644 results match your criteria Ann Clin Transl Neurol[Journal]


Plasma alpha-synuclein detected by single molecule array is increased in PD.

Ann Clin Transl Neurol 2019 Mar 15;6(3):615-619. Epub 2019 Feb 15.

Department of Neurology National Neuroscience Institute Tan Tock Seng Hospital 11 Jalan Tan Tock Seng Singapore 308433 Singapore.

We utilized ultrasensitive single molecule technology to measure plasma alpha-synuclein in 221 subjects (51 controls, 170 PD). Plasma alpha-synuclein levels were significantly higher in PD than controls (15506.3 vs. Read More

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http://dx.doi.org/10.1002/acn3.729DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6414476PMC
March 2019
1 Read

A novel variant causes pure hereditary spastic paraplegia with benign clinical course.

Ann Clin Transl Neurol 2019 Mar 4;6(3):610-614. Epub 2019 Jan 4.

Department of Neurology Peking University People's Hospital Beijing China.

Hereditary spastic paraplegia 73 (SPG73) was currently identified in only one family with variant in the neuronal isoform of carnitine palmitoyl-transferase 1C () gene. We described a new family, in which affected individuals exhibited pure hereditary spastic paraplegia with benign clinical course. Exome sequencing revealed a novel nonsense variant in the gene. Read More

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http://dx.doi.org/10.1002/acn3.717DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6414484PMC
March 2019
1 Read

A likely pathogenic variant in the gene presenting with progressive myoclonus.

Ann Clin Transl Neurol 2019 Mar 1;6(3):605-609. Epub 2019 Feb 1.

Center for Medical Genetics Ghent University Hospital Ghent Belgium.

A 60-year-old man is presented with progressive involuntary muscle movements and neuropsychiatric symptoms who developed a variety of additional complaints over 2 years. Brain imaging revealed bilateral basal ganglia calcifications suggesting primary familial brain calcification. Analysis of the gene revealed a missense mutation (c. Read More

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http://doi.wiley.com/10.1002/acn3.702
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http://dx.doi.org/10.1002/acn3.702DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6414494PMC
March 2019
4 Reads

Motor-imagery ability and function of hemiplegic upper limb in stroke patients.

Ann Clin Transl Neurol 2019 Mar 17;6(3):596-604. Epub 2019 Feb 17.

Neurorehabilitation Research Center Kio University 4-2-2 Umaminaka, Koryo, Kitakatsuragi-gun Nara 635-0832 Japan.

Objectives: We quantitatively examined the motor-imagery ability in stroke patients using a bimanual circle-line coordination task (BCT) and clarified the relationship between motor-imagery ability and motor function of hemiplegic upper limbs and the level of use of paralyzed limbs.

Methods: We enrolled 31 stroke patients. Tasks included unimanual-line (U-L)-drawing straight lines on the nonparalyzed side; bimanual circle-line (B-CL)-drawing straight lines with the nonparalyzed limb while drawing circles with the paralyzed limb; and imagery circle-line (I-CL)-drawing straight lines on the nonparalyzed side during imagery drawing on the paralyzed side, using a tablet personal computer. Read More

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http://dx.doi.org/10.1002/acn3.739DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6414480PMC

Quantitative vibratory sensation measurement is related to sensory cortical thickness in MS.

Ann Clin Transl Neurol 2019 Mar 19;6(3):586-595. Epub 2019 Feb 19.

Center for Movement Studies Kennedy Krieger Institute Baltimore Maryland.

Objective: Vibratory sensation is a quantifiable measure of physical dysfunction and is often related to spinal cord pathology; however, its association with relevant brain areas has not been fully explored. Our objective was to establish a cortical structural substrate for vibration sensation.

Methods: Eighty-four individuals with multiple sclerosis (MS) ( = 54 relapsing,  = 30 progressive) and 28 controls participated in vibratory sensation threshold quantification at the great toe and a 3T MRI evaluating volume of the thalamus and cortical thickness primary and secondary sensory cortices. Read More

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http://doi.wiley.com/10.1002/acn3.734
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http://dx.doi.org/10.1002/acn3.734DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6414478PMC
March 2019
5 Reads

Neuroimaging evolution of ischemia in men and women: an observational study.

Ann Clin Transl Neurol 2019 Mar 21;6(3):575-585. Epub 2019 Feb 21.

Department of Neurology Dell Medical School at The University of Texas Austin Texas.

Objective: We present an exploratory study for identification of sex differences in imaging biomarkers that could further refine selection of patients for acute reperfusion therapy and trials based on sex and imaging targets.

Methods: The Lesion Evolution in Stroke and Ischemia On Neuroimaging (LESION) study included consecutive acute stroke patients who underwent MRI within 24 h of time from last known well and prior to therapy. Those demonstrating a potential therapeutic target on imaging were identified by presence of: (1) arterial occlusion on angiography, (2) focal ischemic region on perfusion maps, or (3) a mismatch of perfusion versus diffusion imaging lesion size. Read More

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http://doi.wiley.com/10.1002/acn3.733
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http://dx.doi.org/10.1002/acn3.733DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6414486PMC
March 2019
5 Reads

Anti-PrP antibody rescues cognition and synapses in transgenic alzheimer mice.

