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https://www.readbyqxmd.com/read/28101650/speech-disorders-in-parkinson-s-disease-early-diagnostics-and-effects-of-medication-and-brain-stimulation
#1
REVIEW
L Brabenec, J Mekyska, Z Galaz, Irena Rektorova
Hypokinetic dysarthria (HD) occurs in 90% of Parkinson's disease (PD) patients. It manifests specifically in the areas of articulation, phonation, prosody, speech fluency, and faciokinesis. We aimed to systematically review papers on HD in PD with a special focus on (1) early PD diagnosis and monitoring of the disease progression using acoustic voice and speech analysis, and (2) functional imaging studies exploring neural correlates of HD in PD, and (3) clinical studies using acoustic analysis to evaluate effects of dopaminergic medication and brain stimulation...
January 18, 2017: Journal of Neural Transmission
https://www.readbyqxmd.com/read/28095350/progressive-multifocal-leukoencephalopathy-in-a-patient-with-systemic-mastocytosis-treated-with-cladribine
#2
Karl B Alstadhaug, Randi Fykse Halstensen, Francis Odeh
BACKGROUND: Progressive multifocal leukoencephalopathy (PML) is a rare opportunistic brain infection caused by the human polyomavirus JC (JCPyV). A particular problem with the drug cladribine seems to be prolonged suppression of the CD4+ T-cells, a well-known risk factor for PML. CASE DESCRIPTION: A 67-year-old male presented with a 3-weeks history of unsteady gait, dysarthria and a dysfunctional right arm. Seven years earlier, he had been diagnosed with urticaria pigmentosa, and 2 years later aggressive systemic mastocytosis...
December 14, 2016: Journal of Clinical Virology: the Official Publication of the Pan American Society for Clinical Virology
https://www.readbyqxmd.com/read/28074593/microtubule-associated-protein-map-1b-novel-paraneoplastic-biomarker-running-head-map-1b-igg-novel-paraneoplastic-biomarker
#3
Avi Gadoth, Thomas J Kryzer, Jim Fryer, Andrew McKeon, Vanda A Lennon, Sean J Pittock
To report the identification of microtubule associated protein (MAP)1B as the antigen of the previously described PCA-2 antibody, its frequency and clinical, oncological and serological associations. Methods Archival serum or cerebrospinal fluid (CSF) specimens were available from 96 of 118 consecutive PCA-2-IgG-seropositive patients identified 1993-2016. The autoantigen, defined in mouse brain lysate by western blot and mass spectrometry, was confirmed by dual immunohistochemical staining using commercial antibodies...
January 11, 2017: Annals of Neurology
https://www.readbyqxmd.com/read/28074215/-management-of-dysphagia-in-acute-stroke-a%C3%A2-prospective-study-for-validation-of-current-recommendations
#4
B Lindner-Pfleghar, H Neugebauer, S Stösser, J Kassubek, A Ludolph, R Dziewas, M Prosiegel, A Riecker
BACKGROUND: The German expert recommendations on the management of dysphagia in patients after acute stroke suggest an algorithm for clinical and technical investigations to identify patients at risk for aspiration and thus reduce the rate of aspiration pneumonia. The effectiveness of this algorithm has, however, not yet been prospectively validated . METHODS: In this study 144 consecutive stroke patients were assessed by a full bedside swallowing assessment including the screening procedures of standardized swallowing assessment (SSA) and 2 out of 6...
January 10, 2017: Der Nervenarzt
https://www.readbyqxmd.com/read/28074148/apraxia-of-speech-and-cerebellar-mutism-syndrome-a-case-report
#5
E De Witte, I Wilssens, D De Surgeloose, G Dua, M Moens, J Verhoeven, M Manto, P Mariën
BACKGROUND: Cerebellar mutism syndrome (CMS) or posterior fossa syndrome (PFS) consists of a constellation of neuropsychiatric, neuropsychological and neurogenic speech and language deficits. It is most commonly observed in children after posterior fossa tumor surgery. The most prominent feature of CMS is mutism, which generally starts after a few days after the operation, has a limited duration and is typically followed by motor speech deficits. However, the core speech disorder subserving CMS is still unclear...
