction: Although atherosclerotic cardiovascular diseases and cardiovascular risks are known to increase in patients with end-stage renal disease, it is not clear whether these risks increase in the geriatric patient population as well. This study aims to evaluate these risks in geriatric patients with end-stage renal disease by evaluating epicardial fat and carotid and femoral intima-media thicknesses, known as markers, for subclinical atherosclerosis and cardiovascular risks. Materials and Methods: This cross-sectional study included 52 patients who started to receive chronic hemodialysis treatment after the age of 65 years (mean age 73.92 +/- 5.63) years with end-stage renal failure and 51 healthy volunteers (mean age: 74.49 +/- 4.63 years). Epicardial fat and carotid and femoral intima-media thicknesses were measured and compared between these groups. Results: Carotid intima-media and epicardial fat thicknesses were significantly higher in the patient group than in the control group (0.91 +/- 0.08 vs. 0.71 +/- 0.1 mm, p<0.001 and 0.84 +/- 0.17 vs. 0.75 +/- 0.17 cm, p=0.01, respectively). However, no significant difference was observed in femoral intima-media thickness between the two groups (0.58 +/- 0.07 vs. 0.56 +/- 0.97 mm, p=0.266). Correlation analysis revealed a significant positive correlation between the duration of dialysis and epicardial fat and carotid intima-media thicknesses (r=0.611, p<0.001 and r=0.337, p=0.015, respectively). Furthermore, regression analysis revealed a significant relationship between the duration of dialysis and carotid intima-media thickness (beta=0.657, p=0.001). Conclusion: Epicardial fat and carotid intima-media thicknesses increase in geriatric patients with end-stage renal disease but with no significant changes in femoral intima-media thickness, indirectly suggesting that subclinical atherosclerosis and cardiovascular risks are increased in these patients.Item Blood pressure limits affecting carotid artery injury: a cross sectional study(2020) Guenesli, Aylin; Acibuca, Aynur; Altin, Cihan; Gezmis, Esin; Tekindal, Mustafa Agah; Yalcin, Cigdem; Alkan, Ozlem; 0000-0002-3444-8845; 0000-0001-7526-3460; 0000-0002-1001-6028; 0000-0001-5483-8253; AAE-8301-2021; ABG-4047-2020; AAM-4169-2021; AAM-4284-2021Purpose: Although it is well known that high blood pressure causes undesirable effects on carotid arteries, it is not clear as to which threshold value this effect starts. The aim of this study is to evaluate and determine this threshold. Materials and Methods: This cross-sectional study included a total of 308 individuals in the following groups; group 1: individuals with systolic blood pressure (SBP) <120 mmHg and diastolic blood pressure (DBP) <80 mmHg (optimal), group 2: SBP 120-129 mmHg and/or DBP 80-84 mmHg (normal), group 3: SBP 130-139 mmHg and/or DBP 85-89 mmHg (high-normal), group 4: SBP 140-159 mmHg and/or DBP 90-99 mmHg, group 5: SBP 160-179 mmHg and/or DBP 100-109 mmHg, and group 6: SBP >= 180 mmHg and/or DBP >= 110 mmHg. Carotid distensibility and elasticity were measured in all groups. Results: A statistically significant difference was determined between the groups in carotid distensibility and elasticity. SBP>135.5 mmHg started to affect carotid distensibility with 78.2% sensitivity and 74.6% specificity, and DBP>86.5 mmHg with 79.3% sensitivity and 71.6% specificity. Carotid elasticity was seen to be affected by SBP>137.5 mmHg with 80.4% sensitivity and 73.1% specificity, and DBP>88.5 mmHg with 79.1% sensitivity and 73.8% specificity. Conclusion: Carotid distensibility and elasticity decreases with increasing blood pressure. Individuals in high-normal group should be evaluated in terms of carotid disease, and it may be necessary to start treatment early in these patients.Item Knosp and Hardy Grading Systems are Useful in Predicting Persistence of Male Hypogonadism in Prolactinomas Following Prolactin Normalization(2020) Bagir, Gulay Simsek; Gunesli, Aylin; Haydardedeoglu, Filiz Eksi; Bakiner, Okan Sefa; Alkan, Ozlem; Ertorer, Melek Eda; 0000-0001-7357-8709; ABI-3705-2020Objective: Despite serum prolactin normalization and tumor shrinkage being obtained using dopamine agonist treatment, hypogonadism may persist in several men with prolactinomas. In this study, we evaluated the effects of tumor magnetic resonance imaging features on the persistence of hypogonadism among normoprolactinemic men with prolactinomas objectively using Knosp and Hardy