Identifying the risk factors for intracranial herniation in patients with cerebral venous thrombosis.

BACKGROUND: Cerebral venous sinus thrombosis (CVST) is not as well understood as an ischemic stroke of arterial origin. Although the prognosis of CVST is usually good, parenchymal lesions may occur in some patients, and the development of intracranial herniation may result in death. For this reason, recognizing the risk factors for intracranial herniation and accurately determining those patients who should undergo decompressive craniectomy is important. OBJECTIVE: This study aims to determine the risk factors for intracranial herniation in patients with CVST. METHODS: A total of 177 patients diagnosed with CVST between 2015 and 2021 in our tertiary center were retrospectively included in this study. RESULTS: Of the 177 patients, 124 were female and 53 were male with mean ages of 40.65 ± 13.23 and 44.13 ± 17.09, respectively. Among those, 18 patients had developed intracranial herniation. A significant statistical relationship was observed between superior sagittal sinus thrombosis, sinus rectus thrombosis, venous collateral score, nonhemorrhagic venous infarct, presence of malignancy, small juxtacortical hemorrhage, and cortical vein thrombosis. The binary logistic regression analysis results showed that the most significant variables were the venous collateral score of 0, malignancy, and small juxtacortical hemorrhages. CONCLUSION: This study identified small juxtacortical hemorrhages, the presence of malignancy, and a venous collateral score of 0 to be independent risk factors for intracranial herniation in CVST patients. Drawing on these results, we recommend close clinical observation of CVST patients, as they may be candidates for decompressive craniectomy.

ANTECEDENTES: A trombose do seio venoso cerebral (CVST) não é tão bem compreendida como um acidente vascular cerebral isquémico de origem arterial. Embora o prognóstico de CVST seja geralmente bom lesões parenquimatosas podem ocorrer em alguns pacientes e o desenvolvimento de herniação intracraniana pode resultar em morte. Por esse motivo é importante reconhecer os fatores de risco para hérnia intracraniana e determinar com precisão os pacientes que devem ser submetidos à craniectomia descompressiva. OBJETIVO: Este estudo tem como objetivo determinar os fatores de risco para herniação intracraniana em pacientes com CVST. MéTODOS: Um total de 177 pacientes diagnosticados com CVST entre 2015 e 2021 em nosso centro terciário foram retrospectivamente incluídos neste estudo. RESULTADOS: Dos 177 pacientes 124 eram do sexo feminino e 53 do masculino com média de idade de 40 65 ± 13 23 e 44 13 ± 17 09 respectivamente. Destes 18 pacientes desenvolveram hérnia intracraniana. Uma relação estatística significativa foi observada entre trombose do seio sagital superior trombose do seio reto escore de colateral venosa infarto venoso não hemorrágico presença de malignidade pequena hemorragia justacortical e trombose da veia cortical. Os resultados da análise de regressão logística binária mostraram que as variáveis mais significativas foram o escore colateral venoso de 0 malignidade e pequenas hemorragias justacorticais. CONCLUSãO: Este estudo identificou pequenas hemorragias justacorticais a presença de malignidade e um escore colateral venoso de 0 como fatores de risco independentes para herniação intracraniana em pacientes CVST. Com base nesses resultados recomendamos uma observação clínica rigorosa dos pacientes CVST pois eles podem ser candidatos à craniectomia descompressiva.

Identifying the risk factors for intracranial herniation in patients with cerebral venous thrombosis / Identificação dos fatores de risco para herniação intracranial em pacientes com trombose venosa cerebral

Abstract Background Cerebral venous sinus thrombosis (CVST) is not as well understood as an ischemic stroke of arterial origin. Although the prognosis of CVST is usually good, parenchymal lesions may occur in some patients, and the development of intracranial herniation may result in death. For this reason, recognizing the risk factors for intracranial herniation and accurately determining those patients who should undergo decompressive craniectomy is important. Objective This study aims to determine the risk factors for intracranial herniation in patients with CVST. Methods A total of 177 patients diagnosed with CVST between 2015 and 2021 in our tertiary center were retrospectively included in this study. Results Of the 177 patients, 124 were female and 53 were male with mean ages of 40.65 ± 13.23 and 44.13 ± 17.09, respectively. Among those, 18 patients had developed intracranial herniation. A significant statistical relationship was observed between superior sagittal sinus thrombosis, sinus rectus thrombosis, venous collateral score, nonhemorrhagic venous infarct, presence of malignancy, small juxtacortical hemorrhage, and cortical vein thrombosis. The binary logistic regression analysis results showed that the most significant variables were the venous collateral score of 0, malignancy, and small juxtacortical hemorrhages. Conclusion This study identified small juxtacortical hemorrhages, the presence of malignancy, and a venous collateral score of 0 to be independent risk factors for intracranial herniation in CVST patients. Drawing on these results, we recommend close clinical observation of CVST patients, as they may be candidates for decompressive craniectomy.

