10,206 results match your criteria Journal of neurology[Journal]


Congenital myasthenic syndrome caused by novel COL13A1 mutations.

J Neurol 2019 Feb 14. Epub 2019 Feb 14.

Department of Neurology, Friedrich-Baur-Institute, University Hospital, LMU Munich, Marchioninistrasse 17, 81377, Munich, Germany.

Collagen XIII is a non-fibrillar transmembrane collagen which has been long recognized for its critical role in synaptic maturation of the neuromuscular junction. More recently, biallelic COL13A1 loss-of-function mutations were identified in three patients with congenital myasthenic syndrome (CMS), a rare inherited condition with defective neuromuscular transmission, causing abnormal fatigability and fluctuating muscle weakness and often successfully treated with acetylcholinesterase inhibitors. Here we report six additional CMS patients from three unrelated families with previously unreported homozygous COL13A1 loss-of-function mutations (p. Read More

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http://dx.doi.org/10.1007/s00415-019-09239-7DOI Listing
February 2019

MRI detects peripheral nerve and adjacent muscle pathology in non-systemic vasculitic neuropathy (NSVN).

J Neurol 2019 Feb 14. Epub 2019 Feb 14.

Department of Radiology, University of Cologne, Cologne, Germany.

Background: Diagnosis and disease monitoring of non-systemic vasculitic neuropathy (NSVN) are based on electrophysiological and clinical measures. However, these methods are insensitive to detect subtle differences of axonal injury. We here assessed the utility of a multiparametric MRI protocol to quantify axonal injury and neurogenic muscle damage in NSVN. Read More

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http://dx.doi.org/10.1007/s00415-019-09224-0DOI Listing
February 2019

Parkinson's disease among migrants in Europe: estimating the magnitude of an emerging phenomenon.

J Neurol 2019 Feb 14. Epub 2019 Feb 14.

Department of Human Neuroscience, Sapienza University, Viale dell'Università 30, 00185, Rome, Italy.

Introduction: The occurrence of age-related pathological conditions among subjects with a migration background and composing ethnic minorities is an emerging challenge for Western countries. Specifically, the onset of neurodegenerative diseases in these populations of individuals might assume special relevance and generate additional complexities for our healthcare systems. The aim of the present study was to estimate the number of Parkinson's disease (PD) cases in migrant subjects living in Europe. Read More

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http://dx.doi.org/10.1007/s00415-019-09241-zDOI Listing
February 2019

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

J Neurol 2019 Feb 14. 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
February 2019

Cerebral microbleeds are associated with cognitive decline early after ischemic stroke.

J Neurol 2019 Feb 14. Epub 2019 Feb 14.

Department of Neurology, University Hospital of Würzburg, Josef-Schneider Strasse 11, 97080, Würzburg, Germany.

Background And Purpose: The present study aimed to investigate whether cerebral microbleeds (CMB) are associated with vascular cognitive decline (VCD) already in the early course after ischemic stroke, and-if so-whether distinct cognitive domains are affected more preferentially by CMB.

Methods: In a prospective cohort study, cognitive performance was examined in 33 stroke patients showing ≥ 1 CMB on MRI. Matched for age, gender, clinical and radiological characteristics, 33 stroke survivors without CMB served as a control group. Read More

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http://dx.doi.org/10.1007/s00415-019-09236-wDOI Listing
February 2019

Correction to: Usefulness of MOG-antibody titres at first episode to predict the future clinical course in adults.

J Neurol 2019 Feb 7. Epub 2019 Feb 7.

Service de neurologie, sclérose en plaques, pathologies de la myéline et neuro-inflammation and Centre de référence pour les maladies inflammatoires rares du cerveau et de la moelle (MIRCEM), Hôpital Neurologique Pierre Wertheimer Hospices Civils de Lyon, 59 boulevard Pinel, BRON cedex, 69677, Lyon, France.