Ann Clin Transl Neurol 2019 Mar 27;6(3):554-574. Epub 2019 Feb 27.

Cellular Neuroscience Neurodegeneration & Repair Departments of Neurology and of Neuroscience Yale University School of Medicine New Haven 06536 Connecticut.

Objective: Amyloid-beta oligomers (Aßo) trigger the development of Alzheimer's disease (AD) pathophysiology. Cellular prion protein (PrPC) initiates synaptic damage as a high affinity receptor for Aßo. Here, we evaluated the preclinical therapeutic efficacy of a fully human monoclonal antibody against PrPC. Read More

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http://doi.wiley.com/10.1002/acn3.730
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http://dx.doi.org/10.1002/acn3.730DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6414488PMC
March 2019
3 Reads

Randomized, double-blind, placebo-controlled study of interferon- 1b in Friedreich Ataxia.

Ann Clin Transl Neurol 2019 Mar 27;6(3):546-553. Epub 2019 Feb 27.

Friedreich's Ataxia Research Alliance 533 W Uwchlan Ave Downingtown Pennsylvania 19335.

Objective: In vitro, in vivo, and open-label studies suggest that interferon gamma (IFN- 1b) may improve clinical features in Friedreich Ataxia through an increase in frataxin levels. The present study evaluates the efficacy and safety of IFN- 1b in the treatment of Friedreich Ataxia through a double-blind, multicenter, placebo-controlled trial.

Methods: Ninety-two subjects with FRDA between 10 and 25 years of age were enrolled. Read More

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http://dx.doi.org/10.1002/acn3.731DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6414489PMC

A metabolomic approach to identifying biomarkers in blood of Alzheimer's disease.

Ann Clin Transl Neurol 2019 Mar 27;6(3):537-545. Epub 2019 Feb 27.

Department of Neurology Chang Gung Memorial Hospital at Linkou Medical Center Chang Gung University College of Medicine Taoyuan Taiwan.

Objective: This study aims to identify metabolites with altered levels of expression in patients with early and progressive stages of Alzheimer's disease (AD).

Methods: All participants of the study underwent genetic screening and were diagnosed using both neuropsychological assessment and amyloid imaging before metabolome analysis. According to these assessments, the patients were classified as normal ( = 15), with mild cognitive impairment ( = 10), and with AD ( = 15). Read More

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http://dx.doi.org/10.1002/acn3.726DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6414491PMC
March 2019
1 Read

Metabolomics-based identification of metabolic alterations in PARK2.

Ann Clin Transl Neurol 2019 Mar 21;6(3):525-536. Epub 2019 Feb 21.

Department of Neurology Juntendo University School of Medicine Tokyo Japan.

Objective: is the causative gene for autosomal recessive familial Parkinson's disease (PD), although it remains unclear how parkin dysfunction is involved with the general condition. Recently, serum and/or plasma metabolomics revealed alterations in metabolic pathways that might reflect pathomechanisms of idiopathic PD (iPD). Thus, we hypothesized that serum metabolomics of patients with homozygous or compound heterozygous mutations (namely, PARK2) might reflect metabolic alterations due to parkin dysfunction. Read More

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http://dx.doi.org/10.1002/acn3.724DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6414487PMC

Leigh syndrome caused by mutations in is associated with a better prognosis.

Ann Clin Transl Neurol 2019 Mar 17;6(3):515-524. Epub 2019 Feb 17.

Wellcome Centre for Mitochondrial Research Institute of Neuroscience Newcastle University Newcastle upon Tyne United Kingdom.

Objectives: Mitochondrial methionyl-tRNA formyltransferase (MTFMT) is required for the initiation of translation and elongation of mitochondrial protein synthesis Pathogenic variants in have been associated with Leigh syndrome (LS) and mitochondrial multiple respiratory chain deficiencies. We sought to elucidate the spectrum of clinical, neuroradiological and molecular genetic findings of patients with bi-allelic pathogenic variants in .

Methods: Retrospective cohort study combining new cases and previously published cases. Read More

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http://dx.doi.org/10.1002/acn3.725DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6414492PMC

Grades of brain arteriovenous malformations and risk of hemorrhage and death.

Ann Clin Transl Neurol 2019 Mar 22;6(3):508-514. Epub 2019 Jan 22.

Neurologist at the Institute of Neurology Columbia University Medical Center New York New York.

Objective: To assess the relationship of the grade of unruptured and untreated Brain Arteriovenous Malformations (AVMs), with the risk of subsequent stroke and death during follow-up.

Methods: This prospective study was drawn from a cohort of adult patients with unruptured AVMs, who participated in the conservative treatment arm (medical management only for headache or seizures) of the randomized clinical trial of unruptured brain AVMs (ARUBA study). The grade of AVMs (Spetzler-Martin scale) was dichotomized into categories: AVMs of grades I and II were considered low grade; AVMs of grades III and IV were considered high grade. Read More

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http://dx.doi.org/10.1002/acn3.723DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6414495PMC
March 2019
1 Read

A multicenter real-world review of 10 kHz SCS outcomes for treatment of chronic trunk and/or limb pain.

