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https://www.readbyqxmd.com/read/28238739/consensus-based-recommendations-for-the-management-of-rapid-cognitive-decline-due-to-alzheimer-s-disease
#1
Jianping Jia, Serge Gauthier, Sarah Pallotta, Yong Ji, Wenshi Wei, Shifu Xiao, Dantao Peng, Qihao Guo, Liyong Wu, Shengdi Chen, Weihong Kuang, Junjian Zhang, Cuibai Wei, Yi Tang
INTRODUCTION: Rapid cognitive decline (RCD) occurs in dementia due to Alzheimer's disease (AD). METHODS: Literature review, consensus meetings, and a retrospective chart review of patients with probable AD were conducted. RESULTS: Literature review showed that RCD definitions varied. Mini-Mental State Examination scores <20 at treatment onset, vascular risk factors, age <70 years at symptom onset, higher education levels, and early appearance of hallucinations, psychosis, or extrapyramidal symptoms are recognized RCD risk factors...
February 23, 2017: Alzheimer's & Dementia: the Journal of the Alzheimer's Association
https://www.readbyqxmd.com/read/28228797/femoral-neck-s-fracture-in-fahr-s-syndrome-case-report
#2
Marcello Sallì, Antonio D'Arienzo, Mariella Bonanno, Salvatore Morello, Antonino Sanfilippo, Giulia Letizia Mauro, Michele D'Arienzo
Fahr's syndrome, also known as "Bilateral Striopallidodentate Calcinosis" (BSPDC) primitive, is a rare neurological disease characterized by the presence of idiopathic, bilateral, symmetrical and abnormal deposition of calcium in areas of the brain that control movements including the basal ganglia, dentate nuclei of the cerebellum, nuclei of thalamus and semi-oval center. We describe a case of a 76-year-old male patient underwent reduction and fixation of a subtrochanteric fracture with intramedullary nail...
September 2016: Clinical Cases in Mineral and Bone Metabolism
https://www.readbyqxmd.com/read/28219485/acute-antipsychotic-treatment-of-children-and-adolescents-with-schizophrenia-spectrum-disorders-a-systematic-review-and-network-meta-analysis
#3
REVIEW
Anne Katrine Pagsberg, Simon Tarp, Dorte Glintborg, Anne Dorte Stenstrøm, Anders Fink-Jensen, Christoph Ulrich Correll, Robin Christensen
OBJECTIVE: To determine the comparative efficacy and safety of antipsychotics for youth with early-onset schizophrenia using network meta-analytic methods combining direct and indirect trial data. METHOD: The authors systematically searched MEDLINE, the Cochrane Library, and clinicaltrials.gov and selected randomized controlled trials allocating youth with schizophrenia spectrum disorders to a (non-clozapine) antipsychotic versus placebo or another antipsychotic...
March 2017: Journal of the American Academy of Child and Adolescent Psychiatry
https://www.readbyqxmd.com/read/28217166/multifocal-myoclonus-as-a-heralding-manifestation-of-wilson-disease
#4
Rajesh Verma, Vikram V Holla, Suchit Pandey, Imran Rizvi
Wilson disease (WD) is one of the few curable movement disorders that manifests with varied presentations so that WD needs to be considered in any patient with a movement disorder under the age of 50 years. Although WD is one of the causes of myoclonus, it is rarely seen in WD and usually as an associated finding. We report a case of an adolescent female patient of WD who presented with cortical multifocal myoclonus of 6-month duration with later development of generalized dystonia, extrapyramidal syndrome, and cognitive decline...
