3,685 results match your criteria Brain : a journal of neurology[Journal]


Pathologic and MRI analysis in acute atypical inflammatory demyelinating lesions.

J Neurol 2019 Apr 23. Epub 2019 Apr 23.

Department of Radiology, Strasbourg University Hospital, Strasbourg, France.

Background: The diagnosis of atypical inflammatory demyelinating lesions can be difficult. Brain biopsy is often required to exclude neoplasms. Moreover, the relationship between these lesions and multiple sclerosis and NMOSD is not clear. Read More

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http://dx.doi.org/10.1007/s00415-019-09328-7DOI Listing

Structural correlates of attention dysfunction in Lewy body dementia and Alzheimer's disease: an ex-Gaussian analysis.

J Neurol 2019 Apr 21. Epub 2019 Apr 21.

Institute of Neuroscience, Newcastle University, Biomedical Research Building 3rd Floor, Campus for Ageing and Vitality, Newcastle upon Tyne, NE4 5PL, UK.

Background: Lewy body dementia (LBD) and Alzheimer's disease (AD) are common forms of degenerative dementia. While they are characterized by different clinical profiles, attentional deficits are a common feature. The objective of this study was to investigate how attentional problems in LBD and AD differentially affect different aspects of reaction time performance and to identify possible structural neural correlates. Read More

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http://dx.doi.org/10.1007/s00415-019-09323-yDOI Listing

Exploring hyperhidrosis and related thermoregulatory symptoms as a possible clinical identifier for the dysautonomic subtype of Parkinson's disease.

J Neurol 2019 Apr 17. Epub 2019 Apr 17.

King's College London, Institute of Psychiatry, Psychology and Neuroscience, Department of Basic and Clinical Neurosciences, De Crespigny Park, London, SE5 8AF, UK.

Objective: To identify associated (non-)motor profiles of Parkinson's disease (PD) patients with hyperhidrosis as a dominant problem.

Methods: This is a cross-sectional, exploratory, analysis of participants enrolled in the Non-motor Longitudinal International Study (NILS; UKCRN No: 10084) at the Parkinson's Centre at King's College Hospital (London, UK). Hyperhidrosis scores (yes/no) on question 28 of the Non-Motor Symptom Questionnaire were used to classify patients with normal sweat function (n = 172) and excessive sweating (n = 56) (Analysis 1; n = 228). Read More

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http://dx.doi.org/10.1007/s00415-019-09325-wDOI Listing
April 2019
1 Read

Is Parkinson's disease a chronic low-grade inflammatory bowel disease?

J Neurol 2019 Apr 12. Epub 2019 Apr 12.

Inserm, U1235, 1 rue Gaston Veil, 44035, Nantes, France.

While the pathogenesis of Parkinson's disease is not fully understood, there is increasing evidence that inflammatory responses in the brain are implicated in both disease initiation and progression. The inflammatory process in Parkinson's disease is, however, not limited to the brain but also involves the gastrointestinal tract. High amounts of cytokines and inflammatory markers are found in the colon of Parkinson's disease patients and there is now strong epidemiological and genetical evidence linking Parkinson's disease to inflammatory bowel diseases. Read More

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http://dx.doi.org/10.1007/s00415-019-09321-0DOI Listing
April 2019
2 Reads

Hereditary neuropathy with liability to pressure palsies.

J Neurol 2019 Apr 15. Epub 2019 Apr 15.

Unit of Rare Neurodegenerative and Neurometabolic Diseases, Department of Clinical Neurosciences, Fondazione IRCCS Istituto Neurologico Carlo Besta, Milan, Italy.

Hereditary neuropathy with liability to pressure palsies (HNPP) is characterized by recurrent sensory and motor neuropathy in individual nerves starting in adolescence or young adulthood, focal conduction abnormalities at entrapment sites on nerve conduction studies, and sausage-like swellings (tomacula) of the myelin sheaths by nerve biopsy. It is characterized genetically by the deletion of the chromosome 17p11.2-p12 region including the peripheral myelin protein-22 gene in the overwhelming majority of cases. Read More

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http://dx.doi.org/10.1007/s00415-019-09319-8DOI Listing

Occurrence of cerebral small vessel disease at diagnosis of MPO-ANCA-associated vasculitis.

J Neurol 2019 Apr 13. Epub 2019 Apr 13.

Division of Neurology, Department of Internal Medicine IV, Osaka Medical College, 2-7 Daigaku-machi, Takatsuki, Osaka, 569-8686, Japan.

Background: Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) often causes peripheral nervous system impairments. However, little is known about subclinical involvements of the central nervous system in AAV. We investigated the frequency and progression of cerebral small vessel disease (SVD) in patients with AAV. Read More

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http://dx.doi.org/10.1007/s00415-019-09318-9DOI Listing

Musical hallucinations and their relation with epilepsy.

J Neurol 2019 Apr 10. Epub 2019 Apr 10.

Parnassia Psychiatric Institute, The Hague, The Netherlands.

Musical hallucinations are poorly understood phenomena. Their relation with epilepsy was first described over a century ago, but never systematically explored. We, therefore, reviewed the literature, and assessed all descriptions of musical hallucinations attributed to epileptic activity. Read More

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http://dx.doi.org/10.1007/s00415-019-09289-xDOI Listing

Amyloid PETs are commonly negative in suspected Alzheimer's disease with an increase in CSF phosphorylated-tau protein concentration but an Aβ42 concentration in the very high range: a prospective study.

