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    3448 results match your criteria Brain : a journal of neurology[Journal]

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    Contribution of EEG in transient neurological deficits.
    J Neurol 2017 Nov 15. Epub 2017 Nov 15.
    Service de Physiologie Clinique-Explorations Fonctionnelles, AP-HP, Hôpital Lariboisière, 2 rue Ambroise Paré, 75010, Paris, France.
    Identification of stroke mimics and 'chameleons' among transient neurological deficits (TND) is critical. Diagnostic workup consists of a brain imaging study, for a vascular disease or a brain tumour and EEG, for epileptiform discharges. The precise role of EEG in this diagnostic workup has, however, never been clearly delineated. Read More

    Serum neuron specific enolase may be a marker to predict the severity and outcome of cerebral venous thrombosis.
    J Neurol 2017 Nov 11. Epub 2017 Nov 11.
    Departments of Neurology, Neurosurgery, Ophthalmology and Emergency, Xuanwu Hospital, Capital Medical University, Beijing Institute for Brain Disorders, Beijing, 100053, China.
    The objective is to explore the effective of baseline serum neuron specific enolase (NSE) on predicting the severity and outcome in patients with cerebral venous thrombosis (CVT). A total of 156 patients confirmed as CVT in Xuanwu Hospital were enrolled in this retrospective study from March 2011 through September 2016. The severity was evaluated with the National Institutes of Health Stroke Score (NIHSS), intracranial pressure (ICP), and CVT-related complications; the outcome was evaluated by modified Rankin Scale (mRS); the relationship between baseline serum NSE and mRS was analyzed with receiver operating characteristic curve (ROC), logistic regression analysis, and Kaplan-Meier curves. Read More

    Tracking posttraumatic hemianopia.
    J Neurol 2017 Nov 6. Epub 2017 Nov 6.
    Department of Neurosurgery, University Hospitals Leuven, Herestraat 49, 3000, Leuven, Belgium.
    Hemianopia after traumatic brain injury is not infrequent and results from retro-chiasmatic lesions. Differentiating optic pathway lesions can be challenging with classic imaging. Advanced imaging techniques as an investigational tool for posttraumatic hemianopia are discussed and their pitfalls highlighted through an illustrative case study. Read More

    Clinical characteristics of myelin oligodendrocyte glycoprotein seropositive optic neuritis: a cohort study in Shanghai, China.
    J Neurol 2017 Nov 3. Epub 2017 Nov 3.
    Department of Ophthalmology, Eye Ear Nose and Throat Hospital of Fudan University, 83 Fenyang Road, Shanghai, 200031, China.
    Qualitative and quantitative visual outcomes of Asian optic neuritis (ON) patients with seropositive myelin oligodendrocyte glycoprotein (MOG) antibodies remain unknown. We retrospectively evaluated a cohort of new-onset ON patients in Shanghai, China between January 2015 and December 2016. Serum MOG and aquaporin-4 (AQP4) antibodies were detected using cell-based assays, and patients were followed-up for at least 6 months. Read More

    Low intrathecal antibody production despite high seroprevalence of Epstein-Barr virus in multiple sclerosis: a review of the literature.
    J Neurol 2017 Nov 2. Epub 2017 Nov 2.
    Molecular Neuroimmunology Group, Department of Neurology, University of Heidelberg, Heidelberg, Germany.
    Background: Patients with multiple sclerosis (MS) frequently have an intrathecal production of antibodies to different common viruses, which can be detected by elevated antiviral antibody indices (AIs). There is a strong and consistent association of MS and Epstein-Barr virus (EBV) infection.

    Objective: To systematically compare the frequencies of intrathecal antibody production to EBV, measles virus, rubella virus, varicella zoster virus (VZV) and herpes simplex virus (HSV) in patients with MS. Read More

    Neurological update: gliomas and other primary brain tumours in adults.
    J Neurol 2017 Nov 2. Epub 2017 Nov 2.
    Division of Neuropathology, National Hospital for Neurology and Neurosurgery, University College London NHS Foundation Trust, Queen Square, London, WC1N 3BG, UK.
    The emerging understanding of molecular changes in a wide range of brain tumours has led to a significant shift in how these tumours are diagnosed, managed and treated. This article will provide a hands-on overview of the relevant biomarkers and their association with newly defined biological tumour entities. Read More