Resumo Antecedentes A trombose do seio venoso cerebral (CVST) não é tão bem compreendida como um acidente vascular cerebral isquémico de origem arterial. Embora o prognóstico de CVST seja geralmente bom lesões parenquimatosas podem ocorrer em alguns pacientes e o desenvolvimento de herniação intracraniana pode resultar em morte. Por esse motivo é importante reconhecer os fatores de risco para hérnia intracraniana e determinar com precisão os pacientes que devem ser submetidos à craniectomia descompressiva. Objetivo Este estudo tem como objetivo determinar os fatores de risco para herniação intracraniana em pacientes com CVST. Métodos Um total de 177 pacientes diagnosticados com CVST entre 2015 e 2021 em nosso centro terciário foram retrospectivamente incluídos neste estudo. Resultados Dos 177 pacientes 124 eram do sexo feminino e 53 do masculino com média de idade de 40 65 ± 13 23 e 44 13 ± 17 09 respectivamente. Destes 18 pacientes desenvolveram hérnia intracraniana. Uma relação estatística significativa foi observada entre trombose do seio sagital superior trombose do seio reto escore de colateral venosa infarto venoso não hemorrágico presença de malignidade pequena hemorragia justacortical e trombose da veia cortical. Os resultados da análise de regressão logística binária mostraram que as variáveis mais significativas foram o escore colateral venoso de 0 malignidade e pequenas hemorragias justacorticais. Conclusão Este estudo identificou pequenas hemorragias justacorticais a presença de malignidade e um escore colateral venoso de 0 como fatores de risco independentes para herniação intracraniana em pacientes CVST. Com base nesses resultados recomendamos uma observação clínica rigorosa dos pacientes CVST pois eles podem ser candidatos à craniectomia descompressiva.

Specificity and sensitivity of the SeLECT score in predicting late seizures in patients undergoing intravenous thrombolytic treatment and the effect of diabetes mellitus and leukoaraiosis.

BACKGROUND: Seizures after stroke can negatively affect the prognosis of ischemic stroke and cause a decrease in quality of life. The efficacy of intravenous (IV) recombinant tissue plasminogen activator (rt-PA) treatment in acute ischemic stroke has been demonstrated in many studies, and IV rt-PA treatment has been increasingly used around the world. The SeLECT score is a useful score for the prediction of late seizures after stroke and includes the severity of stroke (Se), large artery atherosclerosis (L), early seizure (E), cortical involvement (C), and the territory of the middle cerebral artery (T). However, the specificity and sensitivity of the SeLECT score have not been studied in acute ischemic stroke patients that received IV rt-PA treatment. OBJECTIVE: In the present study, we aimed to validate and develop the SeLECT score in acute ischemic stroke patients receiving IV rt-PA treatment. METHODS: The present study included 157 patients who received IV thrombolytic treatment in our third-stage hospital. The 1-year seizure rates of the patients were detected. SeLECT scores were calculated. RESULTS: In our study, we found that the SeLECT score had low sensitivity but high specificity for predicting the likelihood of late seizure after stroke in patients administered IV rt-PA therapy. In addition to the SeLECT score, we found that the specificity and sensitivity were higher when we evaluated diabetes mellitus (DM) and leukoaraiosis. CONCLUSION: We found that DM was an independent risk factor for late seizures after stroke in a patient group receiving thrombolytic therapy, and late seizures after stroke were less frequent in patients with leukoaraiosis.

