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David W Crumpacker
Pseudobulbar affect (PBA) is a socially debilitating condition that primarily affects people with neurologic diseases, such as Alzheimer's disease or multiple sclerosis. This condition is characterized by uncontrolled, exaggerated expressions of laughing or crying-often when the situation does not warrant this behavior. Although the true prevalence of PBA is surprisingly high, this condition remains widely misdiagnosed and underdiagnosed. While its exact etiology is unknown, PBA likely results from disruptions in the brain structures and/or neurotransmitters that regulate emotions...
September 2016: Journal of Clinical Psychiatry
Petra D Cravens, Rehana Z Hussain, William A Miller-Little, Li-Hong Ben, Benjamin M Segal, Emily Herndon, Olaf Stüve
BACKGROUND: Interleukin (IL)-12 and IL-23 are heterodimers that share the p40 subunit, and both cytokines are critical in the differentiation of T helper (Th)1 and Th17 cells, respectively. Th1 and Th17 effector cells have been implicated in the pathogenesis of experimental autoimmune encephalitis (EAE), an animal model of the human central nervous system (CNS) autoimmune demyelinating disorder multiple sclerosis (MS). However, ustekinumab, a monoclonal antibody (mAb) against p40 failed to show efficacy over placebo in a phase II clinical trial in patients with MS...
2016: PloS One
Wei Gao, Vincent Crosby, Andrew Wilcock, Rachael Burman, Eli Silber, Nilay Hepgul, K Ray Chaudhuri, Irene J Higginson
BACKGROUND: There is no standard palliative care outcome measure for people with progressive long term neurological conditions (LTNC). This study aims to determine the psychometric properties of a new 8-item palliative care outcome scale of symptom burden (IPOS Neuro-S8) in this population. DATA AND METHODS: Data were merged from a Phase II palliative care intervention study in multiple sclerosis (MS) and a longitudinal observational study in idiopathic Parkinson's disease (IPD), multiple system atrophy (MSA) and progressive supranuclear palsy (PSP)...
2016: PloS One
Kristin Politi, Serge Przedborski
A recent study reports that microglia and oligodendrocytes promote motor neuron degeneration by inducing inflammation and necroptosis in a manner dependent on receptor-interacting kinase 1 (RIPK1). These findings could be significant for our understanding of the neurobiology and treatment of neurodegenerative diseases like amyotrophic lateral sclerosis.
October 24, 2016: Current Biology: CB
Joseph J Ryan, David S Kreiner, Laura Glass Umfleet, Samuel T Gontkovsky, Allison Myers-Fabian
We examined relationships between the Wechsler Adult Intelligence Scale-Fourth Edition (WAIS-IV) General Ability Index (GAI) and Cognitive Proficiency Index (CPI) in two clinical samples. The mean pattern produced by 42 individuals with multiple sclerosis (MS) and 47 with traumatic brain injury (TBI) was the same, GAI > CPI. This pattern occurred in 61.9% and 78.7% of the protocols of patients with MS or TBI, respectively. The MS sample earned a significantly larger CPI mean than did patients with TBI...
October 25, 2016: Applied Neuropsychology. Adult
Jeffrey I Zwicker
Bloodborne microparticles are submicron vesicles released from cells within the vascular compartment. Pathologic alterations of microparticle populations have been explored in a large number of conditions ranging from multiple sclerosis to dengue fever.[1, 2] Despite wide ranging interest in the field of microparticles and their potential as biomarkers for disease states, their use in clinical practice is essentially nonexistent. A number of obstacles thus far have prohibited the translation of published observations into more generalizable clinical applications, not the least of which remains a lack of consensus regarding appropriate methodology for the measurement of microparticles...
October 25, 2016: Journal of Thrombosis and Haemostasis: JTH
Iliana Michailidou, Daphne M P Naessens, Simon Hametner, Willemijn Guldenaar, Evert-Jan Kooi, Jeroen J G Geurts, Frank Baas, Hans Lassmann, Valeria Ramaglia
Microglial clusters with C3d deposits are observed in the periplaque of multiple sclerosis (MS) brains and were proposed as early stage of lesion formation. As such they should appear in the brain of MS donors with acute disease but thus far this has not been shown. Using postmortem brain tissue from acute (n = 10) and chronic (n = 15) MS cases we investigated whether C3d+ microglial clusters are part of an acute attack against myelinated axons, which could have implications for disease pathogenesis...
October 25, 2016: Glia
Aikaterini Patsatsi, Miltiadis Kokolios, Olga Pikou, Vasilios Lambropoulos, Ioannis Efstratiou, Dimitrios Sotiriadis
Regression of congenital nevi is usually associated with loss of pigment or halo formation. In rare cases, regression is characterized by sclerosis and hair loss. We describe a rare case of a sclerotic hypopigmented large congenital melanocytic nevus in which a localized scleroderma-like reaction process of regression seemed to have started in utero and progressed throughout early childhood.