    An open-label study to assess the feasibility and tolerability of rilmenidine for the treatment of Huntington's disease.
    J Neurol 2017 Dec 26;264(12):2457-2463. Epub 2017 Oct 26.
    Addenbrooke's Hospital, Hills Road, Cambridge, CB21 2QQ, UK.
    Preclinical data have shown that rilmenidine can regulate autophagy in models of Huntington's disease (HD), providing a potential route to alter the disease course in patients. Consequently, a 2-year open-label study examining the tolerability and feasibility of rilmenidine in mild-moderate HD was undertaken. 18 non-demented patients with mild to moderate HD took daily doses of 1 mg Rilmenidine for 6 months and 2 mg for a further 18 months followed by a 3-month washout period. Read More

    Efficacy of fingolimod and interferon beta-1b on cognitive, MRI, and clinical outcomes in relapsing-remitting multiple sclerosis: an 18-month, open-label, rater-blinded, randomised, multicentre study (the GOLDEN study).
    J Neurol 2017 Dec 23;264(12):2436-2449. Epub 2017 Oct 23.
    Department of Neurology, Institute of Experimental Neurology, San Raffaele Hospital, Milan, Italy.
    Cognitive impairment (CI) affects 40-65% of multiple sclerosis (MS) patients. This study attempted evaluating the effects of fingolimod and interferon beta-1b (IFN β-1b) on CI progression, magnetic resonance imaging (MRI) and clinical outcomes in relapsing-remitting MS (RRMS) patients over 18 months. The GOLDEN study was a pilot study including RRMS patients with CI randomised (2:1) to fingolimod (0. Read More

    Clinical spectrum and IgG subclass analysis of anti-myelin oligodendrocyte glycoprotein antibody-associated syndromes: a multicenter study.
    J Neurol 2017 Dec 23;264(12):2420-2430. Epub 2017 Oct 23.
    Department of Neuroscience, Biomedicine and Movement Sciences, Neurology Unit, University of Verona, Verona, Italy.
    Anti-myelin oligodendrocyte glycoprotein antibodies (MOG-Ab) recently emerged as a potential biomarker in patients with inflammatory demyelinating diseases of the central nervous system. We here compare the clinical and laboratory findings observed in a cohort of MOG-Ab seropositive and seronegative cases and describe IgG subclass analysis results. Consecutive serum samples referred to Verona University Neuropathology Laboratory for aquaporin-4 (AQP4)-Ab and/or MOG-Ab testing were analysed between March 2014 and May 2017. Read More

    Prevalence and clinical characteristics of cortical superficial siderosis in patients with acute stroke.
    J Neurol 2017 Dec 23;264(12):2413-2419. Epub 2017 Oct 23.
    Department of Neurological Science, Graduate School of Medicine, Nippon Medical School, 1-1-5 Sendagi, Bunkyo-ku, Tokyo, 113-8603, Japan.
    Cortical superficial siderosis (cSS) is a pathologic and radiologic diagnosis of hemosiderin deposition in subpial brain layers. However, cSS has not been fully studied in patients with acute stroke. Here, we investigated the prevalence of cSS in patients with acute stroke and analyzed the relationship between cSS and different clinical and neuroimaging characteristics. Read More

    Nutritional habits, risk, and progression of Parkinson disease.
    J Neurol 2017 Oct 10. Epub 2017 Oct 10.
    Department of Neuroscience, Biomedicine and Movement Science, University of Verona, Piazzale L.A. Scuro 10, 37134, Verona, Italy.
    Parkinson disease (PD) is a multifactorial disease, where a genetic predisposition combines with putative environmental risk factors. Mounting evidence suggests that the initial PD pathological manifestations may be located in the gut to subsequently affect brain areas. Moreover, several lines of research demonstrated that there are bidirectional connections between the central nervous system and the gut, the "gut-brain axis" that influences both brain and gastrointestinal function. Read More

    Prognosis of patients with primary malignant brain tumors admitted to the intensive care unit: a two-decade experience.
    J Neurol 2017 Nov 9;264(11):2303-2312. Epub 2017 Oct 9.
    Neurophysiologie Respiratoire Expérimentale et Clinique, Sorbonne Universités, UPMC Univ Paris 06, INSERM, UMRS_1158, Paris, France.
    The purpose of this study is to describe the reasons for ICU admission and to evaluate the outcome and prognostic factors of patients with primary malignant brain tumors (PMBT) admitted to the intensive care unit (ICU). This is a retrospective observational cohort study of 196 PMBT patients admitted to two ICUs over a 19-year period. Acute respiratory failure was the main reason for ICU admission (45%) followed by seizures (25%) and non-epileptic coma (14%). Read More