ANTECEDENTES: As convulsões após o AVC podem afetar negativamente o prognóstico do AVC isquêmico e causar uma diminuição na qualidade de vida. A eficácia do tratamento com ativador do plasminogênio tecidual recombinante (rt-PA) intravenoso (IV) no AVC isquêmico agudo foi demonstrada em muitos estudos, e o tratamento com rt-PA IV tem sido cada vez mais usado em todo o mundo. A pontuação SeLECT é uma pontuação útil para a previsão de convulsões tardias após AVC e inclui a gravidade do AVC (Se), aterosclerose de grandes artérias (L), convulsão precoce (E), envolvimento cortical (C) e o território do meio artéria cerebral (T). No entanto, a especificidade e a sensibilidade do escore SeLECT não foram estudadas em pacientes com AVC isquêmico agudo que receberam tratamento IV com rt-PA. OBJETIVO: No presente estudo, objetivamos validar e desenvolver o escore SeLECT em pacientes com AVC isquêmico agudo recebendo tratamento IV com rt-PA. MéTODOS: O presente estudo incluiu 157 pacientes que receberam tratamento trombolítico IV em nosso hospital de terceiro estágio. As taxas de convulsão de 1 ano dos pacientes foram detectadas. Os escores SeLECT foram calculados. RESULTADOS: Em nosso estudo, descobrimos que o escore SeLECT apresentou baixa sensibilidade, mas alta especificidade para prever a probabilidade de convulsão tardia após AVC em pacientes que receberam terapia IV com rt-PA. Além do escore SeLECT, descobrimos que a especificidade e a sensibilidade foram maiores quando avaliamos diabetes mellitus (DM) e leucoaraiose. CONCLUSãO: Descobrimos que DM foi um fator de risco independente para convulsões tardias após AVC em um grupo de pacientes recebendo terapia trombolítica, e convulsões tardias após AVC foram menos frequentes em pacientes com leucoaraiose.

Specificity and sensitivity of the SeLECT score in predicting late seizures in patients undergoing intravenous thrombolytic treatment and the effect of diabetes mellitus and leukoaraiosis / Especificidade e sensibilidade do escore SeLECT na predição de convulsões tardias em pacientes submetidos a tratamento trombolítico intravenoso e o efeito do diabetes mellitus e leucoaraiose

Abstract Background Seizures after stroke can negatively affect the prognosis of ischemic stroke and cause a decrease in quality of life. The efficacy of intravenous (IV) recombinant tissue plasminogen activator (rt-PA) treatment in acute ischemic stroke has been demonstrated in many studies, and IV rt-PA treatment has been increasingly used around the world. The SeLECT score is a useful score for the prediction of late seizures after stroke and includes the severity of stroke (Se), large artery atherosclerosis (L), early seizure (E), cortical involvement (C), and the territory of the middle cerebral artery (T). However, the specificity and sensitivity of the SeLECTscore have not been studied in acute ischemic stroke patients that received IV rt-PA treatment. Objective In the present study, we aimed to validate and develop the SeLECT score in acute ischemic stroke patients receiving IV rt-PA treatment. Methods The present study included 157 patients who received IV thrombolytic treatment in our third-stage hospital. The 1-year seizure rates of the patients were detected. SeLECT scores were calculated. Results In our study, we found that the SeLECT score had low sensitivity but high specificity for predicting the likelihood of late seizure after stroke in patients administered IV rt-PA therapy. In addition to the SeLECT score, we found that the specificity and sensitivity were higher when we evaluated diabetes mellitus (DM) and leukoaraiosis. Conclusion We found that DM was an independent risk factor for late seizures after stroke in a patient group receiving thrombolytic therapy, and late seizures after stroke were less frequent in patients with leukoaraiosis.

Resumo Antecedentes As convulsões após o AVC podem afetar negativamente o prognóstico do AVC isquêmico e causar uma diminuição na qualidade de vida. A eficácia do tratamento com ativador do plasminogênio tecidual recombinante (rt-PA) intravenoso (IV) no AVC isquêmico agudo foi demonstrada em muitos estudos, e o tratamento com rt-PA IV tem sido cada vez mais usado em todo o mundo. A pontuação SeLECT é uma pontuação útil para a previsão de convulsões tardias após AVC e inclui a gravidade do AVC (Se), aterosclerose de grandes artérias (L), convulsão precoce (E), envolvimento cortical (C) e o território do meio artéria cerebral (T). No entanto, a especificidade e a sensibilidade do escore SeLECT não foram estudadas em pacientes com AVC isquêmico agudo que receberam tratamento IV com rt-PA. Objetivo No presente estudo, objetivamos validar e desenvolver o escore SeLECT em pacientes com AVC isquêmico agudo recebendo tratamento IV com rt-PA. Métodos O presente estudo incluiu 157 pacientes que receberam tratamento trombolítico IV em nosso hospital de terceiro estágio. As taxas de convulsão de 1 ano dos pacientes foram detectadas. Os escores SeLECT foram calculados. Resultados Em nosso estudo, descobrimos que o escore SeLECT apresentou baixa sensibilidade, mas alta especificidade para prever a probabilidade de convulsão tardia após AVC em pacientes que receberam terapia IV com rt-PA. Além do escore SeLECT, descobrimos que a especificidade e a sensibilidade foram maiores quando avaliamos diabetes mellitus (DM) e leucoaraiose. Conclusão Descobrimos que DM foi um fator de risco independente para convulsões tardias após AVC em um grupo de pacientes recebendo terapia trombolítica, e convulsões tardias após AVC foram menos frequentes em pacientes com leucoaraiose.