October 2016: Journal of Pediatric Neurosciences
https://www.readbyqxmd.com/read/28205498/cognitive-control-learning-and-clinical-motor-ratings-are-most-highly-associated-with-basal-ganglia-brain-volumes-in-the-premanifest-huntington-s-disease-phenotype
#5
Maria B Misiura, Spencer Lourens, Vince D Calhoun, Jeffrey Long, Jeremy Bockholt, Hans Johnson, Ying Zhang, Jane S Paulsen, Jessica A Turner, Jingyu Liu, Betul Kara, Elizabeth Fall
OBJECTIVES: Huntington's disease (HD) is a debilitating genetic disorder characterized by motor, cognitive and psychiatric abnormalities associated with neuropathological decline. HD pathology is the result of an extended chain of CAG (cytosine, adenine, guanine) trinucleotide repetitions in the HTT gene. Clinical diagnosis of HD requires the presence of an otherwise unexplained extrapyramidal movement disorder in a participant at risk for HD. Over the past 15 years, evidence has shown that cognitive, psychiatric, and subtle motor dysfunction is evident decades before traditional motor diagnosis...
February 2017: Journal of the International Neuropsychological Society: JINS
https://www.readbyqxmd.com/read/28202694/pure-autonomic-failure-predictors-of-conversion-to-clinical-cns-involvement
#6
Wolfgang Singer, Sarah E Berini, Paola Sandroni, Robert D Fealey, Elizabeth A Coon, Mariana D Suarez, Eduardo E Benarroch, Phillip A Low
OBJECTIVE: Based on the observation that a subset of patients originally diagnosed with pure autonomic failure (PAF) eventually develops extrapyramidal or cerebellar involvement consistent with multiple system atrophy (MSA), Parkinson disease (PD), or dementia with Lewy bodies (DLB), we aimed to identify predictors of progression of PAF to more sinister synucleinopathies. METHODS: In this retrospective cohort study, we reviewed patients seen at Mayo Clinic Rochester by autonomic specialists between 2001 and 2011 and during initial evaluation diagnosed with orthostatic hypotension consistent with PAF (possible PAF)...
February 15, 2017: Neurology
https://www.readbyqxmd.com/read/28199071/effect-of-antipsychotic-type-and-dose-changes-on-tardive-dyskinesia-and-parkinsonism-severity-in-patients-with-a-serious-mental-illness-the-cura%C3%A3-ao-extrapyramidal-syndromes-study-xii
#7
Charlotte L Mentzel, P Roberto Bakker, Jim van Os, Marjan Drukker, Glenn E Matroos, Hans W Hoek, Marina A J Tijssen, Peter N van Harten
OBJECTIVE: To test the efficacy of current treatment recommendations for parkinsonism and tardive dyskinesia (TD) severity in patients with severe mental illness (SMI). METHODS: We present an 18-year prospective study including all 223 patients with SMI (as defined by the 1987 US National Institute of Mental Health, which were based on DSM-III-R diagnostic criteria) receiving care from the only psychiatric hospital of the former Netherlands Antilles. Eight clinical assessments (1992-2009) focused on movement disorders and medication use...
February 14, 2017: Journal of Clinical Psychiatry
https://www.readbyqxmd.com/read/28196698/-when-and-how-prescribe-antipsychotics
#8
S Tebeka, G Airagnes, F Limosin
Antipsychotics are commonly prescribed in the general population since they have many indications. They can be used in acute care such as agitation or behavior disorders, or to treat more characterized psychiatric disorders like psychotic or mood disorders. Consequently, any practitioner will have to prescribe or renew a prescription of antipsychotics. These treatments require a benefit/risk balance assessment taking into account the specific context of each patient. Indeed, antipsychotics have many side effects, mainly neurological (extrapyramidal syndrome, dyskinesia, akathisia), metabolic and cardiac...
February 10, 2017: La Revue de Médecine Interne
https://www.readbyqxmd.com/read/28193543/reduction-of-manganese-intake-improves-neuropsychological-manifestations-in-rats-with-minimal-hepatic-encephalopathy
#9
Ying Li, Lihong Mei, Jinwei Qiang, Changxue Ji, Shuai Ju
Brain manganese deposition is led by liver dysfunction and/or portal-systemic shunting in minimal hepatic encephalopathy (MHE). Manganese is toxic and can cause cognitive disorders and extrapyramidal symptoms. Thus, reduction of manganese intake might be considered as a potential treatment strategy for MHE. In this study we aimed to investigate whether low- or no-manganese feed can improve the neuropsychological manifestations in MHE rats. Rats with MHE were established by partially ligating the portal vein and fed a manganese diet (MHE-M, 10mg per kg feed; n=24), a no-manganese diet (MHE-N; n=24) and a half-manganese diet (MHE-H; n=24) for 2, 4, 6 and 8weeks, with six rats in each subgroup...