October 25, 2016: Pediatric Dermatology
A Gouveia, S P Dias, T Santos, H Rocha, C R Coelho, L Ruano, O Galego, M C Diogo, D Seixas, M J Sá, S Batista
OBJECTIVES: To characterize cognitive impairment in primary progressive multiple sclerosis (PPMS) and to correlate the pattern of cognitive deficits with brain magnetic resonance imaging (MRI) volumetric data. MATERIALS AND METHODS: In a multicenter cross-sectional study, we recruited consecutive patients with PPMS as well as age, sex, and education level-matched healthy controls (HC). All participants underwent neuropsychological (NP) assessment, and brain MRI was performed in patients with PPMS for analysis of lesion load, subcortical GM volumes, and regional cortical volumes...
October 24, 2016: Acta Neurologica Scandinavica
Quinten van Geest, B Westerik, Y D van der Werf, J J G Geurts, H E Hulst
Sleep disturbances are common in multiple sclerosis (MS), but its impact on cognition and functional connectivity (FC) of the hippocampus and thalamus is unknown. Therefore, we investigated the relationship between sleep disturbances, cognitive functioning and resting-state (RS) FC of the hippocampus and thalamus in MS. 71 MS patients and 40 healthy controls underwent neuropsychological testing and filled out self-report questionnaires (anxiety, depression, fatigue, and subjective cognitive problems). Sleep disturbances were assed with the five-item version of the Athens Insomnia Scale...
October 24, 2016: Journal of Neurology
Fary Khan, Bhasker Amatya, Mary P Galea, Roman Gonzenbach, Jürg Kesselring
The prevalence of disability due to neurological conditions is escalating worldwide. Neurological disorders have significant disability-burden with long-term functional and psychosocial issues, requiring specialized rehabilitation services for comprehensive management, especially treatments tapping into brain recovery 'neuroplastic' processes. Neurorehabilitation is interdisciplinary and cross-sectorial, requiring coordinated effort of diverse sectors, professions, patients and community to manage complex condition-related disability...
October 24, 2016: Journal of Neurology
Takashi Yamashita, Yoshihide Asano, Takashi Taniguchi, Kouki Nakamura, Ryosuke Saigusa, Shunsuke Miura, Tetsuo Toyama, Takehiro Takahashi, Yohei Ichimura, Ayumi Yoshizaki, Maria Trojanowska, Shinichi Sato
Systemic sclerosis (SSc) is a multisystem inflammatory and vascular disease resulting in extensive tissue fibrosis. Glycyrrhizin, clinically used for chronic hepatic diseases and itching dermatitis, modulates the pathological processes of inflammation, vasculopathy, and fibrosis in human diseases and their animal models. Therefore, we investigated a potential impact of glycyrrhizin on the key pathological manifestations of SSc, including inflammation, vasculopathy, and tissue fibrosis, with bleomycin (BLM)-treated mice mimicking the fibrotic and inflammatory components of SSc and endothelial cell-specific Fli1 knockout mice recapitulating SSc vasculopathy...
October 21, 2016: Journal of Investigative Dermatology
Nadera Methia, Samia Latreche, Omar Ait Mokhtar, Jean-Jacques Monsuez, Salim Benkhedda
BACKGROUND: Prognosis of systemic sclerosis (SSc) is affected by pulmonary artery hypertension (PAH). METHODS: Among 202 patients (mean age: 46.1 ± 13.3 years; 177 women) with SSc, those with a tricuspid regurgitation (TR) jet maximal velocity at 2D-echocardiography (2DE) < 2.8m/second were not considered at high risk for PAH, whereas those with a TR velocity >3m/second or between 2.8 and 3m/second and associated with dyspnea were. RESULTS: Among 22 patients at risk, 15 (mean age: 50...
October 2016: American Journal of the Medical Sciences
Shantanu Dixit, Chaithra Kalkur, Atul P Sattur, Michael M Bornstein, Fred Melton
BACKGROUND: Scleroderma is a chronic connective tissue disorder with unknown etiology. It is characterized by excessive deposition of extracellular matrix in the connective tissues causing vascular disturbances which can result in tissue hypoxia. These changes are manifested as atrophy of the skin and/or mucosa, subcutaneous tissue, muscles, and internal organs. Such changes can be classified into two types, namely, morphea (localized) and diffuse (systemic). Morphea can manifest itself as hemifacial atrophy (Parry-Romberg syndrome) although this remains debatable...