    Absence of clinical cerebellar syndrome after serial injections of more than 20 doses of gadoterate, a macrocyclic GBCA: a monocenter retrospective study.
    J Neurol 2017 Nov 27;264(11):2277-2283. Epub 2017 Sep 27.
    FNRS, ULB-Hôpital Erasme, Route de Lennik 808, 1070, Brussels, Belgium.
    Sound evidence of gadolinium accumulation in brain has been recently provided after repeated administrations of linear gadolinium-based contrast agents (GBCAs), especially at the cerebellum level. Although data regarding brain accumulation of macrocyclic GBCAs are more reassuring, there is now a genuine concern ("gadolinium-phobia") about possible long-term consequences of gadolinium deposits, especially in terms of cerebellar sequelae. We, therefore, questioned about the clinical impact of serial administration of gadoterate meglumine, a macrocyclic GBCA. Read More

    Hypothalamic dysfunction is related to sleep impairment and CSF biomarkers in Alzheimer's disease.
    J Neurol 2017 Nov 12;264(11):2215-2223. Epub 2017 Sep 12.
    Sleep Medicine Centre, Neurophysiopathology Unit, Department of Systems Medicine, University of Rome "Tor Vergata", Viale Oxford 81, 00133, Rome, Italy.
    Hypothalamus is a key brain region regulating several essential homeostatic functions, including the sleep-wake cycle. Alzheimer's disease (AD) pathology affects nuclei controlling sleep-wake rhythm sited in this brain area. Since only post-mortem studies documented the relationship between hypothalamic atrophy and sleep-wake cycle impairment, we investigated in AD patients the possible hypothalamic in vivo alteration using 2-deoxy-2-(18F) fluoro-D-glucose ([18F]FDG) positron emission tomography ([18F]FDG PET), and its correlations with sleep impairment and cerebrospinal-fluid (CSF) AD biomarkers (tau proteins and β-amyloid42). Read More

    Characterization of brain tumours with spin-spin relaxation: pilot case study reveals unique T 2 distribution profiles of glioblastoma, oligodendroglioma and meningioma.
    J Neurol 2017 Nov 11;264(11):2205-2214. Epub 2017 Sep 11.
    Radiology, University of British Columbia, Vancouver, Canada.
    Prolonged spin-spin relaxation times in tumour tissue have been observed since some of the earliest nuclear magnetic resonance investigations of the brain. Over the last three decades, numerous studies have sought to characterize tumour morphology and malignancy using quantitative assessment of T 2 relaxation times, although attempts to categorize and differentiate tumours have had limited success. However, previous work must be interpreted with caution as relaxation data were typically acquired using a variety of multiple echo sequences with a range of echoes and T 2 decay curves and were frequently fit with monoexponential analysis. Read More

    Thalamic transitory ischemic attacks presenting as Jacksonian sensory march.
    J Neurol 2017 Oct 11;264(10):2158-2161. Epub 2017 Sep 11.
    Department of Neurology and Epileptology, Hertie Institute for Clinical Brain Research, Eberhard-Karls University Tübingen, Hoppe-Seyler Str. 3, 72076, Tübingen, Germany.
    Spreading somatosensory symptoms appearing as Jacksonian sensory march are usually considered to be due to an epileptic seizure. We report on three cases in which these symptoms were caused by thalamic ischemia. Two patients presented with stereotypically recurring hemiparesthesias lasting 2-5 min that gradually spread from the face to the arm and leg on one side. Read More

    'Timed up and go' and brain atrophy: a preliminary MRI study to assess functional mobility performance in multiple sclerosis.
    J Neurol 2017 Nov 11;264(11):2201-2204. Epub 2017 Sep 11.
    Multiple Sclerosis Center, Department of Medical Sciences and Public Health, Binaghi Hospital, University of Cagliari, Via Is Guadazzonis 2, 09126, Cagliari, Italy.
    Motor and cognitive disabilities are related to brain atrophy in multiple sclerosis (MS). 'Timed up and go' (TUG) has been recently tested in MS as functional mobility test, as it is able to evaluate ambulation/coordination-related tasks, as well as cognitive function related to mobility. The objective of this study is to evaluate the relationship between brain volumes and TUG performances. Read More

    Parkinsonian symptoms in normal pressure hydrocephalus: a population-based study.
    J Neurol 2017 Oct 6;264(10):2141-2148. Epub 2017 Sep 6.
    Department of Pharmacology and Clinical Neuroscience, Umeå University, Umeå, Sweden.
    It may be challenging to differentiate normal pressure hydrocephalus (NPH) from neurodegenerative disorders such as Parkinson's disease. In this population-based study, we wanted to describe the frequency of parkinsonian symptoms among individuals with and without NPH, and whether the motor examination part of the Unified Parkinson's Disease Rating Scale (UPDRS-m) score differs between these groups. Furthermore, we wanted to find out whether there was a relationship between UPDRS-m score, NPH symptoms, and radiological signs of NPH. Read More