grading systems. Material and Methods: The patients with prolactinomas who achieved serum prolactin normalization using cabergoline therapy were evaluated, respectively. The extent of tumor growth was evaluated on the basis of Knosp and Hardy grading systems both at diagnosis and six months of medical therapy with serum prolactin normalization. Results: A total of 28 cases (18 macro- and 10 microprolactinomas) were included. After six months of treatment with cabergoline, all microprolactinoma patients with hypogonadism at baseline showed recovery (3, 100%). Moreover, nine of 14 macroprolactinoma patients with hypogonadism at inclusion recovered at the end (group 1), and five did not (group 2). Baseline Knosp grades and Hardy numbers did not differ between groups. However, higher Knosp grades and Hardy numbers were observed in patients who consistently had low serum testosterone in the sixth month (group 2) (p=0.01, p=0.02, respectively). All patients in group 2 had invasive tumors (Hardy number III-IV) both at inclusion and the sixth month according to this classification. Conclusion: We demonstrated that macroprolactinomas with persistent hypogonadism despite serum prolactin normalization more commonly showed cavernous sinus invasion and sellar destruction. We proposed that Knosp and Hardy grading systems are useful in predicting the persistence of male hypogonadism in prolactinomas following prolactin normalization.Item Is fetal magnetic resonance imaging indicated in patients with isolated ventriculomegaly?(2019) Durdag, Gulsen Dogan; Baran, Safak Yilmaz; Kalayci, Hakan; Alkan, Ozlem; 0000-0001-5874-7324; 31228676; ABF-6439-2020Objective: Ventriculomegaly is one of the most common anomalies encountered at obstetric ultrasound and it necessitates follow up. Fetal magnetic resonance imaging (MRI) can be used to confirm the ultrasound diagnose or to detect additional anomalies. Aim of this study is to assess follow up and management of fetal ventriculomegaly shown by ultrasound, and to evaluate additional diagnostic contribution of MRI. Study Design: This study was conducted retrospectively including 89 patients who had fetal MRI subsequent to ultrasound diagnose of ventriculomegaly in between 2011-2017. Medical records of patients were investigated and accompanying anomalies, congenital infection. chromosomal examination, degree and progression of ventriculomegaly, neonatal imaging and diagnose, and neurodevelopmental findings on follow up were evaluated. Patients were classified in two groups as isolated and nonisolated ventriculomegaly, and subgroups mild, moderate, severe were formed according to their findings. SPSS 23.0 programme was used for statistical analysis. Results: Ultrasound and following MRI was performed in a range of 18-35 th gestational weeks, diagnoses were isolated ventriculomegaly for 56 patients and nonisolated ventriculomegaly for 33 patients. Progression and neurodevelopmental delay was higher in severe nonisolated ventriculomegaly group. There was not significant contribution of MRI in the follow up of isolated ventriculomegaly (p < 0.001), and diagnostic imaging findings declined in neonatal period with proceeding normal neurodevelopment in 92.7% of patients followed with diagnosis of isolated ventriculomegaly. Conclusion: When isolated ventriculomegaly is detected, ultrasound performed by an experienced team is mostly sufficient. MRI can be used in suspicious cases or when ventriculomegaly progresses. (C) 2019 Elsevier B.V. All rights reserved.Item Diffusion-Weighted MRI in Cranial Bone Marrow Metastasis(2015) Alkan, Ozlem; Pekoz, Burcak Cakir; Altinkaya, NaimePurpose: Diffusion-weighted MR imaging (DW-MRI) is generally applied to diagnose intra-axial brain pathologies such as parenchyma metastasis, abscesses, and infarcts in patients with systemic cancer. This study aimed to evaluate the DW-MRI findings in cranial bone marrow metastases to facilitate diagnosis. Material and Methods: Conventional MR imaging and DW-MRI findings of 57 patients with cranial bone marrow metastases were evaluated retrospectively. These images were evaluated by two neuroradiologists for lesion detection and lesion consciousness. Reader agreement was assessed by. statistics. Results: Fifty-seven patients exhibited 113 cranial bone marrow metastases. The sensitivities of DW-MRI and the apparent diffusion coefficient to detect cranial bone metastasis for all types of primary malignancy were 