Vertebral artery hypoplasia as an independent risk factor of posterior circulation atherosclerosis and ischemic stroke.

ABSTRACT: Vertebral artery hypoplasia (VAH) is a frequent anatomical variation of vertebral arteries, with emerging evidence suggesting that it contributes to posterior circulation ischemia. However, the relationship between VAH and ischemic stroke remains unknown. Hence, this study aimed to determine the prevalence of VAH in patients diagnosed with acute ischemic stroke who were followed up in a neurology clinic and to determine if it can potentially be a risk factor for atherosclerotic stenosis in vertebrobasilar circulation.This retrospective study included 609 patients diagnosed with acute ischemic stroke between January 1, 2019 and January 1, 2020. Demographic of patients, risk factors, radiological and clinical characteristics were evaluated.Posterior circulation was very common in patients with VAH, and the most common locations of atherosclerotic stenosis were V1 and V4 segments of the vertebral artery and the middle segment of basilar artery. Analysis of the risk factors for atherosclerotic stenosis in patients with posterior circulation acute ischemic stroke suggested that VAH was an independent risk factor.Findings of the study suggest that VAH pre-disposes atherosclerotic stenosis in vertebrobasilar circulation, although its mechanism remains unknown. Hemodynamic parameters associated with atherosclerosis could not be measured in vivo. Thus, to better understand the underlying mechanism, conducting studies that examine blood flow parameters with high-resolution magnetic resonance angiography in patients diagnosed with acute cerebral ischemia patients with VAH is warranted.

Headache as the sole presenting symptom of cerebral venous sinuses thrombosis: Subgroup analysis of data from the VENOST study.

OBJECTIVES: Headache is the most common complaint in cerebral venous sinus thrombosis (CVST) and it may sometimes be the only symptom in these patients. This retrospective and prospective study was an investigation of any differences in terms of clinical risk factors, radiological findings, or prognosis in patients with CVST who presented with isolated headache (IH) and cases with other concomitant findings (non-isolated headache [NIH]). METHODS: A total of 1144 patients from a multicenter study of cerebral venous sinus thrombosis (VENOST study) were enrolled in this research. The demographic, biochemical, clinical, and radiological aspects of 287 IH cases and 857 NIH cases were compared. RESULTS: There were twice as many women as men in the study group. In the IH group, when gender distribution was evaluated by age group, no statistically significant difference was found. The onset of headache was frequently subacute and chronic in the IH group, but an acute onset was more common in the NIH group. Other neurological findings were observed in 29% of the IH group during follow-up. A previous history of deep, cerebral, or other venous thromboembolism was less common in the IH group than in the NIH group. Transverse sinus involvement was greater in the IH group, whereas sagittal sinus involvement was greater in the NIH group. The presence of a plasminogen activator inhibitor (PAI) mutation was significantly greater in the IH group. CONCLUSION: IH and CVST should be kept in mind if a patient has subacute or chronic headache. PAI, which has an important role in thrombolytic events, may be a risk factor in CVST. Detailed hematological investigations should be considered. Additional studies are needed.

Evaluation of the Clinical Features Accompanied by the Gene Mutations: The 2 Novel PSEN1 Variants in a Turkish Early-onset Alzheimer Disease Cohort.