February 11, 2017: Neuroscience
https://www.readbyqxmd.com/read/28193195/assessing-the-burden-of-treatment-emergent-adverse-events-associated-with-atypical-antipsychotic-medications
#10
Pierre-Michel Llorca, Christophe Lançon, Ann Hartry, T Michelle Brown, Dana B DiBenedetti, Siddhesh A Kamat, Clément François
BACKGROUND: Treatment of schizophrenia and major depressive disorder (MDD) with atypical antipsychotics (AAPs) show improved efficacy and reduced side effect burden compared with older antipsychotic medications. However, a risk of treatment-emergent adverse events (TEAEs) remains. TEAEs are hard to quantify and perspectives on the importance of TEAEs differ across patients and between patients and physicians. The current study is a qualitative assessment that investigates TEAEs of AAPs from both patient and physician perspectives to provide better understanding of the occurrence and burden of TEAEs associated with these medications...
February 13, 2017: BMC Psychiatry
https://www.readbyqxmd.com/read/28183359/unexpected-extrapyramidal-symptoms-and-pulmonary-aspergillosis-in-exertional-heatstroke-with-fulminant-liver-failure-a-case-report
#11
Jie Jiao, Feihu Zhou, Hongjun Kang, Chao Liu, Mengmeng Yang, Jie Hu
BACKGROUND: Exertional heatstroke is a life-threatening condition with high mortality because of the rapid progress of multiple organ dysfunction syndrome even if aggressive treatments are initiated rapidly. Mild to moderate hepatic injury is common in exertional heatstroke, while fulminant liver failure is rare. Extrapyramidal symptoms and pulmonary aspergillosis secondary to liver failure induced by exertional heatstroke have never been reported in prior cases. CASE PRESENTATION: A 25-year-old Han Chinese man presented with exertional heatstroke with fulminant liver failure, subsequent pulmonary aspergillosis, and extrapyramidal symptoms...
February 10, 2017: Journal of Medical Case Reports
https://www.readbyqxmd.com/read/28181956/extrapyramidal-symptoms-in-schizophrenia-evidence-of-blunted-cerebral-hemodynamics-during-a-planning-task
#12
Daniel Schuepbach, Matthias Michel, Gert Wagner, Stefan Duschek, Sabine C Herpertz
Acute extrapyramidal symptoms (EPS) occur frequently in schizophrenia, mostly caused by antidopaminergic substances. There have been no published reports on the impact of acute EPS on cerebral blood flow (CBF) or related measures. In the following study, we examined schizophrenic patients with and without EPS during a planning task and measurements of CBF velocity. Sixteen patients with chronic schizophrenia and 16 healthy participants performed a planning paradigm during bilateral functional transcranial Doppler sonography of the middle cerebral arteries...
February 8, 2017: International Clinical Psychopharmacology
https://www.readbyqxmd.com/read/28168129/a-quandary-of-cuprum-wilson-s-disease-disguising-as-progressive-myoclonic-epilepsy
#13
Monika Sachan, Suman Kushwaha, Shah Faisal Ahmad Tarfarosh, Vineet Banga, Ashutosh Gupta
Although metals are indispensable for the production of articles in our daily usage, the deposition of these metals in human tissue is known to cause disease. However, it is not always the ingestion of abnormal amounts of lead, iron, or copper that makes our tissues morbid; our hereditary and metabolic issues are to be blamed as well. Wilson's disease is one such hereditary disease that creates chaos in tissues, usually the brain and liver, via deposition of abnormal amounts of copper in them. While Wilson's disease almost seems to bring a picture of a young patient with dystonia and other extrapyramidal symptoms in our imagination, seizures are very uncommon in this disorder...