The original version of this article unfortunately contained a mistake. Read More

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http://dx.doi.org/10.1007/s00415-019-09215-1DOI Listing
February 2019

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

J Neurol 2019 Feb 13. 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
February 2019

Technology-assisted recovery of walking after spinal cord injury.

J Neurol 2019 Feb 13. Epub 2019 Feb 13.

Institute of Psychological Medicine and Clinical Neuroscience, Cardiff University, University Hospital of Wales, Heath Park, Cardiff, CF14 4XN, UK.

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http://dx.doi.org/10.1007/s00415-019-09227-xDOI Listing
February 2019

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

J Neurol 2019 Feb 12. 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
February 2019
3 Reads

Anger, hostility and risk of stroke: a meta-analysis of cohort studies.

J Neurol 2019 Feb 11. Epub 2019 Feb 11.

Department of Neurology, The First Affiliated Hospital, China Medical University, Shenyang, China.

Objective: A growing number of studies have been conducted on the relationship between anger and hostility and the risk of stroke, and their conclusions are not consistent. Accordingly, we performed a meta-analysis to evaluate the relationship between anger and hostility and the risk of stroke.

Methods: We searched the PubMed and Embase databases for cohort studies, focusing on the relationship between anger and hostility and risk of stroke. Read More

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http://dx.doi.org/10.1007/s00415-019-09231-1DOI Listing
February 2019

Population studies highlight an increased risk of dementia in both dependent and non-dependent drinkers.

Authors:
Mo Hu Robin Corkill

J Neurol 2019 Feb 12. Epub 2019 Feb 12.

Department of Neurology, University Hospital Wales, CF14 4XW, Cardiff, UK.

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http://dx.doi.org/10.1007/s00415-019-09192-5DOI Listing
February 2019
1 Read

A 30 s test for quantitative assessment of a relative afferent pupillary defect (RAPD): the infrared pupillary asymmetry (IPA).

J Neurol 2019 Feb 12. Epub 2019 Feb 12.

Department of Neuro-ophthalmology, Moorfields Eye Hospital, 162 City Road, London, EC1V 2PD, UK.

Background: Detection of a relative afferent pupillary defect (RAPD) by the swinging-light test can be challenging in clinical practice (dark eyes, anisocoria, dark environment). We developed a new method of RAPD quantification based on the recording of the infrared pupillary asymmetry (IPA) with a standard optical coherence tomography (OCT) device.

Methods: The diagnostic value of the IPA for detection of the RAPD was determined by receiver-operating characteristic (ROC) curves and area under the curve (AUC). Read More

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http://dx.doi.org/10.1007/s00415-019-09223-1DOI Listing
February 2019

Thymomatous myasthenia gravis: novel association with HLA DQB1*05:01 and strengthened evidence of high clinical and serological severity.

J Neurol 2019 Feb 11. Epub 2019 Feb 11.

Laboratorio d'Immunogenetica dei Trapianti, Dipartimento di Oncoematologia e Terapia Cellulare e Genica, IRCCS OPBG, Rome, Italy.

Background: The relative prevalence of myasthenia gravis (MG) subtypes is changing, and their differential features and association with HLA class II alleles are not completely understood.

Methods: Age at onset, presence/absence of autoantibodies (Ab) and thymoma were retrospectively considered in 230 adult Italian patients. Clinical severity, assessed by MGFA scale, and the highest Ab titer were recorded. Read More

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http://dx.doi.org/10.1007/s00415-019-09225-zDOI Listing
February 2019
1 Read

Sleep disturbances are common in patients with autoimmune encephalitis.

J Neurol 2019 Feb 11. 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
February 2019
1 Read

Infliximab biosimilar for treating neurosarcoidosis: tolerance and efficacy in a retrospective study including switch from the originator and initiation of treatment.

J Neurol 2019 Feb 9. Epub 2019 Feb 9.

