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Journal of Neurology

Poonam Zham, Dinesh Kumar, Rekha Viswanthan, Kit Wong, Kanae J Nagao, Sridhar Poosapadi Arjunan, Sanjay Raghav, Peter Kempster
Levodopa treatment does improve Parkinson's disease (PD) dysgraphia, but previous research is not in agreement about which aspects are most responsive. This study investigated the effect of levodopa on the kinematics of writing. Twenty-four patients with PD of less than 10 years duration and 25 age-matched controls were recruited. A practically defined off state method was used to assess the levodopa motor response, measured on the Unified Parkinson's Disease Rating Scale Part III. The kinematic features for six handwriting tasks involving different levels of complexity were recorded from PD patients in off and on states and from the control group...
March 15, 2019: Journal of Neurology
A Al-Ansari, N P Robertson
No abstract text is available yet for this article.
March 15, 2019: Journal of Neurology
Helle Petri, Karim Wahbi, Nanna Witting, Lars Køber, Henning Bundgaard, Emna Kamoun, Geoffroy Vellieux, Tanya Stojkovic, Anthony Béhin, Pascal Laforet, John Vissing
BACKGROUND: To evaluate the prevalence of cardiac involvement in patients with congenital myopathies and the association to specific genotypes. METHODS: We evaluated patients with physical examination, electrocardiogram, echocardiography, and 48-h Holter monitoring. Follow-up was performed for major events. RESULTS: We included 130 patients, 55 men (42%), with a mean age of 34 ± 17 years. A genetic diagnosis was established in 97 patients (75%)...
March 14, 2019: Journal of Neurology
Toshio Shimizu, Yuki Nakayama, Chiharu Matsuda, Michiko Haraguchi, Kota Bokuda, Kazuko Ishikawa-Takata, Akihiro Kawata, Eiji Isozaki
BACKGROUND: Body weight reduction after disease onset is an independent predictor of survival in amyotrophic lateral sclerosis (ALS), but significance of weight variation after diagnosis remains to be established. OBJECTIVE: To investigate weight variation after diagnosis and its prognostic significance in patients with ALS as a prospective cohort study. METHODS: Seventy-nine patients with ALS were enrolled in this study. At the time of diagnosis and about 1 year later, we evaluated the following parameters: age, sex, onset age, onset region, body mass index (BMI) and premorbid BMI, forced vital capacity and the revised ALS functional rating scale...
March 13, 2019: Journal of Neurology
Markus Kraemer, Jan Claudius Schwitalla, Frank Diesner, Orhan Aktas, Hans-Peter Hartung, Peter Berlit
INTRODUCTION: Moyamoya angiopathy (MMA) is a rare vasopathy, especially among European Caucasians. Data about demographics, clinical presentation, comorbid conditions, radiological findings as well as laboratory and cerebral spinal fluid (CSF) data are sparse. METHODS: Patients with MMA treated in the Alfried Krupp Hospital, Essen, Germany, between 2010 and 2017 with focus on demographic, clinical, radiological and laboratory as well as CSF data were evaluated retrospectively...
March 13, 2019: Journal of Neurology
Akira Kuriyama, Jeffrey L Jackson
No abstract text is available yet for this article.
March 12, 2019: Journal of Neurology
Jessica Frau, Giancarlo Coghe, Lorena Lorefice, Giuseppe Fenu, Luigina Musu, Eleonora Cocco
BACKGROUND: No postmarketing randomised clinical trials are available about alemtuzumab, and real-world data are limited. We aimed to analyse the efficacy and safety of alemtuzumab in a single-centre cohort of patients with relapsing-remitting MS. METHODS: Patients who took alemtuzumab were enrolled. We collected the following data: age, sex, MS history, expanded disability status scale (EDSS), relapses, magnetic resonance imaging (MRI) parameters after alemtuzumab, and adverse events...