J Neurol 2019 Apr 8. Epub 2019 Apr 8.

Department of Nuclear Medicine and Nancyclotep Imaging Platform, Université de Lorraine, CHRU-Nancy, 54000, Nancy, France.

Background: Atypical cerebrospinal fluid (CSF) patterns, involving an increase in the concentration of phosphorylated-tau (P-tau) proteins but normal amyloid-β concentration, are not uncommon in patients with mild neurocognitive disorders and suspected Alzheimer's disease (AD). In these conditions, however, AD diagnosis may be ruled out in the absence of any amyloid deposition at positron-emission tomography (PET). This pilot cross-sectional study was aimed to determine whether this negativity of amyloid PET can be predicted by CSF profiles in such patients. Read More

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http://dx.doi.org/10.1007/s00415-019-09315-yDOI Listing
April 2019
4 Reads

Parkinsonian traits in amyotrophic lateral sclerosis (ALS): a prospective population-based study.

J Neurol 2019 Apr 4. Epub 2019 Apr 4.

Department of Neuroscience "Rita Levi Montalcini", Movement Disorders Center, University of Turin, Turin, Italy.

Background: Amyotrophic lateral sclerosis (ALS) is characterized by a spectrum of phenotypes, but only a few studies have addressed the presence of parkinsonian (PK) symptoms. The aim of our study was to investigate the occurrence of PK features in a prospective population-based cohort of ALS patients, determining their demographic, clinical, neuropsychological and genetic characteristics, and identifying their morphological and functional imaging correlates.

Methods: A consecutive series of ALS patients were enrolled and prospectively followed for 2 years. Read More

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http://link.springer.com/10.1007/s00415-019-09305-0
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http://dx.doi.org/10.1007/s00415-019-09305-0DOI Listing
April 2019
4 Reads

Mapping the neuroanatomy of functional decline in Alzheimer's disease from basic to advanced activities of daily living.

J Neurol 2019 Apr 4. Epub 2019 Apr 4.

School of Health Sciences, University of East Anglia, Norwich, UK.

Background: Impairments in activities of daily living (ADL) are a criterion for Alzheimer's disease (AD) dementia. However, ADL gradually decline in AD, impacting on advanced (a-ADL, complex interpersonal or social functioning), instrumental (IADL, maintaining life in community), and finally basic functions (BADL, activities related to physiological and self-maintenance needs). Information and communication technologies (ICT) have become an increasingly important aspect of daily functioning. Read More

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http://dx.doi.org/10.1007/s00415-019-09260-wDOI Listing
April 2019
2 Reads
3.377 Impact Factor

Crossed aphasia confirmed by fMRI in a case with nonfluent variant of primary progressive aphasia carrying a GRN mutation.

J Neurol 2019 May 29;266(5):1274-1279. Epub 2019 Mar 29.

Neuroimaging Research Unit, Institute of Experimental Neurology, Division of Neuroscience, San Raffaele Scientific Institute, Vita-Salute San Raffaele University, Via Olgettina, 60, 20132, Milan, Italy.

Objectives: To characterize patterns of language lateralization in a right-handed woman with nonfluent/agrammatic primary progressive aphasia (nfvPPA) clinical picture despite showing a prevalent right-sided brain damage.

Methods: We report a case of a 58-year-old woman with nfvPPA diagnosis (age at onset = 55) previously described as a crossed aphasia case with progranulin mutation. At 2 years from the first visit, patient underwent 3DT1-weighted and a task-based functional MRI (fMRI). Read More

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http://dx.doi.org/10.1007/s00415-019-09298-wDOI Listing
May 2019
1 Read

Brain white matter changes in asymptomatic carriers of Leber's hereditary optic neuropathy.

J Neurol 2019 Mar 25. Epub 2019 Mar 25.

Department of Radiology and Tianjin Key Laboratory of Functional Imaging, Tianjin Medical University General Hospital, No. 154, Anshan Road, Heping District, Tianjin, 300052, China.

Objective: Subclinical abnormalities, including microangiopathy, swelling of nerve fibers, visual field abnormalities and visual functional impairments had been reported in Leber's hereditary optic neuropathy (LHON) carriers. The purpose of this study was to investigate microstructural changes of brain white matter in asymptomatic LHON carriers using DTI and tract-based spatial statistics (TBSS).

Methods: DTI and neuro-ophthalmologic measurements were acquired in 14 LHON carriers and 15 gender- and age-matched healthy controls, and diffusion metrics, including fractional anisotropy (FA), axial (AD), radial diffusion (RD) and mean diffusion (MD) were calculated. Read More

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http://dx.doi.org/10.1007/s00415-019-09284-2DOI Listing
March 2019
8 Reads

Screening of SLC2A1 in a large cohort of patients suspected for Glut1 deficiency syndrome: identification of novel variants and associated phenotypes.

J Neurol 2019 Mar 20. Epub 2019 Mar 20.

Department of Pediatric Neuroscience, Fondazione IRCCS Istituto Neurologico Carlo Besta, Milan, Italy.

Glucose transporter type 1 deficiency syndrome (Glut1 DS) is a rare neurological disorder caused by impaired glucose delivery to the brain. The clinical spectrum of Glut1 DS mainly includes epilepsy, paroxysmal dyskinesia (PD), developmental delay and microcephaly. Glut1 DS diagnosis is based on the identification of hypoglycorrhachia and pathogenic mutations of the SLC2A1 gene. Read More

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http://dx.doi.org/10.1007/s00415-019-09280-6DOI Listing
March 2019
1 Read
3.377 Impact Factor

A cluster of disseminated small cortical lesions in MELAS: its distinctive clinical and neuroimaging features.