INTRODUCTION: Early-onset Alzheimer disease (EOAD) is an earlier Alzheimer disease form which is characterized by the mutations in the amyloid precursor protein, presenilin-1/2 (PSEN1/2), and triggering receptor expressed on myeloid cells 2 (TREM2). However, it is still necessary to report mutational screening in multiethnic groups to improve the genetic background of EOAD due to the variant classification challenge. METHODS: We performed targeted sequencing for the amyloid precursor protein, PSEN1, PSEN2, and TREM2 genes in 74 patients and 1 family diagnosed with EOAD. RESULTS: Among the detected variants, 8 were coding and 6 were noncoding in 15 of 74 patients. In PSEN1, 2 pathogenic coding variants (T274K and L364P) detected in 2 patients were novel and 3 coding variants (G183V, E318G, and L219P) detected in 2 patients were previously reported. We found 4 patients with the compound heterozygosity for the PSEN2 A23= and N43= and a family with the coexistence of them, and 1 patient with TREM2 Y38C. The coding variation frequency was 12.1%. In silico analysis indicated pathogenic potentials and clinical interpretations of the detected variants. CONCLUSION: Our study reveals the rare gene variants including novel ones from the Turkish EOAD cohort and provides to clinicians the list of detected variants in the screened genes, which may also be useful for accurate genetic counseling.

Assessment of Patients with Intracerebral Hemorrhage or Hemorrhagic Transformation in the VENOST Study.

INTRODUCTION: Cerebral venous and sinus thrombosis (CVST) may lead to cerebral edema and increased intracranial pressure; besides, ischemic or hemorrhagic lesions may develop. Intracerebral hemorrhages occur in approximately one-third of CVST patients. We assessed and compared the findings of the cerebral hemorrhage (CH) group and the CVST group. MATERIALS AND METHODS: In the VENOST study, medical records of 1,193 patients with CVST, aged over 18 years, were obtained from 35 national stroke centers. Demographic characteristics, clinical symptoms, signs at the admission, radiological findings, etiologic factors, acute and maintenance treatment, and outcome results were reported. The number of involved sinuses or veins, localizations of thrombus, and lesions on CT and MRI scans were recorded. RESULTS: CH was detected in the brain imaging of 241 (21.1%) patients, as hemorrhagic infarction in 198 patients and intracerebral hemorrhage in 43 patients. Gynecologic causes comprised the largest percentage (41.7%) of etiology and risk factors in the CVST group. In the CH group, headache associated with other neurological symptoms was more frequent. These neurological symptoms were epileptic seizures (46.9%), nausea and/or vomiting (36.5%), altered consciousness (36.5%), and focal neurological deficits (33.6%). mRS was ≥3 in 23.1% of the patients in the CH group. DISCUSSION AND CONCLUSION: CVST, an important cause of stroke in the young, should be monitored closely if the patients have additional symptoms of headache, multiple sinus involvement, and CH. Older age and parenchymal lesion, either hemorrhagic infarction or intracerebral hemorrhage, imply poor outcome.

Cerebral Venous Sinus Thrombosis in Women: Subgroup Analysis of the VENOST Study.

BACKGROUND: Early diagnosis of cerebral venous sinus thrombosis (CVST) associated with reproductive health-related risk factors (RHRF) including pregnancy, puerperium, and oral contraceptive (OC) use can prevent severe neurological sequelae; thus, the symptoms must be documented in detail for each group. METHODS: Out of 1144 patients with CVST, a total of 777 women were enrolled from a multicenter for the study of cerebral venous sinus thrombosis (VENOST). Demographic, biochemical, clinical, and radiological aspects were compared for 324 cases with RHRF and 453 cases without RHRF. RESULTS: The mean age of the RHRF (-) group (43.2 ± 13 years) was significantly higher than of the RHRF (+) group (34 ± 9 years). A previous history of deep venous thrombosis (3%), isolated cavernous sinus involvement (1%), cranial neuropathy (13%), comorbid malignancy (7%), and its disability scores after 12 months (9%) were significantly higher in the RHRF (-) group. The RHRF (+) group consisted of 44% cases of puerperium, 33% cases of OC users and 23% of pregnant women. The mean age was found to be higher in OC users (38 ± 9 years). A previous history of deep venous thrombosis was slightly higher in the pregnancy subgroup (4%). Epileptic seizures were more common in the puerperium group (44%). CONCLUSION: The results of our study indicate that the risk of CSVT increases parallel to age, OC use, and puerperium period. In addition, when considering the frequency of findings and symptoms, epileptic seizures in the puerperium subgroup of the RHRF (+) group and malignancies in the RHRF (-) group may accompany the CSVT. In daily practice, predicting these risks for the CSVT and early recognition of the symptoms will provide significant benefits to patients.

Epileptic seizures in cerebral venous sinus thrombosis: Subgroup analysis of VENOST study.