March 12, 2019: Journal of Neurology
Jan Rusz, Tereza Tykalová, Giulio Salerno, Serena Bancone, Johara Scarpelli, Maria Teresa Pellecchia
Although motor speech disorders represent an early and prominent clinical feature of multiple system atrophy (MSA), the potential usefulness of speech assessment as a diagnostic tool has not yet been explored. This cross-sectional study aimed to provide a comprehensive, objective description of motor speech function in the parkinsonian (MSA-P) and cerebellar (MSA-C) variants of MSA. Speech samples were acquired from 80 participants including 18 MSA-P, 22 MSA-C, 20 Parkinson's disease (PD), and 20 healthy controls...
March 11, 2019: Journal of Neurology
Jennifer Massey, Susan Walker, Malcolm Galloway, Suran Fernando, John Parratt, Ann-Marie Quigley, Desmond P Kidd
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.
March 11, 2019: Journal of Neurology
Gargi Banerjee, Edgar Chan, Gareth Ambler, Duncan Wilson, Lisa Cipolotti, Clare Shakeshaft, Hannah Cohen, Tarek Yousry, Gregory Y H Lip, Keith W Muir, Martin M Brown, Hans Rolf Jäger, David J Werring
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. Cognitive impairment was defined as MoCA < 26...
March 7, 2019: Journal of Neurology
Atsushi Hashizume, Masahisa Katsuno, Keisuke Suzuki, Haruhiko Banno, Yu Takeuchi, Motoshi Kawashima, Noriaki Suga, Tomoo Mano, Amane Araki, Yasuhiro Hijikata, Akihiro Hirakawa, Gen Sobue
BACKGROUND: Spinal and bulbar muscular atrophy (SBMA) is an adult-onset, hereditary neuromuscular disease characterized by muscle atrophy, weakness, contraction fasciculation, and bulbar involvement. Although the causative gene, androgen receptor, has been identified, the development of novel therapeutics for SBMA is incomplete. In this study, the efficacy and safety of leuprorelin acetate administration for patients with SBMA, using the pooled data of two randomized-controlled trials, was studied...
March 7, 2019: Journal of Neurology
Federica Montagnese, Haris Babačić, Peter Eichhorn, Benedikt Schoser
BACKGROUND: Myositis-associated antibodies (MAA) and myositis-specific antibodies (MSA) are detected in patients with idiopathic inflammatory myopathies (IIM); their role as diagnostic biomarkers is however still debated. The aim of our study was to assess the utility of MAA/MSA assessed by new line immunoassays in detecting myositis among neuromuscular patients. METHODS: We retrospectively analysed sera samples obtained from patients tested for myositis antibodies with the "Euroline: Autoimmune Inflammatory Myopathies 16Ag" and "myositis profile 3" kits (Mi-2, TIF1γ, MDA5, NXP2, SAE1, Jo-1, SRP, PL-7/12, EJ, OJ, Ro-52, Ku, PM-Scl75/100)...
March 6, 2019: Journal of Neurology
Adam P Vogel, Lisa H Stoll, Andreas Oettinger, Natalie Rommel, Eva-Maria Kraus, Dagmar Timmann, Dion Scott, Christina Atay, Elsdon Storey, Ludger Schöls, Matthis Synofzik
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)...
March 6, 2019: Journal of Neurology
Sebastian Zaremba, Luca Albus, Patrick Schuss, Hartmut Vatter, Thomas Klockgether, Erdem Güresir
OBJECTIVES: Recent retrospective studies found sleep disorders, including obstructive sleep apnea and its symptoms to occur more often in patients following aneurysmal subarachnoid hemorrhage, but studies investigating the incidence of subarachnoid hemorrhage in patients with diagnosed obstructive sleep apnea [OSA] compared to other sleep disorders are missing. METHODS: To test our hypothesis that aneurysmal subarachnoid hemorrhage occurs more often in patients with OSA compared to other sleep disorders, we analyzed clinical data of 5514 patients with OSA, 4150 with other sleep disorders, and 964 patients with aneurysmal subarachnoid hemorrhage diagnosed between 01/01/2007 and 12/31/2016...
