Atypical clinical and pathological findings in a patient with isolated cortical vein thrombosis

Isolated cortical vein thrombosis often produces a focal lesion. Because of the rapid development of collateral circulation, increased intracranial pressure has never been reported in a patient with isolated cortical vein thrombosis. The diagnosis of isolated cortical vein thrombosis is based mainly on MRI, catheter digital subtraction angiography, and histological findings, but may be challenging. We report a patient who presented with intermittent seizures and left-sided limb weakness. Her symptoms gradually progressed, and she eventually developed signs of increased intracranial pressure. Imaging studies showed a space-occupying lesion in the right frontal lobe of the brain. As we could not diagnose isolated cortical vein thrombosis based on the preoperative findings, surgical excision of the lesion was performed under general anesthesia. Histological examination showed destruction of the brain parenchyma with infiltration of macrophages, proliferation of reactive astrocytes and small vessels, and foci of hemorrhage. Further examination found that a number of small vessels in both the subarachnoid space and brain parenchyma were filled with thrombus, some of which was organized. Elastic fiber staining showed that the obstructed vessels were veins. We diagnosed isolated cortical vein thrombosis with atypical clinical features.

Advances in longitudinal studies of amnestic mild cognitive impairment and Alzheimer's disease based on multi-modal MRI techniques

Amnestic mild cognitive impairment （aMCI） is a prodromal stage of Alzheimer＇s disease （AD）, and 75%-80% of aMCI patients finally develop AD. So, early identification of patients with aMCI or AD is of great significance for prevention and intervention. According to cross-sectional studies, it is known that the hippocampus, posterior cingulate cortex, and corpus callosum are key areas in studies based on structural MRI （sMRI）, functional MRI （fMRI）, and diffusion tensor imaging （DTI） respectively. Recently, longitudinal studies using each MRI modality have demonstrated that the neuroimaging abnormalities generally involve the posterior brain regions at the very beginning and then gradually affect the anterior areas during the progression of aMCI to AD. However, it is not known whether follow-up studies based on multi-modal neuroimaging techniques （e.g., sMRI, fMRI, and DTI） can help build effective MRI models that can be directly applied to the screening and diagnosis of aMCI and AD. Thus, in the future, large-scale multi-center follow-up studies are urgently needed, not only to build an MRI diagnostic model that can be used on a single person, but also to evaluate the variability and stability of the model in the general population. In this review, we present longitudinal studies using each MRI modality separately, and then discuss the future directions in this field.

Awareness of secondary stroke prevention guidelines among neurologists in China： a cross-sectional online survey

Background Recurrent stroke following a transient ischemic attack or minor stroke is frequent but potentially preventable. The prevention outcome is, by and large, dependent on how well physicians understand and implement prevention guidelines. Yet, little is known about the scope of physicians＇ awareness and implementation of secondary stroke prevention guidelines in the mainland of China. This study aimed to identify the gaps between physicians＇ clinical practice and guidelines for secondary stroke prevention in China. Methods A nation-wide online survey with a 16-item questionnaire that reflects major components of the Chinese National Secondary Stroke Prevention Guidelines was performed between September 1, 2009 and October 31, 2009. Results A total of 3489 neurologists from third tier hospitals completed the survey. The percentage of the physicians who were aware of the control targets and prevention measures recommended in the Chinese national guidelines was generally lower than reported for developed countries. Conclusions Physicians in the mainland of China lack sufficient awareness of secondary stroke prevention guidelines. Continuing education is highly recommended.

Coexistence of High Fibrinogen and Low High-density Lipoprotein Cholesterol Levels Predicts Recurrent Cerebral Venous Thrombosis

Background： Cerebral venous thrombosis （CVT） may lead to serious neurological disorders; however, little is known about the risk factors for recurrent CVT. Our aim was to determine the association between elevated fibrinogen and decreased high-density lipoprotein cholesterol （HDL-C） levels with recurrent CVT. Methods： This retrospective cohort study included participants if they had a first episode of objectively defined CVT and were admitted to Xuan Wu Hospital, Capital Medical University from August 2005 to September 2009. Demographic and clinical variables were collected, as well as laboratory parameters, inchiding plasma fibrinogen and HDL-C. Patients with CVT were tbllowed for recurrent symptomatic CVT. Follow-up was through the end of September 2010. Potential predictors of recurrence were analyzed using Cox survival analysis. Results： At tile end of the lbllow-up, 95 patients were eligible lbr the study. Twelve of 95 patients （12.6%） had recurred CVT. Tile median time of recurrence was 7 months （range： 1-39 months）. Eight of these 12 （66.7%） experienced rectirrence within the first 12 months alter their initial CVT. The recurrence rate of CVT was 2.76 per 100 patient-years. Multivariate Cox regression analysis demonstrated that the coexistence of high fibrinogen （〉4.00 g/L） and low HDL-C （〈1.08 mmol/L） levels at baseline was the only independent predictor for recurrent CVT （hazard ratio： 4.69; 95% confidence interval： 1.10-20,11; P 〈 0.05）. Of tile twelve patients with recurrent CVT in our study, 7 （58.3%） had high fibrinogen plus low HDL-C levels. All 7 of these patients took warfarin for 3-12 months, and 6 of 7 had rectirrent CVT after the discontinuation of anticoagulant treatment. Conclusions： Concomitant high fibrinogen and low HDL-C levels may be associated with recurrence of CVT. The ett＇ect of potential risk lhctors related to atherothrombosis on rectinent CVT should be closely monitored.