PURPOSE: The aim of this study is to evaluate the presence and prognostic impact of early seizures in cerebral venous sinus thrombosis patients (CVST). METHOD: VENOST is a retrospective and prospective national multicenter observational study. CVST patients with or without epileptic seizures (ES) were analyzed and compared in terms of demographic and imaging data, causative factors, clinical variables, and prognosis in a total of 1126 patients. RESULTS: The mean age of the patients in the ES group was 39.73 ± 12.64 and 40.17 ± 14.02 years in the non-ES group (p > 0.05). Epileptic seizures were more common (76.6 %) in females (p < 0.001). Early ES occurred in 269 of 1126 patients (23.9 %). Epileptic seizures mainly presented in the acute phase (71.4 %) of the disease (p < 0.001). Majority of these (60.5 %) were in the first 24 h of the CVST. The most common neurological signs were focal neurologic deficits (29.9 %) and altered consciousness (31.4 %) in the ES group. Superior sagittal sinus (SSS) and cortical veins (CV) involvement were the most common sites of thrombosis and the mostly related etiology were found puerperium in seizure group (30.3 % vs 13.9 %). Patients with seizures had worse outcome in the first month of the disease (p < 0.001) but these did not have any influence thereafter. CONCLUSIONS: In this largest CVST cohort (VENOST) reported female sex, presence of focal neurological deficits and altered consciousness, thrombosis of the SSS and CVs, hemorrhagic infarction were risk factors for ES occurrence in patients with CVST.

Cerebral Venous Sinus Thrombosis as a Rare Complication of Systemic Lupus Erythematosus: Subgroup Analysis of the VENOST Study.

AIM: Systemic lupus erythematosus (SLE) is an unusual risk factor for cerebral venous sinus thrombosis (CVST). As few CVST patients with SLE have been reported, little is known regarding its frequency as an underlying etiology, clinical characteristics, or long-term outcome. We evaluated a large cohort of CVST patients with SLE in a multicenter study of cerebral venous thrombosis, the VENOST study, and their clinical characteristics. MATERIAL AND METHOD: Among the 1144 CVST patients in the VENOST cohort, patients diagnosed with SLE were studied. Their demographic and clinical characteristics, etiological risk factors, venous involvement status, and outcomes were recorded. RESULTS: In total, 15 (1.31%) of 1144 CVST patients had SLE. The mean age of these patients was 39.9 ± 12.1 years and 13 (86.7%) were female. Presenting symptoms included headache (73.3%), visual field defects (40.0%), and altered consciousness (26.7%). The main sinuses involved were the transverse (60.0%), sagittal (40.0%), and sigmoid (20.0%) sinuses. Parenchymal involvement was not seen in 73.3% of the patients. On the modified Rankin scale, 92.9% of the patients scored 0-1 at the 1-month follow-up and 90.9% scored 0-1 at the 1-year follow-up. CONCLUSIONS: SLE was found in 1.31% of the CVST patients, most frequently in young women. Headache was the most common symptom and the CVST onset was chronic in the majority of cases. The patient outcomes were favorable. CVST should be suspected in SLE patients, even in those with isolated chronic headache symptoms with or without other neurological findings.

Good outcome of the single-center pediatric kidney transplant program in Abu Dhabi.

Renal transplantation is the treatment of choice for ESRD in children. It is associated with better quality of life, growth of children, and improved long-term survival. The aim of the study was to evaluate the outcomes of pediatric renal transplantation at a tertiary care center in UAE. A retrospective chart review was undertaken for all the pediatric renal transplants performed at Sheikh Khalifa Medical City, Abu Dhabi, UAE, over the past 9 years. The study evaluated the demographic data, outcomes, and complications of pediatric renal transplantation. The post-transplantation outcomes including surgical complications, documented infections, graft rejection, graft and patient survival, effect on growth, and eGFR were reviewed. Between 2010 and 2018, 30 pediatric patients underwent renal transplantation. The follow-up period ranged from 1 to 9 years with a mean of 3.3 years. The mean age of the patients at the time of transplant was 9.8 years, and 56.7% were males. Prior to the transplantation, the majority of the recipients were on peritoneal dialysis (70.0%). Main source of renal donation at our center was from LRD, chiefly from parents. Patient survival at 1 and 5 years was 100% and 96.7%, respectively. Graft survival at 1 and 5 years was 96.7% and 83.3%, respectively. During the 9-year follow-up period, 5 (16.7%) recipients experienced rejection episode. This study demonstrates that during 5-year period, pediatric kidney transplantation program has achieved optimal patient (96.7%) and graft (83.3%) survival rates and is comparable to well-established centers.