J Neurol 2019 Mar 19. Epub 2019 Mar 19.

Department of Neurology, Kitasato University School of Medicine, 1-15-1 Kitasato, Minami-ku, Sagamihara, Kanagawa, 252-0374, Japan.

Objectives: To investigate a diversity of stroke-like episodes (SLEs) in mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS), and report a disseminated form of SLEs (D-SLEs) attributed to a cluster of disseminated small cortical lesions.

Methods: We retrospectively reviewed the clinical information of 27 MELAS patients seen at Kitasato University Hospital between January 1990 and April 2018. Among those, we selected 13 patients with m. Read More

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http://dx.doi.org/10.1007/s00415-019-09283-3DOI Listing
March 2019
1 Read

Granulomatous CNS inflammation associated with seminoma.

J Neurol 2019 Mar 11. Epub 2019 Mar 11.

Centre for Neurosarcoidosis, Neuroimmunology Unit, Institute of Immunity and Transplantation, University College London, London, NW3 2PF, UK.

Two cases in which a neurological disorder was identified pathologically to be due to a granulomatous infiltration were found after diagnosis to have an associated testicular seminoma with pathologically proven lymphatic metastasis. We present the clinical and imaging features, and pathological appearances of the lymphatic tissue and the brain. We summarise the literature to date and discuss the pathogenesis of the disorder and its treatment. Read More

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http://dx.doi.org/10.1007/s00415-019-09270-8DOI Listing
March 2019
2 Reads

Effect of small-vessel disease on cognitive trajectory after atrial fibrillation-related ischaemic stroke or  TIA.

J Neurol 2019 May 7;266(5):1250-1259. Epub 2019 Mar 7.

Department of Brain Repair and Rehabilitation, Stroke Research Centre, UCL Queen Square Institute of Neurology and National Hospital for Neurology and Neurosurgery, Russell Square House, 10-12 Russell Square, London, WC1B 5EH, UK.

Post-stroke dementia is common but has heterogenous mechanisms that are not fully understood, particularly in patients with atrial fibrillation (AF)-related ischaemic stroke or TIA. We investigated the relationship between MRI small-vessel disease markers (including a composite cerebral amyloid angiopathy, CAA, score) and cognitive trajectory over 12 months. We included patients from the CROMIS-2 AF study without pre-existing cognitive impairment and with Montreal Cognitive Assessment (MoCA) data. Read More

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http://dx.doi.org/10.1007/s00415-019-09256-6DOI Listing
May 2019
3 Reads

Speech treatment improves dysarthria in multisystemic ataxia: a rater-blinded, controlled pilot-study in ARSACS.

J Neurol 2019 May 6;266(5):1260-1266. Epub 2019 Mar 6.

Department of Neurodegeneration, Hertie Institute for Clinical Brain Research, Germany and Center for Neurology, University of Tübingen, University Hospital Tübingen, Tübingen, Germany.

We aimed to provide proof-of-principle evidence that intensive home-based speech treatment can improve dysarthria in complex multisystemic degenerative ataxias, exemplified by autosomal recessive spastic ataxia Charlevoix-Saguenay (ARSACS). Feasibility and piloting efficacy of speech training specifically tailored to cerebellar dysarthria was examined through a 4-week program in seven patients with rater-blinded assessment of intelligibility (primary outcome) and naturalness and acoustic measures of speech (secondary outcomes) performed 4 weeks before, immediately prior to, and directly after training (intraindividual control design). Speech intelligibility and naturalness improved post treatment. Read More

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http://link.springer.com/10.1007/s00415-019-09258-4
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http://dx.doi.org/10.1007/s00415-019-09258-4DOI Listing
May 2019
3 Reads

Sustained attention failures on a 3-min reaction time task is a sensitive marker of dementia.

J Neurol 2019 Mar 5. Epub 2019 Mar 5.

School of Psychology, The University of Sydney, Sydney, Australia.

The objective of the study is to determine the utility of a simple reaction time task as a marker of general cognitive decline across the frontotemporal lobar degeneration (FTLD) spectrum and in Alzheimer's disease (AD). One hundred and twelve patients presenting with AD or FTLD affecting behaviour (behavioural-variant frontotemporal dementia), language (progressive non fluent aphasia, logopenic progressive aphasia, semantic dementia) or motor function (corticobasal syndrome, progressive supranuclear palsy, frontotemporal dementia-motor neuron disease) and 25 age-matched healthy controls completed the Psychomotor Vigilance Task (PVT), a 3-min reaction time (RT) task. The proportion of lapses (RT > 500 ms) was significantly increased in dementia patients compared to healthy controls, except for semantic dementia, and correlated with all cognitive functions except language. Read More

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http://dx.doi.org/10.1007/s00415-019-09261-9DOI Listing
March 2019
4 Reads

Changing epidemiology of motor neurone disease in Scotland.

J Neurol 2019 Apr 25;266(4):817-825. Epub 2019 Feb 25.

Centre for Clinical Brain Sciences, University of Edinburgh, Chancellor's Building 49 Little France Crescent, Edinburgh, EH16 4TJ, UK.