    Vestibulo-ocular reflex deficits with medial longitudinal fasciculus lesions.
    J Neurol 2017 Oct 6;264(10):2119-2129. Epub 2017 Sep 6.
    Institute of Clinical Neuroscience, Royal Prince Alfred Hospital, Missenden Road, Sydney, NSW 2050, Australia.
    The medial longitudinal fasciculus (MLF) is the final common pathway for all conjugate adducting horizontal eye movements, as well as for the vertical-torsional vestibulo-ocular reflex (VOR). MLF lesion causes adduction paresis of ipsilesional (adducting) eye with dissociated nystagmus of contralesional (abducting) eye-the well-known clinical syndrome of internuclear ophthalmoplegia (INO). We measured the VOR stimulation and also any catch-up saccades, from individual semicircular canal (SCC) evoked by the head impulse test (HIT), using head and binocular 3-dimensional scleral search coils in 27 multiple sclerosis (MS) patients, 8 with unilateral, 19 with bilateral INO. Read More

    Late-onset Pompe disease: a genetic-radiological correlation on cerebral vascular anomalies.
    J Neurol 2017 Oct 30;264(10):2110-2118. Epub 2017 Aug 30.
    Department of Molecular Medicine, University of Pavia and Fondazione IRCCS Policlinico S. Matteo, Pavia, Italy.
    Pompe disease is an autosomal recessive disorder in which deficiency of the lysosomal enzyme acid alpha-glucosidase results in the accumulation of glycogen mostly in muscle tissues. Several reports suggest a higher incidence of intracranial vascular abnormalities (IVAs) in this condition, as well as brain microbleeds and cerebral vasculopathy. The aim of our study was to evaluate through neuroimaging studies the incidence of these anomalies in our cohort of late-onset Pompe disease (LOPD) patients asymptomatic for cerebrovascular disease, looking for correlations with clinical and genetic data. Read More

    A specific pattern of gray matter atrophy in Alzheimer's disease with depression.
    J Neurol 2017 Oct 30;264(10):2101-2109. Epub 2017 Aug 30.
    Department of Medical Physics, Medical School, Democritus University of Thrace, Alexandroupolis, Greece.
    Considering the high incidence of depressive symptoms in Alzheimer's disease (AD), we conducted a large-sample study to investigate the pattern of gray matter (GM) abnormalities that differentiates depressive from non-depressive AD patients. We included 201 AD patients who underwent MRI assessment and categorized them into depressive and non-depressive subgroups based on the Geriatric Depression Scale (GDS; cut-off score: ≤9). We performed whole-brain voxel-based morphometry analysis in 173 patients after MRI quality control and used between-group comparisons and regression analysis models to analyze the volumetric data controlling for nuisance variables. Read More

    The role of N-terminal pro-brain natriuretic peptide in evaluating the prognosis of patients with intracerebral hemorrhage.
    J Neurol 2017 Oct 24;264(10):2081-2087. Epub 2017 Aug 24.
    Department of Neurosurgery, Guangdong 999 Brain Hospital, Guangzhou City, Guangdong Province, China.
    A prognostic biomarker that can provide a good prediction of prognosis in patients with intracerebral hemorrhage (ICH) would be beneficial in guiding the initial management decisions in the setting of ICH. N-terminal pro-brain natriuretic peptide (NT-proBNP) is a biomarker of prognosis in patients with cardiovascular disease and ischemic stroke. However, the prognostic role of NT-proBNP in patients with spontaneous ICH is still a controversial issue. Read More

    Astrocyte and Alzheimer's disease.
    J Neurol 2017 Oct 18;264(10):2068-2074. Epub 2017 Aug 18.
    Guangdong Key Laboratory of Age-Related Cardiac and Cerebral Diseases, Affiliated Hospital of Guangdong Medical University, Zhanjiang, People's Republic of China.
    The past several decades have given rise to more insights into the role of astrocytes in normal brain function and diseases. Astrocytes elicit an effect which may be neuroprotective or deleterious in the process of Alzheimer's disease (AD). Impairments in astrocytes and their other functions, as well as physiological reactions of astrocytes to external injury, can trigger or exacerbate hyperphosphorylated tau and amyloid-beta (Aβ) pathologies, leading to the formation of both amyloid plaques and neurofibrillary tangles (NFTs), as well as neuronal dysfunction. Read More

