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Annals of Clinical and Translational Neurology

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https://www.readbyqxmd.com/read/27844035/intravenous-thrombolysis-in-acute-ischemic-stroke-after-dabigatran-reversal-with-idarucizumab-a-case-report
#1
Johannes Sebastian Mutzenbach, Slaven Pikija, Ferdinand Otto, Ursula Halwachs, Friedrich Weymayr, Johann Sellner
Much excitement has been generated with the approval of idarucizumab, a humanized monoclonal antigen-binding antibody fragment that is capable of reversing the anticoagulant activity of dabigatran. Here, we describe our initial experience of using tissue plasminogen activator (tPA) in an acute posterior circulation ischemic stroke after dabigatran reversal with idarucizumab. Both treatments were well tolerated and no hemorrhagic or procoagulatory complications were observed. We propose that the option of dabigatran reversal needs to be considered for contemporary treatment concepts of acute ischemic stroke...
November 2016: Annals of Clinical and Translational Neurology
https://www.readbyqxmd.com/read/27844034/neural-coupling-of-cooperative-hand-movements-after-stroke-role-of-ipsilateral-afference
#2
Miriam Schrafl-Altermatt, Volker Dietz
We investigated the role of ipsilateral ascending pathways in the neural coupling underlying cooperative hand movements of stroke subjects. Ipsi- and contralateral somatosensory evoked potentials (SSEP) were recorded following ulnar nerve stimulation during cooperative and non-cooperative hand movements. The amplitude ratio, that is, ipsilateral divided by contralateral amplitude, was highest during the cooperative task when the affected arm was stimulated, reflecting an enhanced afferent volley to the unaffected hemisphere...
November 2016: Annals of Clinical and Translational Neurology
https://www.readbyqxmd.com/read/27844033/novel-nalcn-variant-altered-respiratory-and-circadian-rhythm-anesthetic-sensitivity
#3
Bernarda Lozic, Stefan Johansson, Sanja Lovric Kojundzic, Josko Markic, Per Morten Knappskog, Angelika F Hahn, Helge Boman
The sodium leak channel, a Na(+)-permeable, nonselective cation channel, is widely expressed in the nervous system, contributing a basal Na(+)-leak conductance and regulating neuronal excitability. A 3-year-old girl, heterozygous for a de novo missense mutation in NALCN (c.956C>T; p.Ala319Val) predicted to be deleterious, presented from birth with: stimulus-induced, episodic contractures of the limbs and face with associated respiratory distress; distal arthrogryposis; severe axial hypotonia; and severe global developmental delay (CLIFAHDD syndrome)...
November 2016: Annals of Clinical and Translational Neurology
https://www.readbyqxmd.com/read/27844032/predicting-disease-progression-in-amyotrophic-lateral-sclerosis
#4
Albert A Taylor, Christina Fournier, Meraida Polak, Liuxia Wang, Neta Zach, Mike Keymer, Jonathan D Glass, David L Ennist
OBJECTIVE: It is essential to develop predictive algorithms for Amyotrophic Lateral Sclerosis (ALS) disease progression to allow for efficient clinical trials and patient care. The best existing predictive models rely on several months of baseline data and have only been validated in clinical trial research datasets. We asked whether a model developed using clinical research patient data could be applied to the broader ALS population typically seen at a tertiary care ALS clinic. METHODS: Based on the PRO-ACT ALS database, we developed random forest (RF), pre-slope, and generalized linear (GLM) models to test whether accurate, unbiased models could be created using only baseline data...