Objectives: Scotland benefits from an integrated national healthcare team for motor neurone disease (MND) and a tradition of rich clinical data capture using the Scottish MND Register (launched in 1989; one of the first national registers). The Scottish register was re-launched in 2015 as Clinical Audit Research and Evaluation of MND (CARE-MND), an electronic platform for prospective, population-based research. We aimed to determine if incidence of MND is changing over time. Read More

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http://dx.doi.org/10.1007/s00415-019-09190-7DOI Listing
April 2019
6 Reads

Dissecting the neurological phenotype in children with callosal agenesis, interhemispheric cysts and malformations of cortical development.

J Neurol 2019 May 22;266(5):1167-1181. Epub 2019 Feb 22.

Pediatric Neuroradiology Unit, IRCCS Istituto Giannina Gaslini, Via Gaslini 5, 16147, Genoa, Italy.

Objectives: To describe the neurological phenotype of children with prenatal diagnosis of agenesis of corpus callosum (ACC) and interhemispheric cysts associated with malformations of cortical development (MCD).

Methods: We reviewed the neuroimaging, neurologic, EEG, and genetic data of 36 patients (21 males, mean age 7 years) with ACC and interhemispheric cysts. Associations were tested with Chi-squared and Fisher exact tests. Read More

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http://dx.doi.org/10.1007/s00415-019-09247-7DOI Listing
May 2019
2 Reads

Resolution of apathy after dorsal instead of ventral subthalamic deep brain stimulation for Parkinson's disease.

J Neurol 2019 May 20;266(5):1267-1269. Epub 2019 Feb 20.

Department of Psychiatry, Amsterdam University Medical Center, Amsterdam, The Netherlands.

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http://dx.doi.org/10.1007/s00415-019-09232-0DOI Listing

Deep brain stimulation of the subthalamic nucleus and the temporal discounting of primary and secondary rewards.

J Neurol 2019 May 14;266(5):1113-1119. Epub 2019 Feb 14.

Area of Neuroscience, SISSA, via Bonomea 265, 34136, Trieste, Italy.

Although deep brain stimulation of the subthalamic nucleus is an effective surgical treatment for Parkinson's disease, it may expose patients to non-motor side effects such as increased impulsivity and changes in decision-making behavior. Even if several studies have shown that stimulation of the subthalamic nucleus increases the incentive salience of food rewards in both humans and animals, temporal discounting for food rewards has never been investigated in patients who underwent STN-DBS. In this study, we measured inter-temporal choice after STN-DBS, using both primary and secondary rewards. Read More

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http://dx.doi.org/10.1007/s00415-019-09240-0DOI Listing

Dietary and lifestyle factors in multiple sclerosis progression: results from a 5-year longitudinal MRI study.

J Neurol 2019 Apr 13;266(4):866-875. Epub 2019 Feb 13.

Department of Neurology, Buffalo Neuroimaging Analysis Center, Jacobs School of Medicine and Biomedical Sciences, University at Buffalo, State University of New York, 100 High Street, Buffalo, NY, 142013, USA.

Background: Evidence regarding the role, if any, of dietary and lifestyle factors in the pathogenesis of multiple sclerosis (MS) is poorly understood.

Objective: To assess the effect of lifestyle-based risk factors linked to cardiovascular disease (CVD) on clinical and MRI-derived MS outcomes.

Methods: The study enrolled 175 MS or clinically isolated syndrome (CIS) patients and 42 age- and sex-matched healthy controls (HCs) who were longitudinally followed for 5. Read More

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http://dx.doi.org/10.1007/s00415-019-09208-0DOI Listing
April 2019
2 Reads

Frequent central nervous system, pachymeningeal and plexus MRI changes in POEMS syndrome.

J Neurol 2019 May 12;266(5):1067-1072. Epub 2019 Feb 12.

MRC Centre for Neuromuscular Diseases, National Hospital for Neurology and Neurosurgery, London, UK.

Objective: Polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, skin changes (POEMS) syndrome is a rare multisystem disease associated with a plasma-cell dyscrasia. Although pachymeningeal involvement has occasionally been described, MRI of the central nervous system (CNS) has not yet been extensively investigated.

Methods: We retrospectively evaluated CNS MRI in Europe's largest single-center cohort of POEMS syndrome. Read More

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http://link.springer.com/10.1007/s00415-019-09233-z
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http://dx.doi.org/10.1007/s00415-019-09233-zDOI Listing
May 2019
12 Reads

Sleep disturbances are common in patients with autoimmune encephalitis.

J Neurol 2019 Apr 11;266(4):1007-1015. Epub 2019 Feb 11.

Department of Neurology, Washington University School of Medicine, Saint Louis, MO, USA.

Objectives: Autoimmune encephalitis (AE) is increasingly recognized as an important cause of subacute cognitive decline, seizures, and encephalopathy, with an ever-broadening clinical phenotype. Sleep disturbances are reported in AE patients, including rapid eye movement sleep behavior disorder, hypersomnia, fragmented sleep, and sleep-disordered breathing; however, the prevalence of sleep disturbances and contributions to outcomes in AE patients remain unknown. There is a need to determine the prevalence of sleep disturbances in AE patients, and to clarify the relationship between specific autoantibodies and disruptions in sleep. Read More

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http://dx.doi.org/10.1007/s00415-019-09230-2DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6421090PMC
April 2019
6 Reads

Effectiveness and feasibility of cilostazol in patients with aneurysmal subarachnoid hemorrhage: a systematic review and meta-analysis.