Ann Clin Transl Neurol 2019 Mar 22;6(3):496-507. Epub 2019 Jan 22.

Department of Stereotactic and Functional Neurosurgery Cologne Merheim Medical Center University of Witten/Herdecke Cologne Germany.

Objectives: High-frequency spinal cord stimulation (HF-SCS) at 10 kHz has proven to be efficacious in the treatment of chronic back and leg pain in a randomized, controlled, trial (SENZA-RCT). However, large observational studies have yet to be published. Therefore, we performed a real-world, multicenter, retrospective, review of therapy efficacy in 1660 patients with chronic trunk and/or limb pain. Read More

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http://dx.doi.org/10.1002/acn3.720DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6414485PMC

Eating peptides: biomarkers of neurodegeneration in amyotrophic lateral sclerosis and frontotemporal dementia.

Ann Clin Transl Neurol 2019 Mar 31;6(3):486-495. Epub 2019 Jan 31.

Memory and Cognition Clinic Institute of Clinical Neurosciences Royal Prince Alfred Hospital Sydney New South Wales Australia.

Objective: Physiological changes potentially influence disease progression and survival along the Amyotrophic Lateral Sclerosis (ALS)-Frontotemporal dementia (FTD) spectrum. The peripheral peptides that regulate eating and metabolism may provide diagnostic, metabolic, and progression biomarkers. The current study aimed to examine the relationships and biomarker potential of hormonal peptides. Read More

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http://dx.doi.org/10.1002/acn3.721DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6414477PMC
March 2019
2 Reads

Contribution of ultrarare variants in mTOR pathway genes to sporadic focal epilepsies.

Ann Clin Transl Neurol 2019 Mar 25;6(3):475-485. Epub 2019 Feb 25.

IRCCS Istituto delle Scienze Neurologiche di Bologna Bologna Italy.

Objective: We investigated the contribution to sporadic focal epilepsies (FE) of ultrarare variants in genes coding for the components of complexes regulating mechanistic Target Of Rapamycin (mTOR)complex 1 (mTORC1).

Methods: We collected genetic data of 121 Italian isolated FE cases and 512 controls by Whole Exome Sequencing (WES) and single-molecule Molecular Inversion Probes (smMIPs) targeting 10 genes of the GATOR1, GATOR2, and TSC complexes. We collapsed "qualifying" variants (ultrarare and predicted to be deleterious or loss of function) across the examined genes and sought to identify their enrichment in cases compared to controls. Read More

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http://dx.doi.org/10.1002/acn3.722DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6414475PMC
March 2019
7 Reads

Cognitive deficits in acute mild ischemic stroke and TIA and effects of rt-PA.

Ann Clin Transl Neurol 2019 Mar 22;6(3):466-474. Epub 2019 Jan 22.

Department of Neurology and Institute for Stroke and Cerebrovascular Disease UTHealth Houston Texas.

Introduction: It is unknown if treatment with rt-PA in mild acute ischemic stroke (MIS) is associated with improvement in long term cognition.

Methods: Forty-five patients with suspected acute mild stroke or transient ischemic attacks with NIHSS ≤6 were enrolled in a prospective cohort. Cognitive testing was performed within 24 h of symptom onset. Read More

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http://dx.doi.org/10.1002/acn3.719DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6414481PMC
March 2019
3 Reads

Polygenic risk and hazard scores for Alzheimer's disease prediction.

Ann Clin Transl Neurol 2019 Mar 18;6(3):456-465. Epub 2019 Feb 18.

MRC Centre for Neuropsychiatric Genetics and Genomics Cardiff University Cardiff United Kingdom.

Objective: Genome-wide association studies (GWAS) have identified over 30 susceptibility loci associated with Alzheimer's disease (AD). Using AD GWAS data from the International Genomics of Alzheimer's Project (IGAP), Polygenic Risk Score (PRS) was successfully applied to predict life time risk of AD development. A recently introduced Polygenic Hazard Score (PHS) is able to quantify individuals with age-specific genetic risk for AD. Read More

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http://dx.doi.org/10.1002/acn3.716DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6414493PMC

Cortical activation during sleep predicts dream experience in narcolepsy.

Ann Clin Transl Neurol 2019 Mar 1;6(3):445-455. Epub 2019 Feb 1.

Department of Psychology University of Rome "Sapienza" Rome Italy.

Objective: Narcolepsy type 1 widely affects the architecture of sleep with frequent fast transition to REM sleep at both nighttime and daytime sleep onset. The occurrence of repeated sleep onset REM periods over the Multiple Sleep Latency Test offers a unique opportunity to identify EEG patterns predictive of successful dream recall after short periods composed of only REM or NREM sleep. It also permits to disentangle state- from trait-like differences in dream recall, by using a within-subjects design. Read More

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http://dx.doi.org/10.1002/acn3.718DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6414482PMC
March 2019
1 Read

A novel model for treatment of hypertrophic pachymeningitis.