Sorbonne Université, Assistance Publique-Hôpitaux de Paris, Service de Médecine Interne 2, Centre National de Référence Maladies Systémiques Rares, Hôpital Pitié-Salpêtrière, 47-83 Boulevard de l'hôpital, 75651, Paris Cedex 13, France.

Objectives: Infliximab is increasingly used to treat neurosarcoidosis. We aimed to determine the efficacy and tolerance of an infliximab biosimilar for treating neurosarcoidosis.

Methods: We conducted a retrospective single-center study to describe the efficacy, safety and immunogenicity of an infliximab biosimilar in neurosarcoidosis patients. Read More

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http://dx.doi.org/10.1007/s00415-019-09234-yDOI Listing
February 2019
1 Read

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
2 Reads

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

J Neurol 2019 Feb 9. 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
February 2019

Intravenous immunoglobulins as first-line therapy for IgLON5 encephalopathy.

J Neurol 2019 Feb 8. Epub 2019 Feb 8.

Department of Neurology, Medical Faculty, Heinrich-Heine University Düsseldorf, Moorenstr. 5, 40225, Düsseldorf, Germany.

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http://dx.doi.org/10.1007/s00415-019-09221-3DOI Listing
February 2019
1 Read

Five-year outcome in the copaxone observatory: a nationwide cohort of patients with multiple sclerosis starting treatment with glatiramer acetate in France.

J Neurol 2019 Feb 7. Epub 2019 Feb 7.

Teva Santé, La Défense, France.

The benefits provided by disease-modifying treatments in multiple sclerosis have been demonstrated in clinical trials, but the extent to which they can be extrapolated to everyday care is less clear, as are the long-term benefits of treatment. The objective of this prospective observational cohort study performed in France was to evaluate the effectiveness and safety of glatiramer acetate in patients with relapsing-remitting multiple sclerosis over a 5-year period. All neurologists in France were invited to participate and enroll adult patients starting a first treatment with brand glatiramer acetate 20 mg. Read More

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http://dx.doi.org/10.1007/s00415-019-09211-5DOI Listing
February 2019

Lack of accuracy of the international classification of disease, ninth (ICD-9) codes in identifying patients with encephalitis.

J Neurol 2019 Feb 7. Epub 2019 Feb 7.

Division of Infectious Disease, Department of Medicine, McGovern Medical School at University of Texas Health Sciences Center, 6431 Fannin St. 2.112 MSB, Houston, TX, 77030, USA.

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http://dx.doi.org/10.1007/s00415-019-09229-9DOI Listing
February 2019

Non-alcoholic Wernicke encephalopathy presenting as bilateral hearing loss: a case report.

J Neurol 2019 Feb 7. Epub 2019 Feb 7.

Department of Neurosciences, S. Camillo-Forlanini Hospital, C.ne Gianicolense 87, 00152, Rome, Italy.

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http://dx.doi.org/10.1007/s00415-019-09220-4DOI Listing
February 2019
1 Read
3.377 Impact Factor

Effect of thymectomy in elderly patients with non-thymomatous generalized myasthenia gravis.

J Neurol 2019 Feb 6. Epub 2019 Feb 6.

Department of Neurology, Yonsei University College of Medicine, Seoul, South Korea.

Whether thymectomy is beneficial in elderly patients with myasthenia gravis (MG) is unclear. Thus, we assessed whether conducting thymectomy in MG patients aged ≥ 50 years is beneficial. This retrospective cohort study included patients with MG between 1990 and 2018. Read More

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http://dx.doi.org/10.1007/s00415-019-09222-2DOI Listing
February 2019

Six-minute walk test is reliable and sensitive in detecting response to therapy in CIDP.

J Neurol 2019 Feb 5. Epub 2019 Feb 5.

Department of Neurosciences, Odontostomatology and Reproductive Sciences, University of Naples " Federico II ", Via Pansini, 5, 81025, Naples, Italy.

Objective: The current clinical measures (ONLS, R-ODS, mRS, and MRC) may not be so sensitive in capturing minimal variations or measuring fatigue in chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). Our aim was to assess if 6-min walk test (6MWT) is able to increase the sensitivity in detecting response to therapy and to capture fatigue in CIDP patients.