November 2016: Annals of Clinical and Translational Neurology
https://www.readbyqxmd.com/read/27844031/clinical-and-biophysical-characterization-of-19-gjb1-mutations
#5
Pei-Chien Tsai, De-Ming Yang, Yi-Chu Liao, Tai-Yu Chiu, Hung-Chou Kuo, Yu-Ping Su, Yuh-Cherng Guo, Bing-Wen Soong, Kon-Ping Lin, Yo-Tsen Liu, Yi-Chung Lee
OBJECTIVE: Charcot-Marie-Tooth disease type X1 (CMTX1), which is caused by mutations in the gap junction (GJ) protein beta-1 gene (GJB1), is the second most common form of Charcot-Marie-Tooth disease (CMT). GJB1 encodes the GJ beta-1 protein (GJB1), which forms GJs within the myelin sheaths of peripheral nerves. The process by which GJB1 mutants cause neuropathy has not been fully elucidated. This study evaluated the biophysical characteristics of GJB1 mutants and their correlations with the clinical features of CMTX1 patients...
November 2016: Annals of Clinical and Translational Neurology
https://www.readbyqxmd.com/read/27844030/archetypal-notch3-mutations-frequent-in-public-exome-implications-for-cadasil
#6
Julie W Rutten, Hans G Dauwerse, Gido Gravesteijn, Martine J van Belzen, Jeroen van der Grond, James M Polke, Manuel Bernal-Quiros, Saskia A J Lesnik Oberstein
OBJECTIVE: To determine the frequency of distinctive EGFr cysteine altering NOTCH3 mutations in the 60,706 exomes of the exome aggregation consortium (ExAC) database. METHODS: ExAC was queried for mutations distinctive for cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL), namely mutations leading to a cysteine amino acid change in one of the 34 EGFr domains of NOTCH3. The genotype-phenotype correlation predicted by the ExAC data was tested in an independent cohort of Dutch CADASIL patients using quantified MRI lesions...
November 2016: Annals of Clinical and Translational Neurology
https://www.readbyqxmd.com/read/27844029/dose-dependent-inhibition-of-demyelination-and-microglia-activation-by-ivig
#7
Meike Winter, Christine Baksmeier, Julia Steckel, Sumanta Barman, Manish Malviya, Melanie Harrer-Kuster, Hans-Peter Hartung, Norbert Goebels
OBJECTIVE: Intravenous immunoglobulin (IVIG) is an established treatment for numerous autoimmune conditions. Clinical trials of IVIG for multiple sclerosis, using diverse dose regimens, yielded controversial results. The aim of this study is to dissect IVIG effector mechanisms on demyelination in an ex vivo model of the central nervous system (CNS)-immune interface. METHODS: Using organotypic cerebellar slice cultures (OSC) from transgenic mice expressing green fluorescent protein (GFP) in oligodendrocytes/myelin, we induced extensive immune-mediated demyelination and oligodendrocyte loss with an antibody specific for myelin oligodendrocyte glycoprotein (MOG) and complement...
November 2016: Annals of Clinical and Translational Neurology
https://www.readbyqxmd.com/read/27752517/respiratory-training-in-an-individual-with-amyotrophic-lateral-sclerosis
#8
Lauren C Tabor, Karen M Rosado, Raele Robison, Karen Hegland, Ianessa A Humbert, Emily K Plowman
We examined the impact of expiratory muscle strength training on maximum expiratory pressure, cough spirometry, and disease progression in a 71-year-old male with amyotrophic lateral sclerosis. Maximum expiratory pressure declined 9% over an 8-week sham training period, but subsequently improved by 102% following 8 weeks of expiratory muscle strength training. Improvements in cough spirometry and mitigated disease progression were also observed post expiratory muscle strength training. Improvements in maximum expiratory pressures were maintained 6 months following expiratory muscle strength training and were 79% higher than baseline data obtained 301 days prior...