Ann Clin Transl Neurol 2019 Mar 15;6(3):431-444. Epub 2019 Jan 15.

Department of Neurology Neurological Institute Graduate School of Medical Sciences Kyushu University Fukuoka Japan.

Objective: Immunoglobulin (Ig)G4-related disease is a major cause of hypertrophic pachymeningitis (HP), presenting as a progressive thickening of the dura mater. HP lacks an animal model to determine its underlying mechanisms. We developed a suitable animal model for the treatment of HP. Read More

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http://dx.doi.org/10.1002/acn3.715DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6414490PMC
March 2019
1 Read

Hearing impairment after subarachnoid hemorrhage.

Ann Clin Transl Neurol 2019 Mar 8;6(3):420-430. Epub 2019 Jan 8.

Clinical Neurosciences Clinical and Experimental Sciences Faculty of Medicine University of Southampton Southampton United Kingdom.

Background: Subarachnoid hemorrhage (SAH) survivors experience significant neurological disability, some of which is under-recognized by neurovascular clinical teams. We set out to objectively determine the occurrence of hearing impairment after SAH, characterize its peripheral and/or central origin, and investigate likely pathological correlates.

Methods: In a case-control study ( = 41), participants were asked about new onset hearing difficulty 3 months post-SAH, compared with pre-SAH. Read More

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http://dx.doi.org/10.1002/acn3.714DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6414479PMC
March 2019
1 Read

Erratum: Whole-exome sequencing in 20,197 persons for rare variants in Alzheimer's disease.

Authors:

Ann Clin Transl Neurol 2019 Feb 25;6(2):416. Epub 2019 Feb 25.

[This corrects the article DOI: 10.1002/acn3.582. Read More

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http://dx.doi.org/10.1002/acn3.742DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6389743PMC
February 2019

Dopamine compartmentalization, selective dopaminergic vulnerabilities in Parkinson's disease and therapeutic opportunities.

Authors:
George R Uhl

Ann Clin Transl Neurol 2019 Feb 8;6(2):406-415. Epub 2019 Jan 8.

Neurology and Research Services New Mexico VA HealthCare System Albuquerque New Mexico 87108.

Progressive depletion of selected dopamine neurons is central to much Parkinson's disease (PD) disability. Although symptomatic treatments can ameliorate the disabilities that this neuronal depletion causes, no current strategy is documented to slow these losses. There is substantial evidence that dopamine in intracytoplasmic/extravesicular neuronal compartments can be toxic. Read More

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http://dx.doi.org/10.1002/acn3.707DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6389739PMC
February 2019

A novel mutation associated with recessive distal hereditary motor neuropathy.

Ann Clin Transl Neurol 2019 Feb 3;6(2):401-405. Epub 2018 Dec 3.

Department of Neurology Fujian Medical University Union Hospital Fuzhou 350001 China.

Vaccinia-related kinase 1 () mutations can cause motor phenotypes including axonal sensorimotor neuropathy, distal hereditary motor neuropathy (dHMN), spinal muscular atrophy, and amyotrophic lateral sclerosis. Here, we identify a novel homozygous p.W375X mutation causing recessive dHMN. Read More

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http://dx.doi.org/10.1002/acn3.701DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6389749PMC
February 2019
1 Read

Baylisascariasis: A young boy with neural larva migrans due to the emerging raccoon round worm.

Ann Clin Transl Neurol 2019 Feb 12;6(2):397-400. Epub 2018 Dec 12.

Department of Paediatrics B.C. Children's Hospital and The University of British Columbia Vancouver Canada.

A 17-month-old boy from Vancouver, Canada, presented with a 5-day history of progressive somnolence, ataxia, and torticollis. Additional investigations revealed eosinophilic encephalitis with deep white matter changes on MR imaging. On day 13, serology came back positive for antibodies. Read More

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https://onlinelibrary.wiley.com/doi/abs/10.1002/acn3.694
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http://dx.doi.org/10.1002/acn3.694DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6389752PMC
February 2019
11 Reads

Brain histopathological study and prognosis in MOG antibody-associated demyelinating pseudotumor.

Ann Clin Transl Neurol 2019 Feb 8;6(2):392-396. Epub 2019 Jan 8.

Department of Neurology The Third Affiliated Hospital of Sun Yat-Sen University Guangzhou 510630 China.

Our objective was to examine the brain biopsies by histopathology and investigate the prognosis of patients with myelin oligodendrocyte glycoprotein antibody-associated demyelinating pseudotumor. The clinical, MRI, and histological features of two patients with myelin oligodendrocyte glycoprotein antibody-associated demyelinating pseudotumor were reviewed. Both patients were treated with steroid plus rituximab and followed up. Read More

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http://dx.doi.org/10.1002/acn3.712DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6389737PMC
February 2019
2 Reads

Mild malformations of cortical development in sleep-related hypermotor epilepsy due to mutations.

Ann Clin Transl Neurol 2019 Feb 25;6(2):386-391. Epub 2018 Dec 25.