86% and 90%, respectively. The sensitivities of T1-weighted imaging (T1WI), fluid attenuation inversion recovery, contrast-enhanced fat-suppressed (CE-FS) T1WI, and T2WI sequences to detect cranial bone metastasis were 93%, 90%, 89%, and 84%, respectively. Regarding lesion conspicuousness, DW-MRI was equivalent to CE-FS T1WI and equivalent or superior to non-contrast conventional sequences in most of the cranial bone marrow metastases. Interobserver agreement was excellent. Conclusions: DW-MRI is generally indicated not only to assess the diagnosis of parenchyma involvement, but also to discover the cranial bone marrow metastasis in patients with systemic cancer.Item Watershed Cerebral Infarction in a Patient with Acute Renal Failure(2015) Ozelsancak, Ruya; Erken, Ertugrul; Giray, Semih; Alkan, OzlemAcute renal failure can cause neurologic manifestations such as mood swings, impaired concentration, tremor, stupor, coma, asterixis, dysarthria. Those findings can also be a sign of cerebral infarct. Here, we report a case of watershed cerebral infarction in a 70-year-old female patient with acute renal failure secondary to contrast administration and use of angiotensin converting enzyme inhibitor. Patient was evaluated with magnetic resonance imaging because of dysarthria. Magnetic resonance imaging revealed milimmetric acute ischemic lesion in the frontal and parietal deep white matter region of both cerebral hemisphere which clearly demonstrated watershed cerebral infarction affecting internal border zone. Her renal function returned to normal levels on fifth day of admission (BUN 32 mg/dl, creatinine 1.36 mg/dl) and she was discharged. Dysarthria continued for 20 days.Item Prognostic Impact of Histologic Subtype in Non-Small Cell Lung Cancer Patients Treated with Gamma Knife Radiosurgery: Retrospective Analysis of 104 Patients(2017) Tufan, Kadir; Aydemir, Fatih; Cekinmez, Melih; Kardes, Ozgur; Sarica, Feyzi Birol; Topkan, Erkan; Sonmez, Erkin; Alkan, Ozlem; Ugurluer, Gamze; Altınors, Nur; 0000-0002-5693-3542; 0000-0003-2854-941X; 0000-0001-8120-7123; 0000-0003-1509-4575; 0000-0001-9658-9005; 0000-0001-9985-0184; 27593758; AAI-8820-2021; P-5895-2018; AAG-2213-2021; AAK-1686-2021AIM: In this study, factors affecting survival, local failure, distant brain failure, whole brain failure and whole-brain radiation therapy (WBRT) free survival according to histological subtypes were investigated in patients with brain metastases from non-small cell lung cancer (NSCLC). MATERIAL and METHODS: Patients with positive pathology reports for adenocarcinoma (ACA) and squamous cell carcinoma (SCC) were included in the study. Seventy-eight ACA and 26 SCC patients were included in the study. Patients with previous history of cerebral metastasis surgery and WBRT were excluded from the study. RESULTS: The median survival was calculated as 12.6 months for patients with ACA and 5.9 months for patients with SCC. One-year distant brain failure was calculated as 65.1% in ACA patients and 39.6% in SCC patients. One-year whole brain failure was calculated as 58.1% in ACA patients and 39.6% in SCC patients. The one-year freedom from WBRT rate was calculated as 72.8% in ACA patients and 56.3% in SCC patients. SCC histology was considered as a significant factor in deterioration of overall survival in multivariate analysis. SCC histology, the increase in the number of metastases and RPA class were factors that caused an increase in distant brain failure. Also, SCC histology, the increase in the number of metastases and RPA class were factors that caused an increase in whole brain failure. CONCLUSION: SCC histology may be an important prognostic factor for overall survival. Also, due to high distant brain failure rate in SCC histology, WBRT can be added to treatment early.Item Endovascular Treatment of a Patient with Moyamoya Disease and Seckel Syndrome: A Case Report(2018) Andic, Cagatay; Gunesli, Aylin; Alkan, Ozlem; Erol, Ilknur; Suner, Halil Ibrahim; 30090148Seckel syndrome and Moyamoya diseases are different entities that rarely associated with each other. Several cases presenting with both these diseases were reported. Intracerebral artery aneurysms and collateral vessels can be seen with Moyamoya. They are commonly treated with medical treatment. We present a 12-years old patient with both Seckel syndrome and Moyamoya disease presented with middle cerebral artery aneurysm which was treated with endovascular modalities.