An Overview of Genome-Wide Association Studies in Alzheimer＇s Disease

Genome-wide association studies（GWASs）have revealed a plethora of putative susceptibility genes for Alzheimer＇s disease（AD）. With the sole exception of the APOE gene, these AD susceptibility genes have not been unequivocally validated in independent studies. No single novel functional risk genetic variant has been identified. In this review, we evaluate recent GWASs of AD, and discuss their significance, limitations, and challenges in the investigation of the genetic spectrum of AD.

Impaired Parahippocampal Gyrus-Orbitofrontal Cortex Circuit Associated with Visuospatial Memory Deficit as a Potential Biomarker and Interventional Approach for Alzheimer Disease

The parahippocampal gyrus-orbitofrontal cortex(PHG-OFC)circuit in humans is homologous to the postrhinal cortex(POR)-ventral lateral orbitofrontal cortex(vlOFC)circuit in rodents.Both are associated with visuospatial malfunctions in Alzheimer’s disease(AD).However,the underlying mechanisms remain to be elucidated.In this study,we explored the relationship between an impaired POR-vlOFC circuit and visuospatial memory deficits through retrograde tracing and in vivo local field potential recordings in 5XFAD mice,and investigated alterations of the PHG-OFC circuit by multi-domain magnetic resonance imaging(MRI)in patients on the AD spectrum.We demonstrated that an impaired glutamatergic POR-vlOFC circuit resulted in deficient visuospatial memory in 5XFAD mice.Moreover,MRI measurements of the PHG-OFC circuit had an accuracy of 77.33%for the classification of amnestic mild cognitive impairment converters versus non-converters.Thus,the PHG-OFC circuit explains the neuroanatomical basis of visuospatial memory deficits in AD,thereby providing a potential predictor for AD progression and a promising interventional approach for AD.

Early Neurological Deterioration after Recanalization Treatment in Patients with Acute Ischemic Stroke： A Retrospective Study

Background： Early neurological deterioration （END） is a prominent issue after recanalization treatment. However. few studies have reported the characteristics of END after endovascular treatment （EVT） as so far. This study investigated the incidence, composition, and outcomes of END after intravenous recombinant tissue plasminogen activator （IV rt-PA） and EVT of acute ischemic stroke, and identified risk factors for END. Methods： Medical records of patients who received recanalization treatment between January l, 2014, and December 31, 2015 were reviewed. Patients were classified into IV rt-PA or EVT group according to the methods ofrecanalization treatment. The END was defined as an increase in the National Institutes of Health Stroke Scale （N1HSS） ≥4 or an increase in la of NIHSS ≥I within 72 h after recanalization treatment. Clinical data were compared between the END and non-END subgroups within each recanalization group. Results： Of the 278 patients included in the study, the incidence of END was 34.2%. The incidence rates of END were 29.8% in the IV rt-PA group and 40.2% in the EVT group, lschemia progression （68.4%） was the main contributor to END followed by vasogenic cerebral edema （21. 1%） and symptomatic intracranial hemorrhage （10.5%）. Multivariate logistic regression showed that admission systolic blood pressure （SBP） ≥160 mmHg （odds ratio [OR]： 2.312, 95% confidence interval [CI]： 1.105-4.837） and large artery occlusion after IV rt-PA （OR： 3.628.95% （7： 1.482-8.881 ） independently predicted END after IV rt-PA; and admission SBP 〉 140 mmHg （OR： 5.183, 95% CI：1.967 13.661 ）, partial recanalization （OR： 4.791,95% CI： 1.749-13.121 ）, and nonrecanalization （OR： 5.952, 95% CI： 1.841-19.243） independently predicted END alter EVT. The mortality rate and grave outcome rate at discharge of all the END patients （26.3% and 55.8%） were higher than those of all the non-END patients （1.1% and 18.6%： P 〈 0.01）. Conclusions： END was not an uncommon event and associated with death and grave outcome at discharge. High admission SBP and unsatisfactory recanalization of occluded arteries might predict END.

Dysfunctional autophagy in Alzheimer's disease: pathogenic roles and therapeutic implications

Neuronal autophagy is essential for neuronal survival and the maintenance of neuronal homeostasis. Increasing evidence has implicated autophagic dysfunction in the pathogenesis of Alzheimer＇s disease （AD）. The mechanisms underlying autophagic failure in AD involve several steps, from autophagosome formation to degradation. The effect of modulating autophagy is context-dependent. Stimulation of autophagy is not always beneficial. During the implementation of therapies that modulate autophagy, the nature of the autophagic defect, the timing of intervention, and the optimal level and duration of modulation should be fully considered.