    Simultaneous EEG-PET-fMRI measurements in disorders of consciousness: an exploratory study on diagnosis and prognosis.
    J Neurol 2017 Sep 17;264(9):1986-1995. Epub 2017 Aug 17.
    Neurologische Klinik und Poliklinik, Klinikum rechts der Isar der Technischen Universität München, 81675, Munich, Germany.
    Previous studies could demonstrate that functional magnetic resonance imaging (fMRI), fludeoxyglucose positron emission tomography (FDG-PET), and electroencephalography (EEG) measures contain information about patients suffering from disorders of consciousness (DOC) and thus improve the clinical diagnosis. Additionally, the technical modalities were able to predict the outcome of patients. However, most studies lack proven reproducibility in a clinical setting. Read More

    Non-CNS pathogenic origin of Parkinson's disease.
    J Neurol 2017 Sep 14;264(9):2027-2030. Epub 2017 Aug 14.
    Professor Emeritus, Department of Psychology, Rosalind Franklin University of Medicine and Science, 3333 Green Bay Rd, North Chicago, IL, USA.
    The gut with its variety of microbiota may serve as an etiological origin of diseases. Gut microbes may also play a role in the pathogenesis of diseases beyond their simple nutritional maintenance and support. For example, gut protein aggregation, possibly aided by microbes as well as nasal influences, might be linked to disease that may move to the brain through the vagus nerve. Read More

    High prevalence of neuronal surface autoantibodies associated with cognitive deficits in cancer patients.
    J Neurol 2017 Sep 7;264(9):1968-1977. Epub 2017 Aug 7.
    Department of Neurology, Charité - Universitätsmedizin Berlin, Charitéplatz 1, 10117, Berlin, Germany.
    The recent discovery of neuronal cell-surface antibodies profoundly expanded the clinical spectrum of paraneoplastic neurological syndromes. Many of these syndromes are associated with impaired cognitive function, a clinical symptom that is of increasing concern in cancer patients. However, the frequency of these antibodies in cancer patients and their relation to clinical syndromes is currently unknown. Read More

    Severity of traumatic brain injury correlates with long-term cardiovascular autonomic dysfunction.
    J Neurol 2017 Sep 2;264(9):1956-1967. Epub 2017 Aug 2.
    Department of Neurology, Friedrich-Alexander-Universität Erlangen-Nürnberg (FAU), Erlangen, Germany.
    After traumatic brain injury (TBI), central autonomic dysfunction might contribute to long-term increased mortality rates. Central autonomic dysfunction might depend on initial trauma severity. This study was performed to evaluate differences in autonomic modulation at rest and upon standing between patients with a history of mild TBI (post-mild-TBI patients), moderate or severe TBI (post-moderate-severe-TBI patients), and healthy controls. Read More

    Regional microstructural damage and patterns of eye movement impairment: a DTI and video-oculography study in neurodegenerative parkinsonian syndromes.
    J Neurol 2017 Sep 31;264(9):1919-1928. Epub 2017 Jul 31.
    Department of Neurology, University of Ulm, Oberer Eselsberg 45, 89081, Ulm, Germany.
    Characteristic alterations of eye movement control are a common feature of neurodegenerative parkinsonism, including Parkinson's disease (PD), progressive supranuclear palsy (PSP), and multiple system atrophy (MSA). Regional microstructural alterations as assessed by diffusion tensor imaging (DTI) have been reported in PD, PSP, and MSA. Therefore, we investigated the specific association between eye movement disturbances and microstructural impairment in these diseases. Read More

    Early trigeminal nerve involvement in Listeria monocytogenes rhombencephalitis: case series and systematic review.
    J Neurol 2017 Sep 20;264(9):1875-1884. Epub 2017 Jul 20.
    Department of Neurology, Rigshospitalet, Copenhagen University Hospital, Blegdamsvej 9, 2100, Copenhagen, Denmark.
    Listeria monocytogenes is associated with rhombencephalitis. However, the exact mechanisms of brainstem invasion remains poorly understood. Here, we demonstrate clinical and radiological data suggesting that Listeria may invade the brainstem via the trigeminal nerve. Read More

    Structural connectivity differences in essential tremor with and without resting tremor.
    J Neurol 2017 Sep 20;264(9):1865-1874. Epub 2017 Jul 20.
    Institute of Molecular Bioimaging and Physiology (IBFM-CNR), National Research Council, Catanzaro, Italy.
    In this work, we investigated motor network structure in patients affected by essential tremor (ET) with or without resting tremor, using probabilistic tractography of the cerebello-thalamo-basal ganglia-cortical loop. Twenty-five patients with ET, twenty-two patients with ET associated with resting tremor (rET), and twenty-five age- and sex-matched healthy controls were included in the study. All participants underwent whole-brain 3D T1-weighted and diffusion-weighted MRI, and DAT-SPECT. Read More