J Neurol 2019 Feb 9. Epub 2019 Feb 9.

Department of Critical Care Medicine, West China Hospital, Sichuan University, Chengdu, Sichuan, China.

Background: Delayed cerebral ischemia seriously affects the prognosis of patients surviving the initial aneurysmal subarachnoid hemorrhage. Application of cilostazol was reported to ameliorate vasospasm and improve outcomes in series and clinical trials. But the effectiveness and feasibility of cilostazol on aneurysmal subarachnoid hemorrhage remained controversial. Read More

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http://link.springer.com/10.1007/s00415-019-09198-z
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http://dx.doi.org/10.1007/s00415-019-09198-zDOI Listing
February 2019
11 Reads

Early microvascular cerebral blood flow response to head-of-bed elevation is related to outcome in acute ischemic stroke.

J Neurol 2019 Apr 9;266(4):990-997. Epub 2019 Feb 9.

Department of Neurology, Sant Pau Biomedical Research Institute, Hospital de la Santa Creu i Sant Pau, Barcelona, Spain.

Background And Aims: Previously, microvascular cerebral blood flow (CBF) response to a mild head-of-bed (HOB) elevation has been shown to be altered in acute ischemic stroke (AIS) by diffuse correlation spectroscopy (DCS). We have hypothesized that early CBF response is related to the functional outcome.

Methods: Patients with a non-lacunar AIS in the anterior circulation were monitored by DCS to measure relative CBF (ΔrCBF) on the frontal lobes bilaterally during a 0°-30° HOB elevation at early (≤ 12) or late (> 12) hours from symptom onset. Read More

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http://dx.doi.org/10.1007/s00415-019-09226-yDOI Listing
April 2019
1 Read

Clinical and neuroimaging investigations of language disturbance in frontotemporal dementia-motor neuron disease patients.

J Neurol 2019 Apr 1;266(4):921-933. Epub 2019 Feb 1.

Brain and Mind Centre, The University of Sydney, Sydney, NSW, Australia.

This study systematically investigated the neuropsychological profile of language disturbance in frontotemporal dementia-motor neuron disease (FTD-MND) using a data-driven approach. Neuroanatomical correlates of language profiles were also examined. Patients with FTD-MND (N = 26), pure motor neuron disease (N = 34), progressive non-fluent aphasia (N = 30), semantic dementia (N = 17), and controls (N = 31) underwent comprehensive language assessments. Read More

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http://dx.doi.org/10.1007/s00415-019-09216-0DOI Listing
April 2019
3 Reads

Prospective association between adherence to the MIND diet and subjective memory complaints in the French NutriNet-Santé cohort.

J Neurol 2019 Apr 31;266(4):942-952. Epub 2019 Jan 31.

Equipe de Recherche en Epidémiologie Nutritionnelle (EREN), Centre d'Epidémiologie et Statistiques Sorbonne Paris Cité, Université Paris 13, Inserm (U1153), Cnam, Inra (U1125), COMUE Sorbonne Paris Cité, 74 rue Marcel Cachin, 93017, Bobigny, France.

Objectives: Our objective was to examine whether adherence to the Mediterranean-DASH diet intervention for neurodegenerative delay (MIND) was associated with SMC (as measured by the cognitive difficulties scale; CDS) in the NutriNet-Santé cohort.

Methods: The study sample consisted of 6011 participants aged ≥ 60 years at baseline, without SMC at the beginning. SMC were defined by a CDS score ≥ 43 (corresponding to the 4th CDS quartile) and SMC cases were participants with SMC at least once during follow-up. Read More

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http://dx.doi.org/10.1007/s00415-019-09218-yDOI Listing
April 2019
1 Read

Outcome after individualized stereoelectroencephalography (sEEG) implantation and navigated resection in patients with lesional and non-lesional focal epilepsy.

J Neurol 2019 Apr 30;266(4):910-920. Epub 2019 Jan 30.

Department of Neurosurgery, University Hospital of Munich, Ludwig-Maximilians-University, Marchioninistr. 15, 81377, Munich, Germany.

Background: Refined localization of the epileptogenic zone (EZ) in patients with pharmacoresistant focal epilepsy proceeding to resective surgery might improve postoperative outcome. We here report seizure outcome after stereo EEG (sEEG) evaluation with individually planned stereotactically implanted depth electrodes and subsequent tailored resection.

Methods: A cohort of consecutive patients with pharmacoresistant focal epilepsy, evaluated with a non-invasive evaluation protocol and invasive monitoring with personalized, stereotactically implanted depth electrodes for sEEG was analyzed. Read More

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http://dx.doi.org/10.1007/s00415-019-09213-3DOI Listing

Individual parkinsonian motor signs and striatal dopamine transporter deficiency: a study with [I-123]FP-CIT SPECT.

J Neurol 2019 Apr 28;266(4):826-834. Epub 2019 Jan 28.

Division of Clinical Neurosciences, University of Turku, Turku University Hospital, POB 52, 20521, Turku, Finland.

Introduction: Total parkinsonian motor symptom severity correlates with presynaptic striatal dopamine function in patients with Parkinson's disease. There is a lack of studies that have investigated the associations between parkinsonian motor signs and striatal dopaminergic deficiency in patients with parkinsonism of an unknown origin. Identification of specific motor signs associated with the highest likelihood of striatal dopamine deficiency could aid the differential diagnostics of parkinsonian and tremor syndromes. Read More

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http://dx.doi.org/10.1007/s00415-019-09202-6DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6420881PMC

Transient seizure onset network for localization of epileptogenic zone: effective connectivity and graph theory-based analyses of ECoG data in temporal lobe epilepsy.