Danish Epilepsy Centre, Filadelfia Dianalund Denmark.

Mutations in the sodium-activated potassium channel gene have been associated with nonlesional sleep-related hypermotor epilepsy (SHE). We report the co-occurrence of mild malformation of cortical development (mMCD) and mutations in four patients with SHE. Focal cortical dysplasia type I was neuropathologically diagnosed after epilepsy surgery in three unrelated MRI-negative patients, periventricular nodular heterotopia was detected in one patient by MRI. Read More

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http://dx.doi.org/10.1002/acn3.708DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6389734PMC
February 2019

Longitudinal advanced MRI case report of white matter radiation necrosis.

Ann Clin Transl Neurol 2019 Feb 10;6(2):379-385. Epub 2018 Dec 10.

Department of Medicine Division of Neurology University of British Columbia Vancouver British Columbia Canada.

Radiation necrosis mostly occurs in and near the radiation field. We used magnetic resonance imaging to study radiation-induced necrosis of atypical onset, severity, and extent following stereotactic radiosurgery for a symptomatic arteriovenous malformation. Susceptibility-sensitive imaging, T-relaxation, myelin water imaging, and magnetic resonance spectroscopy were acquired three times up to 52 months postradiosurgery. Read More

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http://dx.doi.org/10.1002/acn3.704DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6389755PMC
February 2019
2 Reads

In vivo evidence for pre-symptomatic neuroinflammation in a MAPT mutation carrier.

Ann Clin Transl Neurol 2019 Feb 2;6(2):373-378. Epub 2019 Jan 2.

Department of Clinical Neurosciences University of Cambridge Cambridge United Kingdom.

Neuroinflammation occurs in frontotemporal dementia, however its timing relative to protein aggregation and neuronal loss is unknown. Using positron emission tomography and magnetic resonance imaging to quantify these processes in a pre-symptomatic carrier of the 10 + 16 MAPT mutation, we show microglial activation in frontotemporal regions, despite a lack of protein aggregation or atrophy in these areas. The distribution of microglial activation better discriminated the carrier from controls than did protein aggregation at this pre-symptomatic disease stage. Read More

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http://dx.doi.org/10.1002/acn3.683DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6389753PMC
February 2019

rt-PA with remote ischemic postconditioning for acute ischemic stroke.

Ann Clin Transl Neurol 2019 Feb 16;6(2):364-372. Epub 2019 Jan 16.

Beijing Key Laboratory of Hypoxia Conditioning Translational Medicine Xuanwu Hospital Capital Medical University Beijing China.

Objective: To investigate the feasibility and safety of remote ischemic postconditioning (RIPC) in acute ischemic stroke patients after intravenous recombinant tissue plasminogen activator (rt-PA) thrombolysis (IVT).

Methods: We performed a pilot randomized trial involving acute ischemic stroke patients with IVT. The patients were randomized 1:1 to receive RIPC or standard medical therapy. Read More

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http://dx.doi.org/10.1002/acn3.713DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6389851PMC
February 2019
1 Read

Teriflunomide induces a tolerogenic bias in blood immune cells of MS patients.

Ann Clin Transl Neurol 2019 Feb 15;6(2):355-363. Epub 2019 Jan 15.

Department of Immunology Hospital Universitario Ramón y Cajal IRYCIS Madrid Spain.

Objectives: Teriflunomide, a disease-modifying treatment approved for multiple sclerosis (MS), inhibits reversibly dihydroorotate dehydrogenase, an enzyme involved in de novo pyrimidine biosynthesis and down-regulates proliferation of activated lymphocytes. We aimed to study the impact of this drug in the lymphocyte profiles of MS patients.

Methods: Fifty-five patients with relapsing-remitting MS who initiated teriflunomide treatment were included in the study. Read More

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http://dx.doi.org/10.1002/acn3.711DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6389853PMC
February 2019
1 Read

Pain affect disrupted in children with posterior cerebellar tumor resection.

Ann Clin Transl Neurol 2019 Feb 2;6(2):344-354. Epub 2019 Jan 2.

Center for Pain and the Brain Department of Anesthesiology, Critical Care and Pain Medicine Boston Children's Hospital Harvard Medical School Boston Massachusetts 02215.

Objectives: Damage to the posterior cerebellum can cause affective deficits in patients. In adults, cerebellar infarcts result in thermal hyperalgesia and affect descending modulation of pain. This study evaluated the effect of resection of low-grade cerebellar tumors on pain processing in human children. Read More

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http://dx.doi.org/10.1002/acn3.709DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6389840PMC
February 2019

Slower alpha rhythm associates with poorer seizure control in epilepsy.

Ann Clin Transl Neurol 2019 Feb 18;6(2):333-343. Epub 2018 Dec 18.

Department of Basic and Clinical Neuroscience Institute of Psychiatry, Psychology and Neuroscience King's College London London United Kingdom.

Objective: Slowing and frontal spread of the alpha rhythm have been reported in multiple epilepsy syndromes. We investigated whether these phenomena are associated with seizure control.