Methods: We tested 6MWT in 42 CIDP patients. Read More

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http://dx.doi.org/10.1007/s00415-019-09207-1DOI Listing
February 2019

A case of anaphylaxis to alemtuzumab.

J Neurol 2019 Feb 4. Epub 2019 Feb 4.

Department of Clinical Neurosciences, Cambridge University Hospitals NHS Foundation Trust, Cambridge, UK.

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http://dx.doi.org/10.1007/s00415-019-09214-2DOI Listing
February 2019
1 Read

Muscle pain in mitochondrial diseases: a picture from the Italian network.

J Neurol 2019 Feb 2. Epub 2019 Feb 2.

Neurological Clinic, University of Pisa, Pisa, Italy.

Muscle pain may be part of many neuromuscular disorders including myopathies, peripheral neuropathies and lower motor neuron diseases. Although it has been reported also in mitochondrial diseases (MD), no extensive studies in this group of diseases have been performed so far. We reviewed clinical data from 1398 patients affected with mitochondrial diseases listed in the database of the "Nation-wide Italian Collaborative Network of Mitochondrial Diseases", to assess muscle pain and its features. Read More

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http://link.springer.com/10.1007/s00415-019-09219-x
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http://dx.doi.org/10.1007/s00415-019-09219-xDOI Listing
February 2019
4 Reads

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

J Neurol 2019 Feb 1. 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
February 2019
1 Read

High performance of cerebrospinal fluid immunoglobulin G analysis for diagnosis of multiple sclerosis.

J Neurol 2019 Feb 1. Epub 2019 Feb 1.

Inserm CIC-EC 1433, Nancy University Hospital, Université de Lorraine, 54 000, Nancy, France.

Background: The 2017 revision of the McDonald criteria highlights the usefulness of cerebrospinal fluid (CSF) immunoglobulin G (IgG) analysis to diagnose multiple sclerosis (MS). The objective of this study was to assess the diagnostic performances of CSF IgG analysis in the absence of a gold standard.

Methods: All patients who underwent CSF IgG analysis for events suggestive of MS in Nancy University Hospital (France) from 2008 to 2011 were retrospectively included. Read More

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http://dx.doi.org/10.1007/s00415-019-09212-4DOI Listing
February 2019

Bethlem myopathy: a series of 16 patients and description of seven new associated mutations.

J Neurol 2019 Jan 31. Epub 2019 Jan 31.

Department of Neurology, Hospital Universitario 12 de Octubre, Madrid, Spain.

Background: Bethlem myopathy represents the milder phenotype of collagen type VI-related myopathies. However, clinical manifestations are highly variable among patients and no phenotype-genotype correlation has been described. We aim to analyse the clinical, pathological and genetic features of a series of patients with Bethlem myopathy, and we describe seven new mutations. Read More

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http://link.springer.com/10.1007/s00415-019-09217-z
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http://dx.doi.org/10.1007/s00415-019-09217-zDOI Listing
January 2019
5 Reads

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

J Neurol 2019 Jan 31. 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
January 2019

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

J Neurol 2019 Jan 30. 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
January 2019

Magnetic resonance imaging in immune-mediated myelopathies.

J Neurol 2019 Jan 29. Epub 2019 Jan 29.

Neurology Clinic and Policlinic, Departments of Medicine, Clinical Research and Biomedical Engineering, University Hospital Basel, University of Basel, Petersgraben 4, 4031, Basel, Switzerland.

Immune-mediated myelopathies are a heterogeneous group of inflammatory spinal cord disorders including autoimmune disorders with known antibodies, e.g. aquaporin-4 IgG channelopathy or anti-myelin oligodendrocyte glycoprotein-associated myelitis, myelopathies in the context of multiple sclerosis and systemic autoimmune disorders with myelopathy, as well as post-infectious and paraneoplastic myelopathies. Read More

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http://dx.doi.org/10.1007/s00415-019-09206-2DOI Listing
January 2019

Pre-treatment cerebral microbleeds and intracranial hemorrhage in patients with ischemic stroke receiving endovascular therapy: a systematic review and meta-analysis.