January 1, 2017: Curēus
https://www.readbyqxmd.com/read/28146110/polyphenolic-extract-of-euphorbia-supina-attenuates-manganese-induced-neurotoxicity-by-enhancing-antioxidant-activity-through-regulation-of-er-stress-and-er-stress-mediated-apoptosis
#14
Entaz Bahar, Geum-Hwa Lee, Kashi Raj Bhattarai, Hwa-Young Lee, Min-Kyung Choi, Harun-Or Rashid, Ji-Ye Kim, Han-Jung Chae, Hyonok Yoon
Manganese (Mn) is an important trace element present in human body, which acts as an enzyme co-factor or activator in various metabolic reactions. While essential in trace amounts, excess levels of Mn in human brain can produce neurotoxicity, including idiopathic Parkinson's disease (PD)-like extrapyramidal manganism symptoms. This study aimed to investigate the protective role of polyphenolic extract of Euphorbia supina (PPEES) on Mn-induced neurotoxicity and the underlying mechanism in human neuroblastoma SKNMC cells and Sprague-Dawley (SD) male rat brain...
January 30, 2017: International Journal of Molecular Sciences
https://www.readbyqxmd.com/read/28137957/loss-of-function-mutations-in-the-atp13a2-park9-gene-cause-complicated-hereditary-spastic-paraplegia-spg78
#15
Alejandro Estrada-Cuzcano, Shaun Martin, Teodora Chamova, Matthis Synofzik, Dagmar Timmann, Tine Holemans, Albena Andreeva, Jennifer Reichbauer, Riet De Rycke, Dae-In Chang, Sarah van Veen, Jean Samuel, Ludger Schöls, Thorsten Pöppel, Danny Mollerup Sørensen, Bob Asselbergh, Christine Klein, Stephan Zuchner, Albena Jordanova, Peter Vangheluwe, Ivailo Tournev, Rebecca Schüle
Hereditary spastic paraplegias are heterogeneous neurodegenerative disorders characterized by progressive spasticity of the lower limbs due to degeneration of the corticospinal motor neurons. In a Bulgarian family with three siblings affected by complicated hereditary spastic paraplegia, we performed whole exome sequencing and homozygosity mapping and identified a homozygous p.Thr512Ile (c.1535C > T) mutation in ATP13A2. Molecular defects in this gene have been causally associated with Kufor-Rakeb syndrome (#606693), an autosomal recessive form of juvenile-onset parkinsonism, and neuronal ceroid lipofuscinosis (#606693), a neurodegenerative disorder characterized by the intracellular accumulation of autofluorescent lipopigments...
February 2017: Brain: a Journal of Neurology
https://www.readbyqxmd.com/read/28137432/higher-than-recommended-dosages-of-antipsychotics-in-male-patients-with-schizophrenia-are-associated-with-increased-depression-but-no-major-neurocognitive-side-effects-results-of-a-cross-sectional-pilot-naturalistic-study
#16
Konstantinos N Fountoulakis, Xenia Gonda, Melina Siamouli, Katerina Moutou, Zoe Nitsa, Brian E Leonard, Siegfried Kasper
INTRODUCTION: The current small pilot naturalistic cross-sectional study assesses whether higher dosages of antipsychotics are related to a satisfactory outcome concerning symptoms of schizophrenia but also to a worse outcome in terms of adverse events and neurocognitive function. MATERIAL AND METHODS: 41 male stabilized hospitalized schizophrenic patients were assessed by PANSS, Calgary Depression Rating Scale, UKU and Simpson-Angus Scale and a battery of neurocognitive tests...