J Neurol 2019 Apr 25;266(4):844-859. Epub 2019 Jan 25.

Department of Geriatric Medicine, Beijing Luhe Hospital, Capital Medical University, No. 82, Xinhua South Street, Tongzhou District, Beijing, 101149, China.

Objective: Abnormal and dynamic epileptogenic networks cause difficulties for clinical epileptologists in the localization of the seizure onset zone (SOZ) and the epileptogenic zone (EZ) in preoperative assessments of patients with refractory epilepsy. The aim of this study is to investigate the characteristics of time-varying effective connectivity networks in various non-seizure and seizure periods, and to propose a quantitative approach for accurate localization of SOZ and EZ.

Methods: We used electrocorticogram recordings in the temporal lobe and hippocampus from seven patients with temporal lobe epilepsy to characterize the effective connectivity dynamics at a high temporal resolution using the full-frequency adaptive directed transfer function (ffADTF) measure and five graph metrics, i. Read More

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http://link.springer.com/10.1007/s00415-019-09204-4
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http://dx.doi.org/10.1007/s00415-019-09204-4DOI Listing
April 2019
9 Reads

Deep brain stimulation for monogenic Parkinson's disease: a systematic review.

J Neurol 2019 Jan 18. Epub 2019 Jan 18.

Division of Clinical Neurosciences, Turku University Hospital, Hämeentie 11, POB 52, 20521, Turku, Finland.

Deep brain stimulation (DBS) is an effective treatment for Parkinson's disease (PD) patients with motor fluctuations and dyskinesias. The key DBS efficacy studies were performed in PD patients with unknown genotypes; however, given the estimated monogenic mutation prevalence of approximately 5-10%, most commonly LRRK2, PRKN, PINK1 and SNCA, and risk-increasing genetic factors such as GBA, proper characterization is becoming increasingly relevant. We performed a systematic review of 46 studies that reported DBS effects in 221 genetic PD patients. Read More

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http://dx.doi.org/10.1007/s00415-019-09181-8DOI Listing
January 2019
3 Reads

Painful legs and moving toes syndrome evaluated through brain single photon emission computed tomography: a case series.

J Neurol 2019 Mar 14;266(3):717-725. Epub 2019 Jan 14.

Department of Neurology, Juntendo University School of Medicine, 3-1-3 Hongo, Bunkyo-ku, Tokyo, 113-8431, Japan.

Painful legs and moving toes (PLMT) syndrome is a clinical entity characterized by persistent pain in the feet or legs and involuntary movements of one or more toes. The precise patho-mechanisms of PLMT still remain unknown. Herein, we examined ten patients clinically identified with PLMT syndrome. Read More

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http://dx.doi.org/10.1007/s00415-019-09194-3DOI Listing
March 2019
3 Reads

Incidence and mechanism of early neurological deterioration after endovascular thrombectomy.

J Neurol 2019 Mar 10;266(3):609-615. Epub 2019 Jan 10.

Department of Neurology, Chung-Ang University Hospital, Chung-Ang University College of Medicine, Seoul, 224-1, Heukseok-dong, Dongjak-gu, Seoul, 156-755, South Korea.

Background: We investigated the prevalence and mechanisms of neurological deterioration after endovascular thrombectomy.

Methods: Between January 2011 and October 2017, acute ischemic stroke patients treated by endovascular thrombectomy in a tertiary university hospital were included. Early neurological deterioration (END) was defined as an increase of 2 or more National Institute of Health Stroke Scale (NIHSS) compared to the best neurological status after stroke within 7 days. Read More

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http://link.springer.com/10.1007/s00415-018-09173-0
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http://dx.doi.org/10.1007/s00415-018-09173-0DOI Listing
March 2019
26 Reads

Continuous subcutaneous apomorphine in advanced Parkinson's disease patients treated with deep brain stimulation.

J Neurol 2019 Mar 7;266(3):659-666. Epub 2019 Jan 7.

Servicio de Neurología, Hospital Clínico Universitario Santiago de Compostela, Santiago de Compostela, Spain.

Introduction: Deep brain stimulation (DBS) is an effective therapy for patients with advanced Parkinson's disease (PD). However, sometimes, it is not sufficient to adequately control motor symptoms. We describe our experience with continuous subcutaneous apomorphine infusion (APO) in patients with DBS. Read More

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http://dx.doi.org/10.1007/s00415-019-09184-5DOI Listing
March 2019
1 Read

Atypical parkinsonism with severely reduced striatal dopamine uptake associated with a 16p11.2 duplication syndrome.

J Neurol 2019 Mar 7;266(3):775-776. Epub 2019 Jan 7.

Department of Neurodegenerative Diseases, Hertie Institute for Clinical Brain Research (HIH), University of Tübingen, Hoppe-Seyler-Str. 3, 72076, Tübingen, Germany.

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http://link.springer.com/10.1007/s00415-019-09182-7
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http://dx.doi.org/10.1007/s00415-019-09182-7DOI Listing
March 2019
10 Reads

Neurological update: MOG antibody disease.

J Neurol 2019 May 19;266(5):1280-1286. Epub 2018 Dec 19.