Methods: We prospectively acquired resting-state electroencephalogram (EEG) in 63 patients with focal and idiopathic generalized epilepsy (FE and IGE) and 39 age- and gender-matched healthy subjects (HS). Read More

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http://dx.doi.org/10.1002/acn3.710DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6389754PMC
February 2019

Risk factors for respiratory failure in Guillain-Barré syndrome in Bangladesh: a prospective study.

Ann Clin Transl Neurol 2019 Feb 7;6(2):324-332. Epub 2019 Jan 7.

National Institute of Neurosciences and Hospital Dhaka Bangladesh.

Objective: We investigated clinical, biological, and electrophysiological risk factors for mechanical ventilation (MV) and patient outcomes in Bangladesh using one of the largest, prospective Guillain-Barré syndrome (GBS) cohorts in developing world.

Methods: A total of 693 GBS patients were included in two GBS studies conducted between 2006 and 2016 in Dhaka, Bangladesh. Associations between baseline characteristics and MV were tested using Fisher's exact test, χ test, or Mann-Whitney -test, as appropriate. Read More

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http://dx.doi.org/10.1002/acn3.706DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6389747PMC
February 2019
1 Read

Short-term remote ischemic conditioning may protect monkeys after ischemic stroke.

Ann Clin Transl Neurol 2019 Feb 15;6(2):310-323. Epub 2019 Jan 15.

Department of Neurology Xuanwu Hospital Capital Medical University Beijing China.

Objective: We aimed to evaluate the safety and effectiveness of short-term remote ischemic postconditioning (RIPC) in acute stroke monkey models.

Methods: Acute stroke monkeys were allocated to four groups based on the number of limbs exposed to RIPC. RIPC was initiated by 5-min cuff inflation/deflation cycles of the target limb(s) for 5-10 bouts. Read More

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http://dx.doi.org/10.1002/acn3.705DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6389742PMC
February 2019
1 Read

Drug repositioning in epilepsy reveals novel antiseizure candidates.

Ann Clin Transl Neurol 2019 Feb 11;6(2):295-309. Epub 2018 Dec 11.

Department of Pediatrics University of Iowa Iowa City Iowa.

Objective: Epilepsy treatment falls short in ~30% of cases. A better understanding of epilepsy pathophysiology can guide rational drug development in this difficult to treat condition. We tested a low-cost, drug-repositioning strategy to identify candidate epilepsy drugs that are already FDA-approved and might be immediately tested in epilepsy patients who require new therapies. Read More

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https://onlinelibrary.wiley.com/doi/abs/10.1002/acn3.703
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http://dx.doi.org/10.1002/acn3.703DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6389756PMC
February 2019
10 Reads

Real-time use of audio-biofeedback can improve postural sway in patients with degenerative ataxia.

Ann Clin Transl Neurol 2019 Feb 28;6(2):285-294. Epub 2018 Nov 28.

Department of Cognitive Neurology Hertie Institute for Clinical Brain Research Tübingen Germany.

Objective: Cerebellar ataxia essentially includes deficient postural control. It remains unclear whether augmented sensory information might help cerebellar patients, as the cerebellum underlies processing of various sensory modalities for postural control. Here, we hypothesized that patients with cerebellar degeneration can still exploit audio-biofeedback (ABF) of trunk acceleration as a real-time assistive signal to compensate for deficient postural control. Read More

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http://dx.doi.org/10.1002/acn3.699DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6389757PMC
February 2019

A new counterintuitive training for adult amblyopia.

Ann Clin Transl Neurol 2019 Feb 28;6(2):274-284. Epub 2018 Dec 28.

Department of Translational Research on New Technologies in Medicine and Surgery University of Pisa Pisa Italy.

Objectives: The aim of this study was to investigate whether short-term inverse occlusion, combined with moderate physical exercise, could promote the recovery of visual acuity and stereopsis in a group of adult anisometropic amblyopes.

Methods: Ten adult anisometropic patients underwent six brief (2 h) training sessions over a period of 4 weeks. Each training session consisted in the occlusion of the amblyopic eye combined with physical exercise (intermittent cycling on a stationary bike). Read More

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http://dx.doi.org/10.1002/acn3.698DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6389748PMC
February 2019

Antemortem CSF A42/A40 ratio predicts Alzheimer's disease pathology better than A42 in rapidly progressive dementias.

Ann Clin Transl Neurol 2019 Feb 14;6(2):263-273. Epub 2018 Dec 14.

IRCCS Istituto delle Scienze Neurologiche di Bologna Bologna 40139 Italy.

Objective: Despite the critical importance of pathologically confirmed samples for biomarker validation, only a few studies have correlated CSF A42 values in vivo with postmortem Alzheimer's disease (AD) pathology, while none evaluated the CSF A42/A40 ratio. We compared CSF A42 and A42/A40 ratio as biomarkers predicting AD neuropathological changes in patients with a short interval between lumbar puncture and death.