J Neurol 2019 Jan 28. Epub 2019 Jan 28.

Cerebrovascular Disease Center, Department of Neurology, People's Hospital, China Medical University, 33 Wenyi Road, Shenhe District, Shenyang, 110016, People's Republic of China.

Background And Purpose: Predicting the risk of intracranial hemorrhage (ICH) is an important aspect for improving the efficacy and safety of endovascular therapy (EVT). We intended to perform a systematic review and meta-analysis to show whether pre-treatment cerebral microbleeds (CMBs) were associated with an increased incidence of ICH in patients with ischemic stroke receiving EVT.

Methods: We searched PubMed, EMBASE, Web of Science and Cochrane Library from their dates of inception to December 18, 2018, and also manually searched reference lists of relevant articles. Read More

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http://dx.doi.org/10.1007/s00415-019-09210-6DOI Listing
January 2019
1 Read

Cognitive changes of older adults with an equivocal amyloid load.

J Neurol 2019 Jan 28. Epub 2019 Jan 28.

Gérontopôle de Toulouse, Centre Hospitalo-Universitaire de Toulouse (CHU Toulouse), Toulouse, France.

Background: Observational and interventional studies addressing the link between amyloid (Aβ) burden and cognitive decline are increasing, but a clear definition of amyloid positivity is still lacking. This may represent a great stake for therapeutic studies enrolling Aβ + patients only. The main objective of this study was to define a population with "equivocal" amyloid status, and evaluate their cognitive changes. Read More

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http://dx.doi.org/10.1007/s00415-019-09203-5DOI Listing
January 2019

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

J Neurol 2019 Jan 28. 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
January 2019

Charles Bonnet syndrome in Leber's hereditary optic neuropathy.

J Neurol 2019 Jan 28. Epub 2019 Jan 28.

Department of Neurology, Friedrich-Baur Institute, Ludwig-Maximilians-Universität München, Ziemssenstr. 1a, 80336, Munich, Germany.

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http://dx.doi.org/10.1007/s00415-019-09205-3DOI Listing
January 2019

Tollgate-based progression pathways of ALS patients.

J Neurol 2019 Jan 25. Epub 2019 Jan 25.

Department of Health Sciences Research, Mayo Clinic, 200 First Street SW, Rochester, MN, 55905, USA.

Objective: To capture ALS progression in arm, leg, speech, swallowing, and breathing segments using a disease-specific staging system, namely tollgate-based ALS staging system (TASS), where tollgates refer to a set of critical clinical events including having slight weakness in arms, needing a wheelchair, needing a feeding tube, etc. METHODS: We compiled a longitudinal dataset from medical records including free-text clinical notes of 514 ALS patients from Mayo Clinic, Rochester-MN. We derived tollgate-based progression pathways of patients up to a 1-year period starting from the first clinic visit. Read More

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http://dx.doi.org/10.1007/s00415-019-09199-yDOI Listing
January 2019

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 Jan 25. 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
January 2019
3 Reads

Evolution of acute lacunar lesions in terms of size and shape: a PICASSO sub-study.

J Neurol 2019 Jan 23. Epub 2019 Jan 23.

Department of Neurology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Republic of Korea.

The imaging definition of lacunar infarcts is variable, particularly regarding their size and the presence of cavitation. We investigated the changes of diameter and evolution pattern of acute lacunar infarcts, and the factors associated with the evolution pattern. Patients with acute single subcortical hemispheric or brainstem ischemic lesions of penetrating arterial territories were included. Read More

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http://link.springer.com/10.1007/s00415-019-09201-7
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http://dx.doi.org/10.1007/s00415-019-09201-7DOI Listing
January 2019
3 Reads

Comparative efficacy and safety of therapy for the behavioral and psychological symptoms of dementia: a systemic review and Bayesian network meta-analysis.