January 28, 2017: Progress in Neuro-psychopharmacology & Biological Psychiatry
https://www.readbyqxmd.com/read/28133816/typical-and-atypical-pathology-in-primary-progressive-aphasia-variants
#17
Edoardo G Spinelli, Maria Luisa Mandelli, Zachary A Miller, Miguel A Santos-Santos, Stephen M Wilson, Federica Agosta, Lea T Grinberg, Eric J Huang, John Q Trojanowski, Marita Meyer, Maya L Henry, Giancarlo Comi, Gil Rabinovici, Howard J Rosen, Massimo Filippi, Bruce L Miller, William W Seeley, Maria Luisa Gorno-Tempini
OBJECTIVE: To characterize in vivo signatures of pathological diagnosis in a large cohort of patients with primary progressive aphasia (PPA) variants defined by current diagnostic classification. METHODS: Extensive clinical, cognitive, neuroimaging, and neuropathological data were collected from 69 patients with sporadic PPA, divided into 29 semantic (svPPA), 25 non-fluent (nfvPPA), 11 logopenic (lvPPA), and 4 mixed PPA. Patterns of grey matter (GM) and white matter (WM) atrophy at presentation were assessed and tested as predictors of pathological diagnosis using support vector machine (SVM) algorithms...
January 30, 2017: Annals of Neurology
https://www.readbyqxmd.com/read/28132899/pathological-consequences-of-micu1-mutations-on-mitochondrial-calcium-signalling-and-bioenergetics
#18
Gauri Bhosale, Jenny Sharpe, Amanda Koh, Antonina Kouli, Gyorgy Szabadkai, Michael R Duchen
Loss of function mutations of the protein MICU1, a regulator of mitochondrial Ca(2+) uptake, cause a neuronal and muscular disorder characterised by impaired cognition, muscle weakness and an extrapyramidal motor disorder. We have shown previously that MICU1 mutations cause increased resting mitochondrial Ca(2+) concentration ([Ca(2+)]m). We now explore the functional consequences of MICU1 mutations in patient derived fibroblasts in order to clarify the underlying pathophysiology of this disorder. We propose that deregulation of mitochondrial Ca(2+) uptake through loss of MICU1 raises resting [Ca(2+)]m, initiating a futile Ca(2+) cycle, whereby continuous mitochondrial Ca(2+) influx is balanced by Ca(2+) efflux through the sodium calcium exchanger (NLCXm)...
January 26, 2017: Biochimica et Biophysica Acta
https://www.readbyqxmd.com/read/28131725/the-5-alpha-reductase-inhibitor-finasteride-reduces-dyskinesia-in-a-rat-model-of-parkinson-s-disease
#19
Roberto Frau, Paola Savoia, Silvia Fanni, Chiara Fiorentini, Camino Fidalgo, Elisabetta Tronci, Roberto Stancampiano, Mario Meloni, Antonino Cannas, Francesco Marrosu, Marco Bortolato, Paola Devoto, Cristina Missale, Manolo Carta
Levodopa-induced dyskinesia (LID) is a disabling motor complication occurring in Parkinson's disease patients (PD) after long-term l-DOPA treatment. Although its etiology remains unclear, there is accumulating evidence that LID relies on an excessive dopamine receptor transmission, particularly at the downstream signaling of D1 receptors. We previously reported that the pharmacological blockade of 5-alpha reductase (5AR), the rate limiting enzyme in neurosteroids synthesis, rescued a number of behavioral aberrations induced by D1 receptor-selective and non-selective agonists, without inducing extrapyramidal symptoms...
January 26, 2017: Experimental Neurology
https://www.readbyqxmd.com/read/28131463/very-early-onset-sporadic-alzheimer-s-disease-with-a-de-novo-mutation-in-the-psen1-gene
#20
Fan Lou, Xiaoguang Luo, Ming Li, Yan Ren, Zhiyi He
We report a 22-year onset age man diagnosed with rapidly progressing early-onset Alzheimer's disease with predominant extrapyramidal symptoms as the initial presenting symptoms and V391G mutation in presenilin 1 gene (PSEN1) was founded. The unaffected parents of the proband are not carriers of the mutation but have histories of extrapyramidal diseases, suggesting de novo origin of V391G mutation. The Val391Gly variation widens the number of PSEN1 mutations responsible for early-onset Alzheimer's disease with extrapyramidal phenotype and would help to establish a functional map of presenilin 1 protein architecture...
January 6, 2017: Neurobiology of Aging
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