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Annals of Clinical and Translational Neurology

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https://www.readbyqxmd.com/read/29296624/efficacy-and-safety-of-anti-amyloid-%C3%AE-immunotherapy-for-alzheimer-s-disease-a-systematic-review-and-network-meta-analysis
#1
REVIEW
Jia-Jie Mo, Jin-Yu Li, Zheng Yang, Zhou Liu, Jin-Shan Feng
To review the optimality and safety of different anti-Amyloid-β(Aβ) immunotherapies for Alzheimer's disease (AD). Published randomized controlled trials were comprehensively reviewed from electronic databases (Cochrane library, Embase, Pubmed, and Google scholar). Pooled outcomes as mean difference or odds ratio values with 95% confidence interval were reported. The network estimates with confidence and predictive intervals for all pairwise relative effects was evaluated. Optimal intervention was ranked by benefit-risk ratio based on the surface under the cumulative ranking curve...
December 2017: Annals of Clinical and Translational Neurology
https://www.readbyqxmd.com/read/29296623/clarion-call-for-histopathological-clot-analysis-in-cryptogenic-ischemic-stroke-implications-for-diagnosis-and-treatment
#2
Sonu Bhaskar, Dennis Cordato, Cecilia Cappelen-Smith, Andrew Cheung, David Ledingham, David Celermajer, Christopher Levi
Diagnosis, treatment, and secondary management of cryptogenic stroke patients pose a formidable challenge. The scenario is further complicated in patients with native and prosthetic valvular heart disease. We present a case study of a 36-year-old man who received intravenous thrombolysis (IV-tPA) and endovascular thrombectomy (EVT) for presumed "cryptogenic" complete middle cerebral artery infarction who made a surprisingly excellent clinical recovery despite poor baseline and postintervention neuroimaging...
December 2017: Annals of Clinical and Translational Neurology
https://www.readbyqxmd.com/read/29296622/relationships-between-grip-strength-myotonia-and-ctg-expansion-in-myotonic-dystrophy-type-1
#3
Jean-Yves Hogrel, Gwenn Ollivier, Isabelle Ledoux, Luc J Hébert, Bruno Eymard, Jack Puymirat, Guillaume Bassez
In myotonic dystrophy type 1, several studies have suggested causal relationships between CTG repeat length and the severity of symptoms, such as weakness or myotonia. We aimed to explore these relationships in a large population of 144 DM1 patients. All patients underwent clinical and functional assessments using a standardized test for grip strength and myotonia assessment. Myotonia was assessed using a fully automatic software based on mathematical modeling of relaxation force curve. CTG repeat length was statistically correlated with both myotonia and grip strength, which are two major primary neuromuscular symptoms of DM1 patients...
December 2017: Annals of Clinical and Translational Neurology
https://www.readbyqxmd.com/read/29296621/rivastigmine-decreases-brain-damage-in-hiv-patients-with-mild-cognitive-deficits
#4
Gaetano Perrotta, Guillaume Bonnier, Djalel-Eddine Meskaldji, David Romascano, Ruslan Aydarkhanov, Alessandro Daducci, Samanta Simioni, Matthias Cavassini, Melanie Metral, François Lazeyras, Reto Meuli, Gunnar Krueger, Renaud A Du Pasquier, Cristina Granziera
Rivastigmine has been shown to improve cognition in HIV+ patients with minor neurocognitive disorders; however, the mechanisms underlying such beneficial effect are currently unknown. To assess whether rivastigmine therapy is associated with decreased brain inflammation and damage, we performed T1/T2* relaxometry and magnetization transfer imaging in 17 aviremic HIV+ patients with minor neurocognitive disorders enrolled on a crossed over randomized rivastigmine trial. Rivastigmine therapy was associated with changes in MRI metrics indicating a decrease in brain water content (i...
December 2017: Annals of Clinical and Translational Neurology
https://www.readbyqxmd.com/read/29296620/infectious-risk-stratification-in-multiple-sclerosis-patients-receiving-immunotherapy
#5
Jonas Graf, Verena I Leussink, Thomas Dehmel, Marius Ringelstein, Norbert Goebels, Ortwin Adams, Colin R MacKenzie, Clemens Warnke, Torsten Feldt, Anna Lammerskitten, Luisa Klotz, Sven Meuth, Heinz Wiendl, Hans-Peter Hartung, Orhan Aktas, Philipp Albrecht
The increasing number of potent treatments for multiple sclerosis warrants screening for infections. To investigate the prevalence of infections in two independent German patient cohorts with multiple sclerosis/neuromyelitis optica spectrum disorders (NMOSD), we performed a retrospective chart review study of multiple sclerosis/NMOSD patients who underwent testing for infections between 2014 and 2016. We show that 6 out of 80 tested patients (Düsseldorf cohort) and 2 out of 97 tested patients (Münster cohort) had a latent tuberculosis infection; total 3...