March 5, 2019: Journal of Neurology
José Gazulla, Isidro Ferrer, Silvia Izquierdo-Alvarez, Sara Alvarez, Rocío Sánchez-Alcudia, María Bestué-Cardiel, María Seral, Isabel Benavente, Esther Sierra-Martínez, José Berciano
OBJECTIVE: To report a kindred with an association between hereditary primary lateral sclerosis (PLS) and progressive nonfluent aphasia. PATIENTS AND METHODS: Six members from a kindred with 15 affected individuals spanning three generations, suffered from spasticity without muscle atrophy or fasciculation, starting in the lower limbs and spreading to the upper limbs and bulbar musculature, followed by effortful speech, nonfluent language and dementia, in 5 deceased members...
March 5, 2019: Journal of Neurology
Tatiana Aldaz, Pasquale Nigro, Almudena Sánchez-Gómez, Celia Painous, Lluís Planellas, Pilar Santacruz, Ana Cámara, Yaroslau Compta, Francesc Valldeoriola, Maria J Martí, Esteban Muñoz
BACKGROUND/AIMS: The presence of non-motor symptoms in Huntington's disease (HD) has not been systematically assessed so far. Our objective was to know their prevalence and to compare it with a cohort of patients with Parkinson's disease (PD). MATERIALS AND METHODS: Participants were consecutively recruited from our outpatient clinic. They were assessed through the motor part of the Unified Huntington's Disease Rating Scale, the motor part of the Unified Parkinson's Disease Rating Scale, the total functional capacity scale and the PD non-motor symptoms questionnaire...
March 5, 2019: Journal of Neurology
Aurélie L Manuel, David Foxe, Nathan Bradshaw, Nicholas J Cordato, John R Hodges, James R Burrell, Olivier Piguet
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...
March 5, 2019: Journal of Neurology
Tim Godel, Philipp Bäumer, Katharina Stumpfe, Nicole Muschol, Moritz Kronlage, Merle Brunnée, Jennifer Kollmer, Sabine Heiland, Martin Bendszus, Victor-Felix Mautner
PURPOSE: To examine dorsal root ganglia and proximal nerve segments in patients carrying the Fabry-related GLA-gene variant p.D313Y in comparison to patients with classical Fabry mutations and healthy controls by morphometric and functional magnetic resonance neurography. METHODS: This prospective multicenter study examines the lumbosacral dorsal root ganglia and sciatic nerve in 11 female p.D313Y patients by a standardized magnetic resonance neurography protocol at 3 T...
March 4, 2019: Journal of Neurology
Yumika Kotaki, Yasutake Tomata, Fumiya Tanji, Shu Zhang, Yumi Sugawara, Ichiro Tsuji
BACKGROUND: This cohort study estimated the population attributable fraction (PAF) of seven combined major risk factors for incident dementia. METHODS: We conducted a cohort study of 8563 community-dwelling individuals aged ≥ 65 years. In a baseline survey (2006), we collected data on major seven risk factors: diabetes mellitus, hypertension, obesity, physical inactivity, severe psychological distress, smoking, and low educational attainment. The total number of risk factors was applied as an exposure variable...
March 2, 2019: Journal of Neurology
Sarah Benghanem, Charlotte Rosso, Céline Arbizu, Eric Moulton, Didier Dormont, Anne Leger, Christine Pires, Yves Samson
OBJECTIVES: The outcome of aphasia at 3 months is variable in patients with moderate/severe stroke. The aim was to predict 3-month aphasia outcome using prediction models including initial severity in addition to the interaction between lesion size and location at the acute phase. METHODS: Patients with post-stroke aphasia (assessed by the Aphasia Rapid Test at day 7-ART D7) and MRI performed at day 1 were enrolled (n = 73). Good outcome at 3-months was defined by an Aphasia Handicap Score of 0-2...
February 28, 2019: Journal of Neurology
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