October 2016: Annals of Clinical and Translational Neurology
https://www.readbyqxmd.com/read/27752516/alpha-synuclein-rt-quic-in-the-csf-of-patients-with-alpha-synucleinopathies
#9
Graham Fairfoul, Lynne I McGuire, Suvankar Pal, James W Ironside, Juliane Neumann, Sharon Christie, Catherine Joachim, Margaret Esiri, Samuel G Evetts, Michal Rolinski, Fahd Baig, Claudio Ruffmann, Richard Wade-Martins, Michele T M Hu, Laura Parkkinen, Alison J E Green
We have developed a novel real-time quaking-induced conversion RT-QuIC-based assay to detect alpha-synuclein aggregation in brain and cerebrospinal fluid from dementia with Lewy bodies and Parkinson's disease patients. This assay can detect alpha-synuclein aggregation in Dementia with Lewy bodies and Parkinson's disease cerebrospinal fluid with sensitivities of 92% and 95%, respectively, and with an overall specificity of 100% when compared to Alzheimer and control cerebrospinal fluid. Patients with neuropathologically confirmed tauopathies (progressive supranuclear palsy; corticobasal degeneration) gave negative results...
October 2016: Annals of Clinical and Translational Neurology
https://www.readbyqxmd.com/read/27752515/rapid-number-naming-in-chronic-concussion-eye-movements-in-the-king-devick-test
#10
John-Ross Rizzo, Todd E Hudson, Weiwei Dai, Joel Birkemeier, Rosa M Pasculli, Ivan Selesnick, Laura J Balcer, Steven L Galetta, Janet C Rucker
OBJECTIVE: The King-Devick (KD) test, which is based on rapid number naming speed, is a performance measure that adds vision and eye movement assessments to sideline concussion testing. We performed a laboratory-based study to characterize ocular motor behavior during the KD test in a patient cohort with chronic concussion to identify features associated with prolonged KD reading times. METHODS: Twenty-five patients with a concussion history (mean age: 31) were compared to control participants with no concussion history (n = 42, mean age: 32)...
October 2016: Annals of Clinical and Translational Neurology
https://www.readbyqxmd.com/read/27752514/white-matter-hyperintensities-incident-mild-cognitive-impairment-and-cognitive-decline-in-old-age
#11
Patricia A Boyle, Lei Yu, Debra A Fleischman, Sue Leurgans, Jingyun Yang, Robert S Wilson, Julie A Schneider, Zoe Arvanitakis, Konstantinos Arfanakis, David A Bennett
OBJECTIVE: Examine the association of white matter hyperintensities (WMH) with risk of incident mild cognitive impairment (MCI) and rate of decline in multiple cognitive systems in community-based older persons. METHODS: Participants (n = 354) were older persons initially free of cognitive impairment from two ongoing longitudinal epidemiologic studies of aging. All underwent brain magnetic resonance imaging (MRI) for quantification of WMH and gray matter volumes and detailed annual clinical evaluations including 17 cognitive tests...
October 2016: Annals of Clinical and Translational Neurology
https://www.readbyqxmd.com/read/27752513/longitudinal-profile-of-iron-accumulation-in-good-grade-subarachnoid-hemorrhage
#12
Christoph Scherfler, Alois Josef Schiefecker, Margarete Delazer, Ronny Beer, Thomas Bodner, Georg Spinka, Mario Kofler, Bettina Pfausler, Christian Kremser, Michael Schocke, Thomas Benke, Elke R Gizewski, Erich Schmutzhard, Raimund Helbok
OBJECTIVE: MRI parameters of iron concentration (R2*, transverse relaxation rate), microstructural integrity (mean diffusivity and fractional anisotropy), as well as gray and white matter volumes were analyzed in patients with subarachnoid hemorrhage (SAH) and uncomplicated clinical course to detect the evolution of brain tissue changes 3 weeks and 12 months after ictus. METHODS: MRI scans of 14 SAH patients (aneurysm of the anterior communicating artery, n = 5; no aneurysm n = 9) were compared with 14 age-matched healthy control subjects...
