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Journal of Neurology

Kenya Nishioka, Michimasa Suzuki, Madoka Nakajima, Takeshi Hara, Masako Iseki, Nobutaka Hattori
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. All patients first presented persistent pain prior to the onset of motor symptoms. Each patient was examined by neurological investigation, neuro-imaging methods including brain magnetic resonance imaging (MRI) and electrophysiological methods...
January 14, 2019: Journal of Neurology
Anders Toft, Steffen Birk, Martin Ballegaard, Morten Dunø, Lena E Hjermind, Jørgen E Nielsen, Kirsten Svenstrup
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. Thirty-eight index cases were molecular-genetically tested, yielding two previously reported pathogenic REEP1 variants and a novel missense variant, in a total of four index patients...
January 12, 2019: Journal of Neurology
V Shaygannejad, E Fayyazi, S Badihian, O Mirmosayyeb, N Manouchehri, F Ashtari, N Asgari
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...
January 11, 2019: Journal of Neurology
Iván Galtier, Antonieta Nieto, Jesús N Lorenzo, José Barroso
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. METHODS: Forty-three PD patients and twenty normal controls were evaluated with a neuropsychological protocol...
January 11, 2019: Journal of Neurology
Stephan R Jaiser, Dipayan Mitra, Timothy L Williams, Mark R Baker
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). The only abnormality on laboratory investigations was a mildly elevated CSF protein in one case. MRI demonstrated focal T2 hyper-intensity located eccentrically in the cervical cord ipsilateral to the symptomatic side. No cerebral abnormality was demonstrated...
January 10, 2019: Journal of Neurology
François Sellal, Benjamin Cretin, Mariano Musacchio, Marie-Camille Berthel, Gabriela Carelli, Jean-Marc Michel
We describe the case of a 58-year-old patient with complete callosal agenesis, who developed after a stroke a long lasting and distressing diagonistic dyspraxia. We found two original treatments to relieve the patient from his left limb conflicting movements. Reinforcing left arm sensory input minimized dyspraxic movements but was difficult to apply daily and was found unsatisfactory by the patient. As left anarchic, unwished movements of diagonistic dyspraxia have been attributed to the lack of inhibition exerted by supplementary motor area on right motor cortex, we applied sham-controlled rTMS to the right motor cortex...
January 10, 2019: Journal of Neurology
Jeong-Min Kim, Jae-Han Bae, Kwang-Yeol Park, Woong Jae Lee, Jun Soo Byun, Suk-Won Ahn, Hae-Won Shin, Su-Hyun Han, Il-Han Yoo
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...
January 10, 2019: Journal of Neurology
Johannes Kaesmacher, Panagiotis Chaloulos-Iakovidis, Leonidas Panos, Pasquale Mordasini, Mirjam R Heldner, Christoph C Kurmann, Patrik Michel, Steven D Hajdu, Marc Ribo, Manuel Requena, Christian Maegerlein, Benjamin Friedrich, Vincent Costalat, Amel Benali, Laurent Pierot, Matthias Gawlitza, Joanna Schaafsma, Vitor Mendes Pereira, Jan Gralla, Urs Fischer
BACKGROUND AND PURPOSE: If patients presenting with large vessel occlusions (LVO) and mild symptoms should be treated with endvoascular treatment (EVT) remains unclear. Aims of this study were (1) assessing the safety and technical efficacy of EVT in patients with NIHSS < 8 as opposed to a comparison group of patients presenting with NIHSS ≥ 8 and (2) evaluation of the clinical effect of reperfusion in patients with NIHSS < 8. METHODS: Patients included into the retrospective multicenter BEYOND-SWIFT registry (NCT03496064) were analyzed...
January 8, 2019: Journal of Neurology
Ari Breiner, David Moher, John Brooks, Wei Cheng, Harald Hegen, Florian Deisenhammer, Christopher R McCudden, Pierre R Bourque
BACKGROUND: Cerebrospinal fluid total protein (CSF-TP) is measured in the diagnosis of a range of immune or infectious disorders of the nervous system. Most laboratories and the medical literature use an antiquated, age-independent upper limit of 0.45 g/L. Therefore, we performed a systematic review of reference studies in the medical literature, with the primary objective of determining the CSF total protein upper reference limit (URL). Secondary objectives were to assess the effects of age, gender, laboratory methods, and methodological quality...
January 8, 2019: Journal of Neurology
Marco Pasi, Andreas Charidimou, Gregoire Boulouis, Panagiotis Fotiadis, Andrea Morotti, Li Xiong, Sandro Marini, Alison Ayres, Matthew P Frosch, Joshua N Goldstein, Jonathan Rosand, M Edip Gurol, Steven M Greenberg, Anand Viswanathan
BACKGROUND: Spontaneous cerebellar-intracerebral hemorrhage (ICH) can be associated with both cerebral amyloid angiopathy (CAA) and hypertensive small vessel disease (HTN-SVD, i.e. arteriolosclerosis). To better understand the underlying microangiopathy of cerebellar-ICH, we aimed to evaluate the spatial distribution of supratentorial cerebral microbleeds (CMBs) and neuropathologic profiles in these patients. METHODS: We enrolled consecutive cerebellar-ICH patients...