Division of Psychological Medicine and Clinical Neurosciences, Cardiff University School of Medicine, University Hospital of Wales, Heath Park, Cardiff, CF14 4XN, UK.

Myelin oligodendrocyte glycoprotein (MOG) antibody disease (MOG-AD) is now recognised as a nosological entity with specific clinical and paraclinical features to aid early diagnosis. Although no age group is exempt, median age of onset is within the fourth decade of life, with optic neuritis being the most frequent presenting phenotype. Disease course can be either monophasic or relapsing, with subsequent relapses most commonly involving the optic nerve. Read More

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http://dx.doi.org/10.1007/s00415-018-9122-2DOI Listing
May 2019
7 Reads

Patient characteristics and outcome associations in AMPA receptor encephalitis.

J Neurol 2019 Feb 17;266(2):450-460. Epub 2018 Dec 17.

Department of Neurology, Washington University in St. Louis, Saint Louis, MO, USA.

Antibody-mediated encephalitis defines a class of diseases wherein antibodies directed at cell-surface receptors are associated with behavioral and cognitive disturbances. One such recently described encephalitis is due to antibodies directed at alpha-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptors (AMPAR). This entity is exceptionally rare and its clinical phenotype incompletely described. Read More

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http://dx.doi.org/10.1007/s00415-018-9153-8DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6367044PMC
February 2019
4 Reads

Clinical and economic comparison of an individualised immunoglobulin protocol vs. standard dosing for chronic inflammatory demyelinating polyneuropathy.

J Neurol 2019 Feb 17;266(2):461-467. Epub 2018 Dec 17.

Regional Neuromuscular Service, University Hospitals Birmingham, Birmingham, UK.

Background: The clinical and economic implications of an individualised intravenous immunoglobulin (IVIg) protocol for chronic inflammatory demyelinating polyneuropathy (CIDP) are unknown. Comparison with standard dosing regimens has not been performed.

Methods: We retrospectively studied 47 IVIg-treated subjects with CIDP over 4 years with an individualised, outcome-measured, dose-modifying protocol. Read More

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http://dx.doi.org/10.1007/s00415-018-9157-4DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6373347PMC
February 2019
1 Read

Clinical feasibility of 1-min ultrafast brain MRI compared with routine brain MRI using synthetic MRI: a single center pilot study.

J Neurol 2019 Feb 7;266(2):431-439. Epub 2018 Dec 7.

MR Applications and Workflow, GE Healthcare Korea, 343 Hakdong-ro, Gangnam-gu, Seoul, 06060, Republic of Korea.

Background: Ultrafast brain MRI is required for uncooperative patients and time-critical diseases such as stroke because it reduces scan times and motion artifacts. This study investigated the clinical feasibility of a 1-min ultrafast brain MRI protocol for detecting intracranial abnormalities in restless and uncooperative patients.

Methods: We retrospectively reviewed the records of 25 patients who underwent a 1-min ultrafast MRI protocol using T1-weighted image, T2-weighted image, echo-planar fluid-attenuated inversion recovery, diffusion-weighted image, and T2*-weighted image between March 2017 and May 2017. Read More

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http://dx.doi.org/10.1007/s00415-018-9149-4DOI Listing
February 2019
3 Reads
3.377 Impact Factor

Gait festination in parkinsonism: introduction of two phenotypes.

J Neurol 2019 Feb 7;266(2):426-430. Epub 2018 Dec 7.

Department of Neurology, Centre of Clinical Neuroscience, First Faculty of Medicine, General University Hospital, Charles University, Prague, Czech Republic.

Gait festination is one of the most characteristic gait disturbances in patients with Parkinson's disease or atypical parkinsonism. Although festination is common and disabling, it has received little attention in the literature, and different definitions exist. Here, we argue that there are actually two phenotypes of festination. Read More

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http://dx.doi.org/10.1007/s00415-018-9146-7DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6373367PMC
February 2019
4 Reads

The applause sign in frontotemporal lobar degeneration and related conditions.

J Neurol 2019 Feb 1;266(2):330-338. Epub 2018 Dec 1.

Neurologische Klinik und Poliklinik, Ludwig Maximilians Universität München, Munich, Germany.

The applause sign, i.e., the inability to execute the same amount of claps as performed by the examiner, was originally reported as a sign specific for progressive supranuclear palsy (PSP). Read More

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http://dx.doi.org/10.1007/s00415-018-9134-yDOI Listing
February 2019
26 Reads
3.377 Impact Factor

ANO10 mutational screening in recessive ataxia: genetic findings and refinement of the clinical phenotype.

J Neurol 2019 Feb 4;266(2):378-385. Epub 2018 Dec 4.

Unit of Medical Genetics and Neurogenetics, Fondazione IRCCS Istituto Neurologico Carlo Besta, via Celoria 11, 20133, Milan, Italy.

Autosomal recessive cerebellar ataxia type 3 (ARCA3) is a rare inherited disorder caused by mutations in the ANO10 gene. The disease is characterized by slowly progressive spastic ataxia variably associated with motor neuron involvement, epilepsy, and cognitive decline. We performed mutational screening in 80 patients with sporadic or autosomal recessive adult-onset ataxia. Read More

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http://link.springer.com/10.1007/s00415-018-9141-z
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http://dx.doi.org/10.1007/s00415-018-9141-zDOI Listing
February 2019
17 Reads

A controlled inflammation and a regulatory immune system are associated with more favorable prognosis of progressive multifocal leukoencephalopathy.