Behçet's disease as a causative factor of cerebral venous sinus thrombosis: subgroup analysis of data from the VENOST study.

OBJECTIVE: This study was performed to determine the rate of cerebral venous sinus thrombosis (CVST) among cases of Behçet's disease (BD) included in a multicentre study of cerebral venous sinus thrombosis (VENOST). METHODS: VENOST was a retrospective and prospective national multicentre observational study that included 1144 patients with CVST. The patients were classified according to aetiologic factors, time of CVST symptom onset, sinus involvement, treatment approach and prognosis. RESULTS: BD was shown to be a causative factor of CVST in 108 (9.4%) of 1144 patients. The mean age of patients in the BD group was 35.27 years and 68.5% were men, whereas in the non-BD CVST group, the mean age was 40.57 years and 28.3% were men (P < 0.001). Among the aetiologic factors for patients aged 18-36 years, BD was predominant for men, and puerperium was predominant for women. The onset of symptoms in the BD group was consistent with the subacute form. The transverse sinuses were the most common sites of thrombosis, followed by the superior sagittal sinuses. The most common symptom was headache (96.2%), followed by visual field defects (38%). CONCLUSIONS: BD was found in 9.4% of patients in our VENOST series. Patients with BD were younger and showed a male predominance. The functional outcome of CVST in patients with BD was good; only 12% of patients presenting with cranial nerve involvement and altered consciousness at the beginning had a poor outcome (modified Rankin Score ⩾2).

Comparison of Brain Atrophy, Cognition and Optical Coherence Tomography Results Between Multiple Sclerosis Patients and Healthy Controls.

INTRODUCTION: Cognitive impairment is also an important cause of disability in MS in addition to motor, sensory, visual, and cerebellar affections. The aim of this study is to show the relation between the cognitive disability in MS with brain atrophy and retinal nerve fiber layer (RNFL). METHODS: Forty-three multiple sclerosis (MS) patients, and 15 healthy individuals as controls were included in the study. MS patients were divided into three groups as relapsing-remitting MS (RRMS), relapsing-remitting with optic neuritis (RRMS+ON), and secondary-progressive MS (SPMS). An experienced psychologist performed modified Wechsler Memory Scale Revised form (WMS-R), Lines Orientation test, Stroop Color Word Interference test (STROOP), Standard Raven Progressive Matrices (SRPM), Benton Facial Recognition Test, verbal fluency test, and Paced Auditory Serial Addition tests in all cases. Optic coherence tomographies (OCT) were done. Cranial subcortical volumes of all subjects were measured using 3-dimensonal T1A imagines obtained by the cranial subcortical 1.5 tesla MR device (fully automatic Freesurfer program). Brain parenchymal fractions were calculated by proportioning the obtained volume measurements to the total intracranial volume. RESULTS: Fifty-eight subjects (65.5% female, 34.5% male) were enrolled in the study. There were significant differences among the groups in terms of parenchymal thickness, volumes of third ventricle, and white matter. There was a significant correlation between the volumes of the deep gray matter, mesial temporal structures and lateral ventricular volumes, and the test results of the WMS-R. OCT scores of all MS patients, whether or not they experienced optic neuritis, had increased, being worse especially in the SPMS group. Correlation between RNFL and the brain parenchymal fractions of the patients were statistically significant. CONCLUSION: Manual methods instead of automatic segmentation method are being more commonly used in the studies with brain atrophy and MS in our country. A significant correlation between OCT scores and brain atrophy is shown with our present study, and this is followed as a reflection of decrease in cognitive tests that provides valuable and reliable knowledge for the literature.

Caregiver Burden in Different Stages of Alzheimer's Disease.

With an increasing number of patients being diagnosed with Alzheimer's disease (AD) daily, it has become one of the major problems in public health. The increase in the number of dementia patients in low- and middle-income countries is expected to be much more than that in developed countries. As a result, the economic burden of dementia, both worldwide and in Turkey, is growing. Moreover, AD leads to emotional burdens and psychological distress in family member(s) and caregiver(s) alongside the patient. Each stage of AD imposes different responsibilities on caregivers, increasing their burden. The suffering and emotional burdens of caregivers from these responsibilities lead to a decreased quality of life and disturbed body physiology. Incapacity, despair, weariness, and loneliness are the hidden emotions of this iceberg. This review aims to gather the results of studies on caregiver burden in different stages of AD, attract attention to those results that may have been ignored in Turkey, and shed light on the solutions required to overcome the problems in caregiving of AD patients.