January 8, 2019: Journal of Neurology
Thomas Decramer, Elsie Premereur, Lieven Lagae, Johannes van Loon, Peter Janssen, Stefan Sunaert, Tom Theys
The concept of functional modularity in human visual processing was proposed 25 years ago with the distinction between a ventral pathway for object recognition and a dorsal pathway for action processing. Lesions along these pathways yield selective deficits. A 15-year-old patient (MW) presented with a seizure due to a lesion in the left occipitotemporal cortex. Surgical resection of the lesion was performed with sparing of the classic language areas and visual fields. Postoperatively MW had great difficulty reading and had a specific agnosia for more complex visual stimuli in the right hemifield...
January 7, 2019: Journal of Neurology
Ángel Sesar, Gustavo Fernández-Pajarín, Begoña Ares, José-Luis Relova, Eduardo Arán, María-Teresa Rivas, Miguel Gelabert-González, Alfonso Castro
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. METHODS: We undertook a retrospective analysis of all patients treated with DBS and APO at our centre over 12 years. Subjects were allocated to four groups: (1) APO temporarily before DBS, (2) APO after DBS complications before a new DBS, (3) APO after definitive DBS removal, and (4) APO in patients with DBS and declining response...
January 7, 2019: Journal of Neurology
Benjamin Roeben, Dominik Blum, Heinz Gabriel, Matthis Synofzik
No abstract text is available yet for this article.
January 7, 2019: Journal of Neurology
Kristina Stahl, Emanuele Rastelli, Benedikt Schoser
BACKGROUND: Rhabdomyolysis (RML) is an interdisciplinary condition due to muscle cell injury followed by the release of cell components into circulation. Etiology of RML has a broad range; a serious complication is acute kidney injury (AKI). Despite its high relevance, there is no established formal definition for RML. OBJECTIVES: A systematic review, focusing on RML definition, providing a recommendation for clinicians. METHOD: Systematic literature research in PubMed and Embase (1968-07/2018)...
January 7, 2019: Journal of Neurology
Jussi O T Sipilä, Merja Soilu-Hänninen, Päivi Rautava, Ville Kytö
OBJECTIVE: To investigate if progressive multifocal leucoencephalopathy (PML) incidence has increased in Finland like in the neighbouring Sweden. METHODS: National administrative registries were searched for all PML admissions aged 16 years or more in 2004-2014 on all neurological and internal medicine wards in Finland. The mortality data of the patients was extracted from the national causes of death registry. National level data on annual predisposing drug use was obtained from the national pharmaceutical authority...
January 5, 2019: Journal of Neurology
Tao Wang, Kun Yang, Jichang Luo, Peng Gao, Yan Ma, Yabing Wang, Long Li, Yiran Liu, Yao Feng, Xue Wang, Liqun Jiao
BACKGROUND: Stenting is a common endovascular therapy for symptomatic intracranial arterial stenosis (ICAS). We sought to update the evaluation of global short-term safety and long-term efficacy outcomes after stenting for symptomatic ICAS and explore their distributional characteristics. METHODS: Major databases including Cochrane Library, MEDLINE, EMBASE were systematically searched from January 1st, 2005, for RCTs and observational studies which reported short- and long-term outcomes after stenting for symptomatic ICAS...
January 5, 2019: Journal of Neurology
Kwang-Dong Choi, Seo Young Choi, Jae-Hwan Choi, Seong-Hee Kim, Seung-Han Lee, Seong-Hae Jeong, Hyo-Jung Kim, Jeong-Yoon Choi, Ji-Soo Kim
The original version of this article unfortunately contained a mistake.
January 4, 2019: Journal of Neurology
Tsung-Han Ho, Fu-Chi Yang, Ruei-Ching Lin, Wu-Chien Chien, Chi-Hsiang Chung, Shang-Lin Chiang, Chung-Hsing Chou, Chia-Kuang Tsai, Chia-Lin Tsai, Yu-Kai Lin, Jiunn-Tay Lee
BACKGROUND AND OBJECTIVES: To date, the efficacy of the virtual reality (VR) application for acute stroke compared with conventional therapy (CT) remains unclear. This retrospective study aims to assess the impact of adjuvant VR technology on multidimensional therapy for patients with acute-stage stroke. METHODS: 100 acute ischemic stroke patients with onset within 7 days who underwent combined adjuvant VR-based rehabilitation program and CT (intervention group-VR + CT) were compared to an equal number of cross-matched patients who received CT alone...
January 4, 2019: Journal of Neurology
Annalisa Parente, Chiara Pastori, Flavio Villani, Anna Rita Giovagnoli
No abstract text is available yet for this article.
January 4, 2019: Journal of Neurology
Hans-Peter Hartung, Jonas Graf, David Kremer
Multiple sclerosis (MS) is an autoimmune disease of the central nervous system (CNS) with a diverse disease course involving inflammation and degeneration of neurons and axons. Multiple sclerosis results from a complex interaction of genetic and environmental factors and clinically several disease subtypes with marked variation in symptoms can be discerned. Disease-modifying therapies (DMTs) impact disease activity and outcome. Long-term follow-up studies of DMTs in MS have generally shown that the short-term effects in clinical trials are maintained for up to 21 years, e...
January 4, 2019: Journal of Neurology
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