December 2017: Annals of Clinical and Translational Neurology
https://www.readbyqxmd.com/read/29296619/4-hydroxybenzoic-acid-restores-coq10-biosynthesis-in-human-coq2-deficiency
#6
Diran Herebian, Annette Seibt, Sander H J Smits, Richard J Rodenburg, Ertan Mayatepek, Felix Distelmaier
The clinical phenotypes of human CoQ10-deficiency caused by COQ2 mutations range from fatal neonatal disease to adult-onset multisystem atrophy. So far, treatment options for these diseases are unsatisfactory. Here, we demonstrate that supplementation of 4-hydroxybenzoic acid (4-HBA) fully restores endogenous CoQ10-biosynthesis in COQ2-deficient cell lines. This was accompanied by increased protein expression of CoQ10-biosynthesis-enzymes as well as a rescue of cell viability during stress conditions. In silico analysis suggested a ligand transportation path for 4-HBA through the COQ2 protein towards the mitochondrial matrix side...
December 2017: Annals of Clinical and Translational Neurology
https://www.readbyqxmd.com/read/29296618/lateral-hypothalamic-activity-indicates-hunger-and-satiety-states-in-humans
#7
Omid Talakoub, Raquel R Paiva, Matija Milosevic, Marcelo Q Hoexter, Ruth Franco, Eduardo Alho, Jessie Navarro, José F Pereira, Milos R Popovic, Cary Savage, Antonio C Lopes, Pedro Alvarenga, Durval Damiani, Manoel J Teixeira, Euripides C Miguel, Erich T Fonoff, Marcelo C Batistuzzo, Clement Hamani
Lateral hypothalamic area (LHA) local field potentials (LFPs) were recorded in a Prader-Willi patient undergoing deep brain stimulation (DBS) for obesity. During hunger, exposure to food-related cues induced an increase in beta/low-gamma activity. In contrast, recordings during satiety were marked by prominent alpha rhythms. Based on these findings, we have delivered alpha-frequency DBS prior to and during food intake. Despite reporting an early sensation of fullness, the patient continued to crave food. This suggests that the pattern of activity in LHA may indicate hunger/satiety states in humans but attest to the complexity of conducting neuromodulation studies in obesity...
December 2017: Annals of Clinical and Translational Neurology
https://www.readbyqxmd.com/read/29296617/flupirtine-and-diazepam-combination-terminates-established-status-epilepticus-results-in-three-rodent-models
#8
Terry Zhang, Marko S Todorovic, John Williamson, Jaideep Kapur
Objective: Status epilepticus (SE) is a neurological emergency requiring rapid termination of seizures. New treatment choices are needed for benzodiazepine-refractory SE or established SE (ESE). Previous studies have demonstrated that the potassium-channel opener flupirtine terminates seizures in neonatal animals. However, its effectiveness in adult ESE has not been tested. We tested whether flupirtine alone or in combination with the benzodiazepine diazepam would terminate ESE in three animal models...
December 2017: Annals of Clinical and Translational Neurology
https://www.readbyqxmd.com/read/29296616/everolimus-for-treatment-of-tuberous-sclerosis-complex-associated-neuropsychiatric-disorders
#9
Darcy A Krueger, Anjali Sadhwani, Anna W Byars, Petrus J de Vries, David N Franz, Vicky H Whittemore, Rajna Filip-Dhima, Donna Murray, Kush Kapur, Mustafa Sahin
Objective: To evaluate if short-term treatment with everolimus was safe and could improve neurocognition and behavior in children with TSC. Methods: This was a prospective, double-blind randomized, placebo-controlled two-center phase II study. Participants diagnosed with TSC and age 6-21 years were treated with 4.5 mg/m2 per day of oral everolimus (n = 32) or matching placebo (n = 15) taken once daily for 6 months. For efficacy, a comprehensive neurocognitive and behavioral evaluation battery was performed at baseline, 3 months, and 6 months...
December 2017: Annals of Clinical and Translational Neurology
https://www.readbyqxmd.com/read/29296615/minocycline-protects-against-delayed-cerebral-ischemia-after-subarachnoid-hemorrhage-via-matrix-metalloproteinase-9-inhibition
#10
Ananth K Vellimana, Meng-Liang Zhou, Itender Singh, Diane J Aum, James W Nelson, Glenn R Harris, Umeshkumar Athiraman, Byung H Han, Gregory J Zipfel
Objective: Delayed cerebral ischemia (DCI) is an independent risk factor for poor outcome after aneurysmal subarachnoid hemorrhage (SAH) and is multifactorial in etiology. While prior studies have suggested a role for matrix metalloproteinase-9 (MMP-9) in early brain injury after SAH, its contribution to the pathophysiology of DCI is unclear. Methods: In the first experiment, wild-type (WT) and MMP-9-/- mice were subjected to sham or endovascular perforation SAH surgery...