Methods: We measured CSF A40 and A42 and assessed AD pathology in 211 subjects with rapidly progressive dementia (RPD) and a definite postmortem diagnosis of Creutzfeldt-Jakob disease ( = 159), AD ( = 12), dementia with Lewy bodies (DLB,  = 4), AD/DLB mixed pathologies ( = 5), and various other pathologies ( = 31). Read More

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http://dx.doi.org/10.1002/acn3.697DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6389744PMC
February 2019
2 Reads

Natalizumab versus fingolimod and dimethyl fumarate in multiple sclerosis treatment.

Ann Clin Transl Neurol 2019 Feb 9;6(2):252-262. Epub 2018 Dec 9.

Division of Neuroimmunology Department of Neurology Rocky Mountain Multiple Sclerosis Center at the University of Colorado Aurora Colorado.

Objective: To compare 2-year effectiveness and discontinuation of natalizumab (NTZ) versus fingolimod (FTY) and dimethyl fumarate (DMF) in the treatment of multiple sclerosis (MS).

Methods: Patients prescribed NTZ, FTY, or DMF at the Rocky Mountain MS Center at University of Colorado were identified. Clinician-reported data were retrospectively collected. Read More

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http://dx.doi.org/10.1002/acn3.700DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6389745PMC
February 2019
5 Reads

miR-30e-5p as predictor of generalization in ocular myasthenia gravis.

Ann Clin Transl Neurol 2019 Feb 24;6(2):243-251. Epub 2019 Jan 24.

Department of Neuroscience Clinical Neurophysiology Uppsala University Uppsala Sweden.

Objective: To determine a predictive factor for the risk of conversion from ocular myasthenia gravis (OMG) to generalized MG (GMG) in a prospective study.

Methods: RNA was isolated from serum samples and detection of microRNA (miRNA) expression analyzed with qPCR. In the discovery set, 179 human miRNAs were assayed for profiling of five OMG patients and four age- and gender-matched healthy controls. Read More

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http://dx.doi.org/10.1002/acn3.692DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6389736PMC
February 2019
5 Reads

Periventricular white matter changes in idiopathic intracranial hypertension.

Ann Clin Transl Neurol 2019 Feb 18;6(2):233-242. Epub 2019 Jan 18.

Department of Medical and Surgical Sciences Center for Headache and Intracranial Pressure Disorders Institute of Neurology Magna Græcia University of Catanzaro Catanzaro Italy.

Objective: To evaluate whether increased cerebrospinal fluid (CSF) pressure causes alteration of periventricular white matter (WM) microstructure in patients with idiopathic intracranial hypertension (IIH).

Methods: In a prospective study, patients with refractory chronic headache with and without IIH performed a neuroimaging study including 3T MRI, 3D Phase Contrast MR venography, and diffusion tensor imaging (DTI) of the brain. Whole-brain voxel-wise comparisons of DTI abnormalities of WM were performed using tract-based spatial statistics. Read More

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http://dx.doi.org/10.1002/acn3.685DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6389746PMC
February 2019
2 Reads

Retinal measurements predict 10-year disability in multiple sclerosis.

Ann Clin Transl Neurol 2019 Feb 19;6(2):222-232. Epub 2019 Jan 19.

Department of Neurology Johns Hopkins University Baltimore Maryland.

Objective: Optical coherence tomography (OCT)-derived measures of the retina correlate with disability and cortical gray matter atrophy in multiple sclerosis (MS); however, whether such measures predict long-term disability is unknown. We evaluated whether a single OCT and visual function assessment predict the disability status 10 years later.

Methods: Between 2006 and 2008, 172 people with MS underwent Stratus time domain-OCT imaging [160 with measurement of total macular volume (TMV)] and high and low-contrast letter acuity (LCLA) testing ( = 150; 87%). Read More

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http://doi.wiley.com/10.1002/acn3.674
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http://dx.doi.org/10.1002/acn3.674DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6389740PMC
February 2019
7 Reads

Fluctuation of primary motor cortex excitability during cataplexy in narcolepsy.

Ann Clin Transl Neurol 2019 Feb 20;6(2):210-221. Epub 2019 Jan 20.

Department of Neurology Changzheng Hospital The Second Military Medical University Shanghai China.

Objective: Cataplexy is a complicated and dynamic process in narcolepsy type 1 (NT1) patients. This study aimed to clarify the distinct stages during a cataplectic attack and identify the changes of the primary motor cortex (PMC) excitability during these stages.

Methods: Thirty-five patients with NT1 and 29 healthy controls were recruited to this study. Read More

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http://doi.wiley.com/10.1002/acn3.670
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http://dx.doi.org/10.1002/acn3.670DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6389735PMC
February 2019
5 Reads

Novel challenges in spinal muscular atrophy - How to screen and whom to treat?

Ann Clin Transl Neurol 2019 Jan 13;6(1):197-205. Epub 2018 Nov 13.

Division of Child Neurology and Metabolic Medicine Center for Child and Adolescent Medicine University Hospital Heidelberg Heidelberg Germany.