Manic-depressive Psychosis as the Initial Symptom in Adult Siblings with Late-onset Combined Methylmalonic Aciduria and Homocystinemia, Cobalamin C Type

Methylmalonic aciduria （MMA） is an autosomal recessive disorder of cobalamin （cbl） metabolism. Cobalamin C （cblC） disease is the most common type of MMA and is characteristically concurrent with homocystinemia （HCY） due to impaired synthesis of two active forms of cbl, namely adenosylcobalamin （AdoCbl） and methylcobalamin （MeCbl）. The estimated worldwide incidence of MMA ranges between 1：48,000 and 1：250,000. Mutations of the MMA and HCY type C protein （MMACtfC） gene are responsible for cblC disease and were first identified by Lerner-Ellis et aL in 2006.By the year 2016, more than 82 different MMACHC gene mutations have been reported （http：// www.hgmd.cf.ac.uk/ac/index.php）. Among these mutations, c.609G〉A （p.W203X） was reported to be the most frequent cblC mutation in Chinese patients.

Dementia studies in Chinese populations

Variations in the prevalence of dementia in different ethnic groups have been reported worldwide, and a number of reviews have provided a picture of epidemiological studies in dementia research. However, little is known about epidemiological studies in Chinese populations. In this review, we searched PubMed and the Web of Science for original research articles published in English up to July 2013 on the prevalence, incidence, risk factors, and prognosis of dementia in Chinese populations worldwide. Except for the prevalence, we included only population-based follow-up studies. We identified 25 studies in elderly Chinese residents in China's Mainland, Hong Kong, Taiwan, and Singapore, and found a higher prevalence of dementia in China's Mainland than in the other locations, which may be due to that the studies from China's Mainland are more recent than those from other locations. A notable increase in incidence was observed when dementia cases were diagnosed using 10/66 diagnostic criteria compared to other criteria. Studies on risk factors for dementia were limited and mostly from China's Mainland. Age, gender, education, smoking, and alcohol consumption were related to the risk of dementia in Chinese populations. Only two prognostic studies were identified, and age, gender, and residential area were related to the prognosis of dementia. In conclusion, the prevalence, incidence, and risk factors for dementia found in Chinese populations were comparable to other ethnic groups, but no conclusive results on prognosis were found. The differences in prevalence and incidence were influenced by the diagnostic criteria and the time of study. Longitudinal population-based studies on the incidence, risk factors, and prognosis of dementia in Chinese populations are required.

Management Recommendations on Sleep Disturbance o Patients with Parkinson's Disease

INTRODUCTION Sleep disturbance is one of the most common nonmotor symptoms in Parkinson's disease (PD).Sleep disturbance affects 40-98% of PD patients in the world. In China, the prevalence of PD patients with sleep disturbance ranges from 47.66% to 89.10%. Sleep disturbance usually has adverse impact on the quality of life of PD patients. Apossible pathogenesis of PD with sleep disturbance include thalamocortical pathway degeneration and changes of neurotransmitter systems. The etiology of sleep disturbance is multifactorial,involving degeneration of areas regulating sleep,sleep structure affected by drugs,sleep disturbance induced by drug,and sleep fragmentation by multiple factors.

Intracranial hypotension and co-existent normal-pressure glaucoma： the Beijing intracranial and intraocular pressure study

n contrast to the so-called intraocular pressure, which is .the transcomeal pressure difference, it is thetrans-lamina cribrosa pressure difference, which is of importance for physiology and pathophysiology of the optic nerve head. The trans-lamina cribrosa pressure difference is the difference between the pressure in the intraocular compartment （i.e., the so-called intraocular pressure） minus the retrobulbar pressure, which is formed by the orbital cerebrospinal fluid （CSF）I pressure and the retrobulbar optic nerve tissue pressure. This anatomical fact has been the basis for the speculation that some patients with so-called normal intraocular pressure glaucoma have an abnormally low orbital CSF pressure. Previous clinical studies have supported this hypothesis.2＇3 The purpose of this study is to report on a patient with low CSF pressure and co-existent open-angle glaucoma with normal intraocular pressure.

Expert Consensus on Clinical Diagnostic Criteria for Fatal Familial Insomnia

INTRODUCTION Fatal familial insomnia （FFI） is a serious and rare prion disease, which was first reported by Lugaresi et al. in 1986.Early diagnosis of FFI might be important for early and sufficient counseling of patients and their relatives, also concerning the risk of inheritance, and potentially also for treatment studies. However, the diagnosis of FFI might be difficult because of the heterogeneity of clinical features, low sensitivity of diagnostic tests, and absence of family history. The aim of the present study was to develop a clinical scheme and diagnostic criteria for FFI based on our research and expert consensus.

Sex differences in Alzheimer’s disease

Recently,Koran et al.^([1])published an article,named“Sex differences in the association between Alzheimer’s disease(AD)biomarkers and cognitive decline”in Brain Imaging and Behavior.The result proved that there were sex-specific associations between biomarkers of AD.This article added evidence to the theory of sex differences in AD.Sex difference is a common phenomenon in AD and manifests in many ways.Females are disproportionately affected by AD.