December 2017: Annals of Clinical and Translational Neurology
https://www.readbyqxmd.com/read/29296614/neuropathology-of-childhood-onset-basal-ganglia-degeneration-caused-by-mutation-of-vac14
#11
Chloe Stutterd, Peter Diakumis, Melanie Bahlo, Miriam Fanjul Fernandez, Richard J Leventer, Martin Delatycki, David Amor, Chung W Chow, Sarah Stephenson, Miriam H Meisler, Catriona Mclean, Paul J Lockhart
Objective: To characterize the clinical features and neuropathology associated with recessive VAC14 mutations. Methods: Whole-exome sequencing was used to identify the genetic etiology of a rapidly progressive neurological disease presenting in early childhood in two deceased siblings with distinct neuropathological features on post mortem examination. Results: We identified compound heterozygous variants in VAC14 in two deceased siblings with early childhood onset of severe, progressive dystonia, and neurodegeneration...
December 2017: Annals of Clinical and Translational Neurology
https://www.readbyqxmd.com/read/29296613/mglur2-3-activation-of-the-sirt1-axis-preserves-mitochondrial-function-in-diabetic-neuropathy
#12
Krish Chandrasekaran, Anjaneyulu Muragundla, Tyler G Demarest, Joungil Choi, Avinash R Sagi, Neda Najimi, Pranith Kumar, Anmol Singh, Cheng-Ying Ho, Gary Fiskum, Lauren G Koch, Steven L Britton, James W Russell
Objectives: There is a critical need to develop effective treatments for diabetic neuropathy. This study determined if a selective mGluR2/3 receptor agonist prevented or treated experimental diabetic peripheral neuropathy (DPN) through glutamate recycling and improved mitochondrial function. Methods: Adult male streptozotocin treated Sprague-Dawley rats with features of type 1 diabetes mellitus (T1DM) or Low Capacity Running (LCR) rats with insulin resistance or glucose intolerance were treated with 3 or 10 mg/kg/day LY379268...
December 2017: Annals of Clinical and Translational Neurology
https://www.readbyqxmd.com/read/29159197/donepezil-enhances-understanding-of-degraded-speech-in-alzheimer-s-disease
#13
Chris J D Hardy, Yun T Hwang, Rebecca L Bond, Charles R Marshall, Basil H Ridha, Sebastian J Crutch, Martin N Rossor, Jason D Warren
Auditory dysfunction under complex, dynamic listening conditions is a clinical hallmark of Alzheimer's disease (AD) but challenging to measure and manage. Here, we assessed understanding of sinewave speech (a paradigm of degraded speech perception) and general cognitive abilities in 17 AD patients, before and following a 10 mg dose of donepezil. Relative to healthy older individuals, patients had impaired sinewave speech comprehension that was selectively ameliorated by donepezil. Our findings demonstrate impaired perception of degraded speech in AD but retained perceptual learning capacity that can be harnessed by acetylcholinesterase inhibition, with implications for designing communication interventions and acoustic environments in dementia...
November 2017: Annals of Clinical and Translational Neurology
https://www.readbyqxmd.com/read/29159196/botulinum-toxin-treatment-for-hypersalivation-in-anti-nmda-receptor-encephalitis
#14
Jin-Sun Jun, Han Gil Seo, Soon-Tae Lee, Kon Chu, Sang Kun Lee
Hypersalivation is one of the intractable symptoms of anti-N-methyl-d-aspartate receptor (NMDAR) encephalitis. While anticholinergic medications partially improve the hypersalivation, they can aggravate the autonomic dysfunctions associated with anti-NMDAR encephalitis. Thus, we investigated the efficacy and safety of botulinum toxin type A injection on hypersalivation refractory to anticholinergics in six patients with anti-NMDAR encephalitis. Hypersalivation was well-controlled without remarkable adverse reaction over 16 weeks after botulinum toxin type A, although two patients were reinjected at 12 weeks due to reaggravation of hypersalivation...