    Disparities in epilepsy surgery in the United States of America.
    J Neurol 2017 Aug 12;264(8):1735-1745. Epub 2017 Jul 12.
    Division of Epilepsy and Clinical Neurophysiology, Department of Neurology, Boston Children's Hospital, Harvard Medical School, Fegan 9, 300 Longwood Avenue, Boston, MA, 02115, USA.
    The aim is to describe the epidemiology of epilepsy surgery in children and adults in the United States. We performed a descriptive study of the National Inpatient Sample (NIS) for the year 2012 and the Kids' Inpatient Database (KID) for the period 2010-2012, the largest all-payer databases on inpatient data in the USA. These databases estimate 97% of all inpatient hospital discharges in the USA. Read More

    Acute disseminated encephalomyelitis: prognostic value of early follow-up brain MRI.
    J Neurol 2017 Aug 10;264(8):1754-1762. Epub 2017 Jul 10.
    Department of Neurology, Massachusetts General Hospital, 165 Cambridge Street, #627, Boston, MA, 02114, USA.
    Patients with acute disseminated encephalomyelitis (ADEM) are presumed to have radiological monophasic disease, but this is uncertain since follow-up brain MRI is not routinely performed. We aimed to ascertain combined radiological and clinical monophasic disease in ADEM patients and to assess whether performing early (<6 months) follow-up brain MRI has prognostic value for subsequent multiphasic disease. We retrospectively studied the medical records of patients initially diagnosed with ADEM (years 2000-2014) at the Massachusetts General Hospital, USA. Read More

    MRI volumetric morphometry in vascular parkinsonism.
    J Neurol 2017 Jul 1;264(7):1511-1519. Epub 2017 Jul 1.
    Department of Neuroradiology, Montpellier University Hospital Center, Gui de Chauliac Hospital, Montpellier, France.
    Vascular parkinsonism is a difficult clinical differential diagnosis in elderly subjects. We aimed at identifying morphometric markers in the brain of elderly patients with vascular parkinsonism (VP) compared with age-matched patients with Parkinson's disease (PD) and healthy controls. In this multicenter prospective study, 46 patients (80 ± 5 years old; male 32) with parkinsonism (32 PD and 14 VP) and 29 controls (mean age 78 ± 3 years; male 21) underwent brain MRI on a 3-T scanner including T1 MPRAGE and FLAIR sequences. Read More

    Higher blood-brain barrier permeability is associated with higher white matter hyperintensities burden.
    J Neurol 2017 Jul 26;264(7):1474-1481. Epub 2017 Jun 26.
    Department of Neurology, Beijing Chao-Yang Hospital, Capital Medical University, No. 8, South Gongti Road, Beijing, 100020, People's Republic of China.
    The pathogenesis of white matter hyperintensities (WMH) is incompletely understood but blood-brain barrier (BBB) dysfunction may play a key role. This study aimed to investigate the relationship between BBB permeability and the severity of WMH burden. Consecutive participants without symptomatic stroke history presented for physical examination were recruited in this cross-sectional study and divided into three WMH burden groups according to total Fazekas scores. Read More

    Lateralized microstructural changes in early-stage Parkinson's disease in anterior olfactory structures, but not in substantia nigra.
    J Neurol 2017 Jul 26;264(7):1497-1505. Epub 2017 Jun 26.
    Department of Psychiatry, Dalhousie University, 4064 AJLB, 5909 Veterans Memorial Lane, Halifax, NS, Canada.
    Parkinson's disease (PD) is a progressive neurological disorder characterized by motor symptoms as well as severe deficits in olfactory function and microstructural changes in olfactory brain regions. Because of the evidence of asymmetric neuropathological features in early-stage PD, we examined whether lateralized microstructural changes occur in olfactory brain regions and the substantia nigra in a group of early-stage PD patients. Using diffusion tensor imaging (DTI) and the University of Pennsylvania Smell Identification Test (UPSIT), we assessed 24 early-stage PD patients (Hoehn and Yahr stage 1 or 2) and 26 healthy controls (HC). Read More

    Optimizing the deep brain stimulation care pathway in patients with Parkinson's disease.
    J Neurol 2017 Jul 19;264(7):1454-1464. Epub 2017 Jun 19.
    Hospices Civils de Lyon, Hôpital Neurologique Pierre Wertheimer, Neurologie C, Bron, France.
    Management of Parkinson's disease (PD) using deep brain stimulation (DBS) requires complex care in specialized, multidisciplinary centers. A well-organized, efficient patient flow is crucial to ensure that eligible patients can quickly access DBS. Delays or inefficiencies in patient care may impact a center's ability to meet demand, creating a capacity bottleneck. Read More