Authors:
Boru Jin Huayan Liu

J Neurol 2019 Jan 21. Epub 2019 Jan 21.

Department of Neurology, First Affiliated Hospital, China Medical University, No. 155, North Nanjing Street, Shenyang, 110001, People's Republic of China.

Objective: To assess the comparative efficacy and safety of both pharmacological and non-pharmacological therapies for the behavioral and psychological symptoms of dementia, using direct and indirect evidence from randomized data.

Method: A systematic review and Bayesian network meta-analysis was conducted on only randomized controlled trials (RCTs) of all the available interventions for BPSD. RCTs were selected from Pubmed, EMBASE, the Cochrane library, and CINAHL. Read More

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http://dx.doi.org/10.1007/s00415-019-09200-8DOI Listing
January 2019

Expanding the importance of HMERF titinopathy: new mutations and clinical aspects.

J Neurol 2019 Jan 21. Epub 2019 Jan 21.

Department of Neurology, Neuromuscular Research Center, Tampere University Hospital and University of Tampere, 33014, Tampere, Finland.

Objective: Hereditary myopathy with early respiratory failure (HMERF) is caused by titin A-band mutations in exon 344 and considered quite rare. Respiratory insufficiency is an early symptom. A collection of families and patients with muscle disease suggestive of HMERF was clinically and genetically studied. Read More

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http://link.springer.com/10.1007/s00415-019-09187-2
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http://dx.doi.org/10.1007/s00415-019-09187-2DOI Listing
January 2019
3 Reads

Treatment with alemtuzumab or rituximab after fingolimod withdrawal in relapsing-remitting multiple sclerosis is effective and safe.

J Neurol 2019 Jan 19. Epub 2019 Jan 19.

Department of Neurology, La Fe Hospital, 106 Avenue Fernando Abril Martorell, 46026, Valencia, Spain.

Background: It has been described that treating relapsing-remitting multiple sclerosis (RRMS) patients with alemtuzumab following fingolimod could be less effective due to the different dynamics of lymphocyte repopulation. Effectiveness and safety of alemtuzumab compared to rituximab after fingolimod withdrawal were analyzed.

Patients And Methods: A follow-up of a cohort of RRMS patients treated with alemtuzumab or rituximab after fingolimod withdrawal was accomplished. Read More

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http://dx.doi.org/10.1007/s00415-019-09195-2DOI Listing
January 2019
1 Read

Sensor-based algorithmic dosing suggestions for oral administration of levodopa/carbidopa microtablets for Parkinson's disease: a first experience.

J Neurol 2019 Jan 18. Epub 2019 Jan 18.

Department of Micro-data Analysis, Dalarna University, 79 131, Falun, Sweden.

Objective: Dosing schedules for oral levodopa in advanced stages of Parkinson's disease (PD) require careful tailoring to fit the needs of each patient. This study proposes a dosing algorithm for oral administration of levodopa and evaluates its integration into a sensor-based dosing system (SBDS).

Materials And Methods: In collaboration with two movement disorder experts a knowledge-driven, simulation based algorithm was designed and integrated into a SBDS. Read More

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http://dx.doi.org/10.1007/s00415-019-09183-6DOI Listing
January 2019

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

Factors interfering with parenthood decision-making in an Italian sample of people with multiple sclerosis: an exploratory online survey.

J Neurol 2019 Jan 16. Epub 2019 Jan 16.

First Division of Neurology, University of Campania Luigi Vanvitelli, Piazza Miraglia, 2, 80138, Naples, Italy.

Background: Little is known about the influence of multiple sclerosis (MS) diagnosis on parenthood attitude in people with MS (pwMS).

Objective: To investigate the impact of diagnosis, clinical features and external disease-related influences on parenthood decision-making in Italian pwMS.