October 24, 2016: Journal of Medical Case Reports
Wenzao Li, Peng Zhou, Congmin Zhao, Yuping Zhang
Tuberous sclerosis complex (TSC), a multisystem genetic syndrome, often affects the central nervous system. The age of onset of TSC ranges from 0 to 15 years. The clinical features manifest as a combination of seizures, mental retardation, facial angiofibroma, renal angiomyolipoma, and cardiac rhabdomyoma. Most cases of TSC are caused by mutations of the TSC1 or TSC2 genes. We characterized a Chinese patient with a novel de novo mutation in the TSC2 gene associated with the TSC detected by next-generation sequencing...
October 25, 2016: Journal of Neurogenetics
Cole R Drifka, Agnes G Loeffler, Kara Mathewson, Adib Keikhosravi, Jens C Eickhoff, Yuming Liu, Sharon M Weber, W John Kao, Kevin W Eliceiri
Risk factors for pancreatic ductal adenocarcinoma (PDAC) progression after surgery are unclear, and additional prognostic factors are needed to inform treatment regimens and therapeutic targets. PDAC is characterized by advanced sclerosis of the extracellular matrix, and interactions between cancer cells, fibrillar collagen, and other stromal components play an integral role in progression. Changes in stromal collagen alignment have been shown to modulate cancer cell behavior and have important clinical value in other cancer types, but little is known about its role in PDAC and prognostic value...
October 20, 2016: Oncotarget
Eric Morel, Gilles Allali, Magali Laidet, Frédéric Assal, Patrice H Lalive, Stéphane Armand
BACKGROUND: Gait abnormalities are subtle in multiple sclerosis (MS) patients with low disability and need to be better determined. As a biomechanical approach, the Gait Profile Score (GPS) is used to assess gait quality by combining nine gait kinematic variables in one single value. This study aims i) to establish if the GPS can detect gait impairments and ii) to compare GPS with discrete spatiotemporal and kinematic parameters in low-disabled MS patients. METHOD: Thirty-four relapsing-remitting MS patients with an Expanded Disability Status Scale (EDSS) score ≤2 (mean age 36...
October 18, 2016: Gait & Posture
Foteini Christidi, Efstratios Karavasilis, Ioannis Zalonis, Panagiotis Ferentinos, Zoi Giavri, Elisabeth A Wilde, Sophia Xirou, Michalis Rentzos, Vasiliki Zouvelou, George Velonakis, Panagiotis Toulas, Efstathios Efstathopoulos, Loukia Poulou, Georgios Argyropoulos, Athanasios Athanasakos, Thomas Zambelis, Harvey S Levin, Nikolaos Karandreas, Nikolaos Kelekis, Ioannis Evdokimidis
We aimed to investigate structural changes in vivo in memory-related white matter tracts (i.e., perforant pathway zone [PPZ]; uncinate fasciculus [UF]; fornix) using diffusion tensor tractography and evaluate possible associations with memory performance in nondemented patients with amyotrophic lateral sclerosis (ALS). Forty-two ALS patients and 25 healthy controls (HCs) underwent a 30-directional diffusion-weighted imaging on a 3T MR scanner, followed by tractography of PPZ, UF, and fornix and analysis of fractional anisotropy (FA), axial diffusivity and radial diffusivity (Dr)...
September 28, 2016: Neurobiology of Aging
Brittany N Flores, Mark E Dulchavsky, Amy Krans, Michael R Sawaya, Henry L Paulson, Peter K Todd, Sami J Barmada, Magdalena I Ivanova
Hexanucleotide repeat expansions in C9orf72 are the most common inherited cause of amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD). The expansions elicit toxicity in part through repeat-associated non-AUG (RAN) translation of the intronic (GGGGCC)n sequence into dipeptide repeat-containing proteins (DPRs). Little is known, however, about the structural characteristics and aggregation propensities of the dipeptide units comprising DPRs. To address this question, we synthesized dipeptide units corresponding to the three sense-strand RAN translation products, analyzed their structures by circular dichroism, electron microscopy and dye binding assays, and assessed their relative toxicity when applied to primary cortical neurons...
2016: PloS One
Mohammadhossein Hajiebrahimi, Scott Montgomery, Sarah Burkill, Shahram Bahmanyar
OBJECTIVE: To investigate risk of premenopausal and postmenopausal breast cancer among Multiple Sclerosis (MS) patients, considering tumor stage. METHODS: The Swedish Patient Register identified 19,330 women with MS between 1968 and 2012, matched individually with a cohort of 193,458 without MS. Matching variables were year of birth, sex, region of residence and vital status at the time of diagnosis. The cancer register identified 471 and 5,753 breast cancer cases among the MS and non-MS cohorts, respectively...
2016: PloS One
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