In recent years, disease-modifying and life-prolonging therapies for spinal muscular atrophy (SMA) have been developed. However, patients are currently diagnosed with significant delay and therapies are often administered in advanced stages of motor neuron degeneration, showing limited effects. Methods to identify children in presymptomatic stages are currently evaluated in newborn screening programs. Read More

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http://doi.wiley.com/10.1002/acn3.689
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http://dx.doi.org/10.1002/acn3.689DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6331314PMC
January 2019
14 Reads

Remote ischemic conditioning for stroke: clinical data, challenges, and future directions.

Ann Clin Transl Neurol 2019 Jan 15;6(1):186-196. Epub 2018 Nov 15.

Beijing Key Laboratory of Hypoxic Conditioning Translational Medicine Xuanwu Hospital Capital Medical University Beijing China.

Despite great improvement during the past several decades, the management of stroke is still far from satisfactory, which warrants alternative or adjunctive strategies. Remote ischemic conditioning (RIC), an easy-to-use and noninvasive therapy, can be performed in various clinical scenarios (e.g. Read More

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http://dx.doi.org/10.1002/acn3.691DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6331204PMC
January 2019
2 Reads

Deep brain stimulation: potential for neuroprotection.

Ann Clin Transl Neurol 2019 Jan 8;6(1):174-185. Epub 2018 Nov 8.

Krembil Research Institute University Health Network Toronto Western Hospital Toronto Ontario Canada.

Over the last two decades there has been an exponential rise in the number of patients receiving deep brain stimulation (DBS) to manage debilitating neurological symptoms in conditions such as Parkinson's disease, essential tremor, and dystonia. Novel applications of DBS continue to emerge including treatment of various psychiatric conditions (e.g. Read More

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http://dx.doi.org/10.1002/acn3.682DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6331208PMC
January 2019
4 Reads

Pilomotor seizures marked by infraslow activity and acetazolamide responsiveness.

Ann Clin Transl Neurol 2019 Jan 12;6(1):167-173. Epub 2018 Nov 12.

Krembil Brain Institute Division of Neurology University Health Network University of Toronto Toronto Canada.

A patient with pilomotor seizures post anti-LGI1 limbic encephalitis, refractory to immunotherapy and anti-epileptic drugs, was investigated with electroencephalography and magnetoencephalography. Seizures occurred daily (14.9 ± 4. Read More

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http://doi.wiley.com/10.1002/acn3.695
Publisher Site
http://dx.doi.org/10.1002/acn3.695DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6331207PMC
January 2019
22 Reads

OGTT is recommended for glucose homeostasis assessments in Friedreich ataxia.

Ann Clin Transl Neurol 2019 Jan 25;6(1):161-166. Epub 2018 Nov 25.

Division of Endocrinology Erasmus Hospital Université Libre de Bruxelles Brussels Belgium.

Diabetes is a common complication of Friedreich ataxia, requiring sensitive diagnostic methods. Here, we compared the performance of different tests that assess glucose tolerance, insulin sensitivity, and -cell function in Friedreich ataxia patients, heterozygous mutation carriers and controls. We find that diabetes is underdiagnosed with fasting glucose alone. Read More

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http://doi.wiley.com/10.1002/acn3.686
Publisher Site
http://dx.doi.org/10.1002/acn3.686DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6331656PMC
January 2019
8 Reads

Novel pathogenic variants causing dysarthria, ataxia, and sensory neuropathy.

Ann Clin Transl Neurol 2019 Jan 9;6(1):154-160. Epub 2018 Nov 9.

Board of Governors Regenerative Medicine Institute Cedars-Sinai Medical Center Los Angeles California.

encodes a mitochondrial complex IV assembly factor important for COX2 activation. Only one homozygous missense mutation has been previously described in two separate consanguineous families. We report four subjects with features that include childhood hypotonia, areflexia, ataxia, dysarthria, dystonia, and sensory neuropathy. Read More

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http://doi.wiley.com/10.1002/acn3.661
Publisher Site
http://dx.doi.org/10.1002/acn3.661DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6331954PMC
January 2019
8 Reads

Lowering systolic blood pressure does not increase stroke risk: an analysis of the SPRINT and ACCORD trial data.

Ann Clin Transl Neurol 2019 Jan 28;6(1):144-153. Epub 2018 Nov 28.

Department of Neurology University of Tennessee Health Science Center Memphis Tennessee.

Objective: Traditional neurology teaching states that when mean arterial pressure dips below a 60 mm Hg threshold, there is an increase in stroke risk due to cerebral hypoperfusion. The aim of this study was to determine whether intensive lowering of systolic blood pressure increases adverse cardiovascular outcomes by examining the association between achieved blood pressure values, specifically mean arterial pressure and pulse pressure, and risk of stroke.

Methods: Data from participants in the Systolic Blood Pressure Intervention Trial (SPRINT) and the Action to Control Cardiovascular Risk in Diabetes (ACCORD) Blood Pressure (BP) trial were examined, using survival analysis to model minimal arterial pressure and average pulse pressure during the study period against risk of stroke, hypotension, and syncope, with death as a competing risk. Read More

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http://dx.doi.org/10.1002/acn3.693DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6331200PMC
January 2019
4 Reads