    A novel gain-of-function mutation in the ITPR1 suppressor domain causes spinocerebellar ataxia with altered Ca(2+) signal patterns.
    J Neurol 2017 Jul 15;264(7):1444-1453. Epub 2017 Jun 15.
    Clinical Genetics, Temple Street Children's University Hospital, Dublin 1, Ireland.
    We report three affected members, a mother and her two children, of a non-consanguineous Irish family who presented with a suspected autosomal dominant spinocerebellar ataxia characterized by early motor delay, poor coordination, gait ataxia, and dysarthria. Whole exome sequencing identified a novel missense variant (c.106C>T; p. Read More

    Assessment of the factorial validity and reliability of the ALSFRS-R: a revision of its measurement model.
    J Neurol 2017 Jul 12;264(7):1413-1420. Epub 2017 Jun 12.
    Department of Neurology, Brain Centre Rudolf Magnus, University Medical Centre Utrecht, Heidelberglaan 100, 3584 CX, Utrecht, The Netherlands.
    The amyotrophic lateral sclerosis functional rating scale-revised (ALSFRS-R) is a widely used primary outcome measure in amyotrophic lateral sclerosis (ALS) clinical practice and clinical trials. ALSFRS-R items cannot, however, validly be summed to obtain a total score, but constitute domain scores reflecting a profile of disease severity. Currently, there are different measurement models for estimating domain scores. Read More

    Relevance of early cervical cord volume loss in the disease evolution of clinically isolated syndrome and early multiple sclerosis: a 2-year follow-up study.
    J Neurol 2017 Jul 9;264(7):1402-1412. Epub 2017 Jun 9.
    Department of Diagnostic and Interventional Radiology and Nuclear Medicine, St. Josef Hospital, Ruhr-University Bochum, Gudrunstr. 56, 44791, Bochum, Germany.
    Upper cervical cord area (UCCA) atrophy is a prognostic marker for clinical progression in longstanding multiple sclerosis (MS). The objectives of the study were to quantify UCCA atrophy and evaluate its impact in clinically isolated syndrome (CIS) and relapsing-remitting MS (RRMS); to compare converting CIS patients with stable CIS, and to study changes of UCCA and brain white matter (WM) and grey matter (GM) at 2-year follow-up. 110 therapy-naive patients including 53 CIS [6 ± 6 months after symptom onset (SO)] and 57 early RRMS (SO: 12 ± 9 months) underwent sagittal 3D-T1w brain MR (3T). Read More

    Understanding Lennox-Gastaut syndrome: insights from focal epilepsy patients with Lennox-Gastaut features.
    J Neurol 2017 Jul 5;264(7):1388-1396. Epub 2017 Jun 5.
    Epileptology Unit, AP-HP, GH Pitie-Salpêtrière-Charles Foix, 75013, Paris, France.
    To delineate the clinical and EEG features of adults with focal epilepsy associated with a generalized paroxysmal fast activity (GPFA) pattern on EEG who developed refractory seizures, notably drop attacks, but do not fulfill the classical triad for the diagnosis of Lennox-Gastaut syndrome (LGS) and provide further insight into LGS mechanisms. Among 957 patients admitted to video-EEG monitoring between 2002 and 2015, we retrospectively research adult patients with refractory focal epilepsy, drop attacks and GPFA on EEG. We collected demographic, anamnestic, and clinical data from medical records. Read More

    HIV-1-associated neurocognitive disorder: epidemiology, pathogenesis, diagnosis, and treatment.
    J Neurol 2017 Aug 31;264(8):1715-1727. Epub 2017 May 31.
    HIV-Schwerpunktpraxis, 30890, Barsinghausen, Germany.
    The modern antiretroviral treatment of human immunodeficiency virus (HIV-1) infection has considerably lowered the incidence of opportunistic infections. With the exception of the most severe dementia manifestations, the incidence and prevalence of HIV-associated neurocognitive disorders (HAND) have not decreased, and HAND continues to be relevant in daily clinical practice. Now, HAND occurs in earlier stages of HIV infection, and the clinical course differs from that before the widespread use of combination antiretroviral treatment (cART). Read More