October 2016: Annals of Clinical and Translational Neurology
https://www.readbyqxmd.com/read/27752512/dysregulation-of-protein-phosphatase-2a-in-parkinson-disease-and-dementia-with-lewy-bodies
#13
Hye-Jin Park, Kang-Woo Lee, Eun S Park, Stephanie Oh, Run Yan, Jie Zhang, Thomas G Beach, Charles H Adler, Michael Voronkov, Steven P Braithwaite, Jeffry B Stock, M Maral Mouradian
OBJECTIVE: Protein phosphatase 2A (PP2A) is a heterotrimeric holoenzyme composed of a catalytic C subunit, a structural A subunit, and one of several regulatory B subunits that confer substrate specificity. The assembly and activity of PP2A are regulated by reversible methylation of the C subunit. α-Synuclein, which aggregates in Parkinson disease (PD) and dementia with Lewy bodies (DLB), is phosphorylated at Ser129, and PP2A containing a B55α subunit is a major phospho-Ser129 phosphatase...
October 2016: Annals of Clinical and Translational Neurology
https://www.readbyqxmd.com/read/27752511/%C3%AE-car-igf-1-vector-targeting-of-motor-neurons-ameliorates-disease-progression-in-als-mice
#14
Ioanna Eleftheriadou, Ioannis Manolaras, Elaine E Irvine, Michael Dieringer, Antonio Trabalza, Nicholas D Mazarakis
OBJECTIVE: We have previously described the generation of coxsackievirus and adenovirus receptor (α CAR)-targeted vector, and shown that intramuscular delivery in mouse leg muscles resulted in specific retrograde transduction of lumbar-motor neurons (MNs). Here, we utilized the α CAR-targeted vector to investigate the in vivo neuroprotective effects of lentivirally expressed IGF-1 for inducing neuronal survival and ameliorating the neuropathology and behavioral phenotypes of the SOD1(G93A) mouse model of ALS...
October 2016: Annals of Clinical and Translational Neurology
https://www.readbyqxmd.com/read/27752510/diagnostic-utility-of-asl-mri-and-fdg-pet-in-the-behavioral-variant-of-ftd-and-ad
#15
Duygu Tosun, Norbert Schuff, Gil D Rabinovici, Nagehan Ayakta, Bruce L Miller, William Jagust, Joel Kramer, Michael M Weiner, Howard J Rosen
OBJECTIVE: To compare the values of arterial spin-labeled (ASL) MRI and fluorodeoxyglucose (FDG) PET in the diagnosis of behavioral variant of frontotemporal dementia (bvFTD) and Alzheimer's disease (AD). METHODS: Partial least squares logistic regression was used to identify voxels with diagnostic value in cerebral blood flow (CBF) and cerebral metabolic rate of glucose (CMRgl) maps from patients with bvFTD (n = 32) and AD (n = 28), who were compared with each other and with cognitively normal controls (CN, n = 15)...
October 2016: Annals of Clinical and Translational Neurology
https://www.readbyqxmd.com/read/27656661/a-multiple-sclerosis-like-disorder-in-patients-with-opa1-mutations
#16
Patrick Yu-Wai-Man, Achillefs Spyropoulos, Holly J Duncan, Joseph V Guadagno, Patrick F Chinnery
We describe three unrelated patients presenting with a spinal cord syndrome and neuroimaging features consistent with multiple sclerosis (MS). All harbored a pathogenic OPA1 mutation. Although the neurological phenotype resembled neuromyelitis optica (NMO), anti-aquaporin 4 antibodies were not detected and the disorder followed a slow progressive course. The coincidental occurrence of OPA1 mutations and an MS-like disorder is likely to have modulated the phenotypic manifestations of both disorders, but unlike the previously reported association of Leber hereditary optic neuropathy and MS (Harding disease), the optic neuropathy in patients with OPA1 mutations and an MS-like disorder can be mild with a good visual prognosis...