Paroxysmal Amnesia Attacks due to Hashimoto's Encephalopathy.

Hashimoto's encephalopathy is a rare disease which is thought to be autoimmune and steroid responsive. The syndrome is characterized by cognitive impairment, encephalopathy, psychiatric symptoms, and seizures associated with increased level of anti-thyroid antibodies. The exact pathophysiology underlying cerebral involvement is still lesser known. Although symptoms suggest a nonlesional encephalopathy in most of the cases, sometimes the clinical appearance can be subtle and may not respond to immunosuppressants or immunomodulatory agents. Here we report a case who presented with drowsiness and amnestic complaints associated with paroxysmal electroencephalography (EEG) abnormalities which could be treated only with an antiepileptic drug.

Impaired cognitive performance and hippocampal atrophy in Parkinson disease.

BACKGROUND/AIM: Dementia is common in Parkinson disease (PD). Since magnetic resonance imaging has been used, hippocampal atrophy has been shown in PD patients with or without dementia. In this study we sought the correlation of cognitive decline with bilateral hippocampal volume in PD patients. MATERIALS AND METHODS: Thirty-three patients with diagnosis of idiopathic PD and 16 healthy subjects were included in this study. PD patients were divided into two groups as normal cognitive function and mild cognitive impairment (MCI). The Mini-Mental State Examination and detailed cognitive assessment tests were performed for all patients for cognitive analyses. Depression was excluded by the Geriatric Depression Scale. RESULTS: The mean onset age of disease was 55 years for PD patients without dementia and 59 for PD patients with MCI. According to the Hoehn-Yahr scales, 24% of patients had grade 1, 58% had grade 2, and 18% had grade 3 disease. Right and left hippocampal volumes decreased along with cognitive test scores in PD patients. Increased right hippocampal volume was correlated with forward number test in the MCI-PD group. CONCLUSION: These findings suggest that memory deficit is associated with hippocampal atrophy in PD patients.

Sporadic Creutzfeldt-Jacob Disease: An 8-Year Experience from a Single Center in Turkey.

INTRODUCTION: Our aim was to conduct a retrospective review to demonstrate the clinical, radiological, and electrophysiological features of patients with sporadic Creutzfeldt-Jacob disease (sCJD). METHOD: A total of 10 patients (5 female and 5 male, with a mean age of 45 years from a range of 40 to 67 years) out of 8.259 adult patients hospitalized from January 2000 to December 2008 were diagnosed with sCJD. RESULTS: Eight of the patients were diagnosed on the basis of clinical, radiological, electroencephalography (EEG), and cerebrospinal fluid (CSF) findings. Two other patients also had a pathological diagnosis. The most common signs and symptoms were behavioral disturbances, movement disorders, cognitive decline, myoclonus, psychosis, focal neurological deficit, and aphasia. Nine of the patients had periodic sharp wave discharges on EEG. Seven patients were positive for the 14.3.3 protein in the CSF. Five patients had pulvinar signs-a bilateral increased signal in the pulvinar thalami-on cranial magnetic resonance imaging. Eight patients were diagnosed with probable sCJD; two were diagnosed with definite sCJD. All of the patients died as a result of the disease within 24 months after the onset of symptoms. DISCUSSION: sCJD should be considered in the diagnosis of patients who present with rapidly progressive dementia. Clinical and radiological data appear to be sufficient for the diagnosis. However, detailed molecular examinations of the subtypes of the disease may be required for early diagnosis of cases given the wide spectra of CJD.

Primary gait ignition disorder: report of three cases.

Gait ignition failure (GIF) classifications all had major limitations. Few years ago, a new and simpler classification was proposed by Liston. The aim of this paper is to discuss three GIF patients with respect to this new classification. All three patients presented with hesitation to start walking and turning and their neurological examination revealed start and turn hesitation without any other abnormality. We classified our patients according to Liston's classification as ignition apraxia, which enabled us to approach the patients in a practical way. This classification helps to understand the underlying pathologies and combines clinical characteristics and pathophysiology. We reported our experience with pergolide in the treatment of patients suffering from primary GIF and underline the fact that more research is needed on the treatment of this condition.