J Neurol 2019 Feb 3;266(2):369-377. Epub 2018 Dec 3.

Department of Neurology and Neurological Science, Tokyo Medical and Dental University Graduate School of Medical and Dental Sciences, 1-5-45 Yushima Bunkyo-ku, Tokyo, 113-8510, Japan.

Objective: In the present study, we analyzed the inflammatory profiles of brain tissues obtained from patients with progressive multifocal leukoencephalopathy (PML) due to John Cunningham (JC) virus infection to identify potential prognostic factors.

Methods: The study included seven patients (two men, five women) who had been pathologically diagnosed with PML, and all of whom were HIV negative. Fixed brain samples were analyzed via hematoxylin and eosin (HE) staining and Klüver-Barrera (KB) staining. Read More

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http://dx.doi.org/10.1007/s00415-018-9140-0DOI Listing
February 2019
3 Reads

Motor neuron involvement in anti-Ma2-associated paraneoplastic neurological syndrome.

J Neurol 2019 Feb 29;266(2):398-410. Epub 2018 Nov 29.

French Reference Center of Paraneoplastic Neurological Syndrome, Hospices Civils de Lyon, Hôpital Neurologique, Lyon, France.

Objective: To present clinical, radiological, and pathological features of a cohort of patients with motor neuron involvement in association with anti-Ma2 antibodies (Ma2-Ab).

Methods: Retrospective case-series of patients with definite paraneoplastic neurological syndrome (PNS) and Ma2-Ab, and cases identified from a review of the literature.

Results: Among 33 Ma2-Ab patients referred between 2002 and 2016, we retrospectively identified three patients (9. Read More

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http://dx.doi.org/10.1007/s00415-018-9143-xDOI Listing
February 2019
5 Reads

Brain tissue volumes and relaxation rates in multiple sclerosis: implications for cognitive impairment.

J Neurol 2019 Feb 29;266(2):361-368. Epub 2018 Nov 29.

Biostructure and Bioimaging Institute, National Research Council, Via De Amicis, 95, 80145, Naples, Italy.

Objective: Both normal gray matter atrophy and brain tissue relaxation rates, in addition to total lesion volume, have shown significant correlations with cognitive test scores in multiple sclerosis (MS). Aim of the study was to assess the relative contributions of macro- and microstructural changes of both normal and abnormal brain tissues, probed, respectively, by their volumes and relaxation rates, to the cognitive status and physical disability of MS patients.

Methods: MRI studies from 241 patients with relapsing-remitting MS were retrospectively analyzed by fully automated multiparametric relaxometric segmentation. Read More

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http://dx.doi.org/10.1007/s00415-018-9139-6DOI Listing
February 2019
1 Read

Different routes of heroin intake cause various heroin-induced leukoencephalopathies.

J Neurol 2019 Feb 26;266(2):316-329. Epub 2018 Nov 26.

Department of Neurology, Linkou Medical Center, Chang Gung Memorial Hospital, Taoyuan, Taiwan.

Objective: Toxic leukoencephalopathy is a rare but critical neurological disorder in heroin abusers. Our aim is to compare the clinical manifestations, brain MRIs and prognoses of heroin-induced leukoencephalopathy by different intake routes.

Methods: We present two patients with toxic leukoencephalopathy caused by intravenous (IV) injection of heroin and 48 additional cases from systematic reviews of the literature published between 1994 and 2018. Read More

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http://dx.doi.org/10.1007/s00415-018-9131-1DOI Listing
February 2019
3 Reads
3.377 Impact Factor

Endovascular stroke treatment's impact on malignant type of edema (ESTIMATE).

J Neurol 2019 Jan 23;266(1):223-231. Epub 2018 Nov 23.

Department of Neurology, University Hospital Ulm, Oberer Eselsberg 45, 89081, Ulm, Germany.

Background And Purpose: In patients with acute ischemic stroke and large vessel occlusion, the prognosis has improved tremendously since the implementation of endovascular thrombectomy (EVT). The effect of EVT on the incidence of malignant middle cerebral artery infarctions (MMI) has not been studied before.

Methods: ESTIMATE, a multicenter retrospective study, evaluates data of ischemic stroke patients with occlusion in the anterior circulation in the years of 2007-2015 comparing three treatment options (no therapy; IV-TPA; IV-TPA plus EVT or EVT only). Read More

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http://dx.doi.org/10.1007/s00415-018-9127-xDOI Listing
January 2019
14 Reads

Structural network topology relates to tissue properties in multiple sclerosis.

J Neurol 2019 Jan 22;266(1):212-222. Epub 2018 Nov 22.

Department of Anatomy and Neurosciences, Amsterdam Neuroscience, MS Center Amsterdam, Amsterdam UMC, Location VU University Medical Center, De Boelelaan 1108, 1081 HZ, Amsterdam, The Netherlands.

Objective: Abnormalities in segregative and integrative properties of brain networks have been observed in multiple sclerosis (MS) and are related to clinical functioning. This study aims to investigate the micro-scale correlates of macro-scale network measures of segregation and integration in MS.

Methods: Eight MS patients underwent post-mortem in situ whole-brain diffusion tensor (DT) imaging and subsequent brain dissection. Read More

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http://dx.doi.org/10.1007/s00415-018-9130-2DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6342882PMC
January 2019
9 Reads