September 2016: Annals of Clinical and Translational Neurology
https://www.readbyqxmd.com/read/27648461/terminal-complement-activation-is-increased-and-associated-with-disease-severity-in-cidp
#17
Isaak Quast, Christian W Keller, Falk Hiepe, Björn Tackenberg, Jan D Lünemann
Chronic inflammatory demyelinating polyneuropathy (CIDP) is the most common chronic autoimmune neuropathy. While both cell-mediated and humoral mechanisms contribute to its pathogenesis, the rapid clinical response to plasmapheresis implicates a circulating factor responsible for peripheral nerve injury. We report that treatment-naïve patients with CIDP show increased serum and CSF levels of the anaphylatoxin C5a and the soluble terminal complement complex (sTCC). Systemic terminal complement activation correlates with clinical disease severity as determined by the Inflammatory Neuropathy Cause and Treatment (INCAT) disability scale...
September 2016: Annals of Clinical and Translational Neurology
https://www.readbyqxmd.com/read/27648460/interplay-of-brain-structure-and-function-in-neonatal-congenital-heart-disease
#18
Ala Birca, Vasily A Vakorin, Prashob Porayette, Sujana Madathil, Vann Chau, Mike Seed, Sam M Doesburg, Susan Blaser, Dragos A Nita, Rohit Sharma, Emma G Duerden, Edward J Hickey, Steven P Miller, Cecil D Hahn
OBJECTIVE: To evaluate whether structural and microstructural brain abnormalities in neonates with congenital heart disease (CHD) correlate with neuronal network dysfunction measured by analysis of EEG connectivity. METHODS: We studied a prospective cohort of 20 neonates with CHD who underwent continuous EEG monitoring before surgery to assess functional brain maturation and network connectivity, structural magnetic resonance imaging (MRI) to determine the presence of brain injury and structural brain development, and diffusion tensor MRI to assess brain microstructural development...
September 2016: Annals of Clinical and Translational Neurology
https://www.readbyqxmd.com/read/27648459/mutation-of-orthologous-prickle-genes-causes-a-similar-epilepsy-syndrome-in-flies-and-humans
#19
Salleh N Ehaideb, Elizabeth A Wignall, Junko Kasuya, William H Evans, Atulya Iyengar, Haley L Koerselman, Anthony J Lilienthal, Alexander G Bassuk, Toshihiro Kitamoto, J Robert Manak
OBJECTIVE: Genetically tractable fruit flies have been used for decades to study seizure disorders. However, there is a paucity of data specifically correlating fly and human seizure phenotypes. We have previously shown that mutation of orthologous PRICKLE genes from flies to humans produce seizures. This study aimed to determine whether the prickle-mediated seizure phenotypes in flies closely parallel the epilepsy syndrome found in PRICKLE patients. METHODS: Virtually all fly seizure studies have relied upon characterizing seizures that are evoked...
September 2016: Annals of Clinical and Translational Neurology
https://www.readbyqxmd.com/read/27648458/progression-of-friedreich-ataxia-quantitative-characterization-over-5-years
#20
Maya Patel, Charles J Isaacs, Lauren Seyer, Karlla Brigatti, Sarah Gelbard, Cassandra Strawser, Debbie Foerster, Julianna Shinnick, Kimberly Schadt, Eppie M Yiu, Martin B Delatycki, Susan Perlman, George R Wilmot, Theresa Zesiewicz, Katherine Mathews, Christopher M Gomez, Grace Yoon, Sub H Subramony, Alicia Brocht, Jennifer Farmer, David R Lynch
OBJECTIVE: Friedreich ataxia (FRDA) is a progressive neurodegenerative disorder of adults and children. This study analyzed neurological outcomes and changes to identify predictors of progression and generate power calculations for clinical trials. METHODS: Eight hundred and twelve subjects in a natural history study were evaluated annually across 12 sites using the Friedreich Ataxia Rating Scale (FARS), 9-Hole Peg Test, Timed 25-Foot Walk, visual acuity tests, self-reported surveys and disability scales...
September 2016: Annals of Clinical and Translational Neurology
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