Methods: A web-based survey was posted on SMsocialnetwork. Read More

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http://dx.doi.org/10.1007/s00415-019-09193-4DOI Listing
January 2019

High efficacy of rituximab for myasthenia gravis: a comprehensive nationwide study in Austria.

J Neurol 2019 Jan 16. Epub 2019 Jan 16.

Department of Neurology, Medical University Innsbruck, Innsbruck, Austria.

Background: Most patients with myasthenia gravis (MG) need long-term immunosuppressive therapy. However, conventional agents may have intolerable side effects, take too long or fail to achieve disease control. Rituximab (RTX) has emerged as an off-label treatment for refractory MG, but data on its use are still sparse. Read More

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http://dx.doi.org/10.1007/s00415-019-09191-6DOI Listing
January 2019
2 Reads

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

J Neurol 2019 Jan 14. 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
January 2019
2 Reads

Peripheral neuropathy in hereditary spastic paraplegia caused by REEP1 variants.

J Neurol 2019 Jan 12. Epub 2019 Jan 12.

Department of Neurology, Rigshospitalet, Neuromuscular Research Center, University of Copenhagen, Copenhagen, Denmark.

SPG31 is a hereditary spastic paraplegia (HSP) caused by pathogenic variants in the REEP1 gene. The phenotype (SPG31) has occasionally been described with peripheral nervous system involvement, in additional to the gradually progressing lower limb spasticity that characterizes HSP. The objective of this study was to characterize patients with pathogenic REEP1 variants and neurophysiologically assess the extent of peripheral nerve involvement in this patient group. Read More

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http://link.springer.com/10.1007/s00415-019-09196-1
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http://dx.doi.org/10.1007/s00415-019-09196-1DOI Listing
January 2019
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Long-term tolerability, safety and efficacy of rituximab in neuromyelitis optica spectrum disorder: a prospective study.

J Neurol 2019 Jan 11. Epub 2019 Jan 11.

Department of Neurobiology, Institute of Molecular Medicine, Institute of Regional Health Research, University of Southern Denmark, Odense, Denmark.

Background: Neuromyelitis optica spectrum disorder (NMOSD) is a B-cell-mediated disease with autoimmunity towards the astrocyte water channel aquaporin-4 (AQP-4) in the central nervous system.

Objective: To assess the long-term safety and efficacy in NMOSD patients receiving maintenance therapy with B-cell-depleting agent rituximab for more than 2 years.

Method: NMOSD patients were included prospectively from 2014 to 2018 and received continuous cycles of rituximab infusions biannually. Read More

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http://dx.doi.org/10.1007/s00415-019-09180-9DOI Listing
January 2019

Subjective cognitive decline and progression to dementia in Parkinson's disease: a long-term follow-up study.

J Neurol 2019 Jan 11. Epub 2019 Jan 11.

School of Psychology, University of La Laguna, 38205, La Laguna, Tenerife, Spain.

Introduction: Increasing evidence suggests that subjective cognitive decline is associated with Alzheimer's disease pathology and with an increased risk for future dementia development. However, the clinical value of subjective cognitive decline in Parkinson's disease (PD-SCD) is unclear. The aim of the present work was to characterize PD-SCD and its progression to dementia. Read More

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http://dx.doi.org/10.1007/s00415-019-09197-0DOI Listing
January 2019

Mills' syndrome revisited.

J Neurol 2019 Jan 10. Epub 2019 Jan 10.

Institute of Neuroscience, Medical School, Newcastle University, Framlington Place, Newcastle upon Tyne, NE2 4HH, UK.

Mills' syndrome is an idiopathic, slowly progressive, spastic hemiparesis. We describe three cases that have been under review for a minimum of 11 years (range 11-19). In all patients, symptoms started in a leg, with a mean age of onset of 59 years (range 53-63). Read More

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http://dx.doi.org/10.1007/s00415-019-09186-3DOI Listing
January 2019
5 Reads