November 2017: Annals of Clinical and Translational Neurology
https://www.readbyqxmd.com/read/29159195/cd4-t-cells-from-multiple-sclerosis-patients-respond-to-a-commensal-derived-antigen
#15
Joseph N Burgess, Anudeep B Pant, Lloyd H Kasper, Sara Colpitts Brass
Multiple sclerosis, an immune-mediated disease of the central nervous system, is characterized by the impaired function of regulatory cells that fail to suppress self-reactive effector cells. We have previously found that polysaccharide A, a capsular antigen derived from the human gut commensal Bacteroides fragilis, can induce a population of regulatory T cells. Herein, we demonstrate that naïve T cells isolated from patients with multiple sclerosis have the capacity to acquire regulatory characteristics when stimulated in vitro with polysaccharide A...
November 2017: Annals of Clinical and Translational Neurology
https://www.readbyqxmd.com/read/29159194/multigeneration-family-with-dominant-spg30-hereditary-spastic-paraplegia
#16
Ricardo H Roda, Alice B Schindler, Craig Blackstone
Autosomal recessive KIF1A missense mutations cause hereditary spastic paraplegia (HSP) type SPG30, while recessive truncations lead to sensory and autonomic neuropathy (HSN2C) and many de novo missense mutations are associated with cognitive impairment. Here, we describe family members across three generations with pure HSP. A heterozygous p.Ser69Leu KIF1A mutation segregates with those afflicted. The same variant was previously reported in a Finnish father and son with pure HSP as well as four members of a Sicilian kindred with more intrafamilial phenotypic variability...
November 2017: Annals of Clinical and Translational Neurology
https://www.readbyqxmd.com/read/29159193/prep2-a-biomarker-based-algorithm-for-predicting-upper-limb-function-after-stroke
#17
Cathy M Stinear, Winston D Byblow, Suzanne J Ackerley, Marie-Claire Smith, Victor M Borges, P Alan Barber
Objective: Recovery of motor function is important for regaining independence after stroke, but difficult to predict for individual patients. Our aim was to develop an efficient, accurate, and accessible algorithm for use in clinical settings. Clinical, neurophysiological, and neuroimaging biomarkers of corticospinal integrity obtained within days of stroke were combined to predict likely upper limb motor outcomes 3 months after stroke. Methods: Data from 207 patients recruited within 3 days of stroke [103 females (50%), median age 72 (range 18-98) years] were included in a Classification and Regression Tree analysis to predict upper limb function 3 months poststroke...
November 2017: Annals of Clinical and Translational Neurology
https://www.readbyqxmd.com/read/29159192/sex-differences-in-lrrk2-g2019s-and-idiopathic-parkinson-s-disease
#18
Marta San Luciano, Cuiling Wang, Roberto A Ortega, Nir Giladi, Karen Marder, Susan Bressman, Rachel Saunders-Pullman
Objective: To evaluate sex differences and the relative effect of G2019S LRRK2 mutations in Parkinson's disease (PD). Methods: 530 LRRK2 PD carriers and 759 noncarrier PD (idiopathic, IPD) evaluated as part of the Fox Foundation (MJFF) Consortium were included. All participants completed a study visit including information on clinical features, treatment, examination, and motor and nonmotor questionnaires. Clinical features were compared between men and women separately for IPD and LRRK2 PD; and features were compared between IPD and LRRK2 PD separately for men and women...
November 2017: Annals of Clinical and Translational Neurology
https://www.readbyqxmd.com/read/29159191/soluble-cd163-in-intracerebral-hemorrhage-biomarker-for-perihematomal-edema
#19
Meaghan Roy-O'Reilly, Liang Zhu, Louise Atadja, Glenda Torres, Jaroslaw Aronowski, Louise McCullough, Nancy J Edwards
Objective: Patients with intracerebral hemorrhage (ICH) may elaborate varying degrees of perihematomal edema (PHE), requiring closer monitoring and a higher intensity of treatment. Here, we explore whether the soluble form of CD163, a scavenger receptor responsible for hemoglobin sequestration, can serve as a prognostic biomarker of PHE development and poor outcome after ICH. Methods: Our study cohort was comprised of 51 primary age- and sex-matched ICH patients with moderate-sized, hypertensive deep hemorrhages...
November 2017: Annals of Clinical and Translational Neurology
https://www.readbyqxmd.com/read/29159190/divergent-neural-responses-to-narrative-speech-in-disorders-of-consciousness
#20
Ivan Iotzov, Brian C Fidali, Agustin Petroni, Mary M Conte, Nicholas D Schiff, Lucas C Parra
Objective: Clinical assessment of auditory attention in patients with disorders of consciousness is often limited by motor impairment. Here, we employ intersubject correlations among electroencephalography responses to naturalistic speech in order to assay auditory attention among patients and healthy controls. Methods: Electroencephalographic data were recorded from 20 subjects with disorders of consciousness and 14 healthy controls during of two narrative audio stimuli, presented both forwards and time-reversed...
November 2017: Annals of Clinical and Translational Neurology
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