    Olfactory impairment in Parkinson's disease is a consequence of central nervous system decline.
    J Neurol 2017 Jun 26;264(6):1236-1246. Epub 2017 May 26.
    Department of Otorhinolaryngology, Interdisciplinary Center "Smell and Taste", TU Dresden, Fetscherstr. 74, 01307, Dresden, Germany.
    Early diagnosis and timely treatment of Parkinson's disease are essential factors to provide these patients with a longer period of a better quality of life. Olfactory loss is among the first non-motor symptoms of the disease; however, in light of the many causes of smell loss, it is a very unspecific biomarker and should only be used as part of a diagnostic test battery. In this study, we investigated the olfactory response in 71 subjects, consisting of Parkinson's disease patients, hyposmic and anosmic patients of other causes, and normosmic individuals searching for sensitive, distinct biomarkers for which we used scalp event-related 64-channel electroencephalography and psychophysical tests. Read More

    Progressive multifocal leukoencephalopathy in patients treated with fumaric acid esters: a review of 19 cases.
    J Neurol 2017 Jun 23;264(6):1155-1164. Epub 2017 May 23.
    Laboratory of Medical Microbiology and Immunology, St. Elisabeth Hospital Tilburg, Tilburg, The Netherlands.
    Progressive multifocal leukoencephalopathy (PML) is a rare and potentially fatal condition caused by a brain infection with JC polyomavirus (JCV). PML develops almost exclusively in immunocompromised patients and has recently been associated with use of fumaric acid esters (FAEs), or fumarates. We reviewed the literature and the Dutch and European pharmacovigilance databases in order to identify all available FAE-associated PML cases and distinguish possible common features among these patients. Read More

    Screening for lipoprotein receptor-related protein 4-, agrin-, and titin-antibodies and exploring the autoimmune spectrum in myasthenia gravis.
    J Neurol 2017 Jun 17;264(6):1193-1203. Epub 2017 May 17.
    Department of Neurology, RWTH Aachen University, Aachen, Germany.
    In autoimmune myasthenia gravis (MG), the identification of antibodies and characterization of serological subgroups is of great importance for diagnosis and management of the disease. Our aims were to study the frequency of antibodies against lipoprotein-related protein 4 (LRP4), agrin, and titin using the most recent techniques, and to characterize corresponding clinical features and autoimmune diseases (AID) in 100 MG-patients. The antibody frequencies in the 55 AChR-antibody positive patients were 7% LRP4, 5% agrin, 53% titin, and in the 45 AChR-antibody negative patients 2% MuSK, 2% LRP4, 2% agrin, and 27% titin. Read More

    Use of EEG in critically ill children and neonates in the United States of America.
    J Neurol 2017 Jun 13;264(6):1165-1173. Epub 2017 May 13.
    Division of Epilepsy and Clinical Neurophysiology, Department of Neurology, Boston Children's Hospital, Harvard Medical School, Boston, MA, USA.
    The objective of the study was to estimate the proportion of patients who receive an electroencephalogram (EEG) among five common indications for EEG monitoring in the intensive care unit: traumatic brain injury (TBI), extracorporeal membrane oxygenation (ECMO), cardiac arrest, cardiac surgery and hypoxic-ischemic encephalopathy (HIE). We performed a retrospective cross-sectional descriptive study utilizing the Kids' Inpatient Database (KID) for the years 2010-2012. The KID is the largest pediatric inpatient database in the USA and it is based on discharge reports created by hospitals for billing purposes. Read More

    Deciphering the causes of sporadic late-onset cerebellar ataxias: a prospective study with implications for diagnostic work.
    J Neurol 2017 Jun 6;264(6):1118-1126. Epub 2017 May 6.
    Service de Neurologie, Hôpitaux Universitaires de Strasbourg, Hôpital de Hautepierre, 1 avenue Molière, Strasbourg, 67098, Cedex, France.
    The management of sporadic late-onset cerebellar ataxias represents a very heterogeneous group of patients and remains a challenge for neurologist in clinical practice. We aimed at describing the different causes of sporadic late-onset cerebellar ataxias that were diagnosed following standardized, exhaustive investigations and the population characteristics according to the aetiologies as well as at evaluating the relevance of these investigations. All patients consecutively referred to our centre due to sporadic, progressive cerebellar ataxia occurring after 40 years of age were included in the prospective, observational study. Read More

    Clinical assessment of dysphagia in neurodegeneration (CADN): development, validity and reliability of a bedside tool for dysphagia assessment.
    J Neurol 2017 Jun 3;264(6):1107-1117. Epub 2017 May 3.
    Department of Neurodegeneration, Hertie Institute for Clinical Brain Research and Center for Neurology, University Hospital Tübingen, Tübingen, Germany.
    Screening assessments for dysphagia are essential in neurodegenerative disease. Yet there are no purpose-built tools to quantify swallowing deficits at bedside or in clinical trials. A quantifiable, brief, easy to administer assessment that measures the impact of dysphagia and predicts the presence or absence of aspiration is needed. Read More

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