2017: Cerebellum & Ataxias
https://www.readbyqxmd.com/read/28063983/loss-of-laforin-or-malin-results-in-increased-drp1-level-and-concomitant-mitochondrial-fragmentation-in-lafora-disease-mouse-models
#6
Mamta Upadhyay, Saloni Agarwal, Pratibha Bhadauriya, Subramaniam Ganesh
Lafora disease (LD) is an autosomal recessive form of a fatal disorder characterized by the myoclonus epilepsy, ataxia, psychosis, dementia, and dysarthria. A hallmark of LD is the presence of abnormal glycogen inclusions called Lafora bodies in the affected tissues including the neurons. LD can be caused by defects either in the laforin phosphatase coded by the EPM2A gene or in the malin E3 ubiquitin ligase coded by the NHLRC1 gene. The mouse models of LD, created by the targeted disruption of the LD genes, display several neurodegenerative changes...
January 4, 2017: Neurobiology of Disease
https://www.readbyqxmd.com/read/28056870/anti-n-methyl-d-aspartate-receptor-nmdar-antibody-encephalitis-presents-in-atypical-types-and-coexists-with-neuromyelitis-optica-spectrum-disorder-or-neurosyphilis
#7
Kaiyu Qin, Wenqing Wu, Yuming Huang, Dongmei Xu, Lei Zhang, Bowen Zheng, Meijuan Jiang, Cheng Kou, Junhua Gao, Wurong Li, Jinglin Zhang, Sumei Wang, Yanfei Luan, Chaoling Yan, Dan Xu, Xinmei Zheng
BACKGROUND: Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is a clinically heterogeneous disorder characterized by epileptic seizures, psychosis, dyskinesia, consciousness impairments, and autonomic instability. Symptoms are always various. Sometimes it presents in milder or incomplete forms. We report 4 cases of anti-NMDAR encephalitis with incomplete forms, 3 cases of which were accompanied by neuromyelitis optica spectrum disorder or neurosyphilis respectively. CASE PRESENTATION: A 33-year-old man presented with dysarthria, movement disorder and occasional seizures...
January 5, 2017: BMC Neurology
https://www.readbyqxmd.com/read/28055131/subthalamic-nuclei-stimulation-in-patients-with-pantothenate-kinase-associated-neurodegeneration-pkan
#8
Ziyuan Liu, Yang Liu, Yingmai Yang, Lin Wang, Wanchen Dou, Jinzhu Guo, Yu Wang, Yi Guo, Xinhua Wan, Wenbin Ma, Renzhi Wang
INTRODUCTION: Pantothenate kinase-associated neurodegeneration (PKAN) is a rare autosomal recessive genetic disease that leads to extrapyramidal symptoms, such as dystonia, ataxia, dysarthria, and involuntary movements. Treatment of PKAN with deep brain stimulation (DBS) has been reported, but mainly focuses on targeting the globus pallidus internus (GPi). Subthalamic nuclei (STN) may also be a potential target for treatment of PKAN. METHODS: In this study, we reviewed three patients with PKAN (two with typical PKAN and one with atypical PKAN) treated by bilateral STN stimulation and present a review of the literature...
January 5, 2017: Neuromodulation: Journal of the International Neuromodulation Society
https://www.readbyqxmd.com/read/28049884/a-case-of-cardioembolic-stroke-due-to-intracardiac-papillary-fibroelastoma-evaluated-by-using-transesophageal-echocardiography
#9
Yuichiro Ohya, Shigeru Fujimoto, Makoto Kanazawa, Naoki Tagawa, Masato Osaki, Takanari Kitazono
A 62-year-old woman had a prior ischemic stroke in the right temporal lobe with dysarthria and dysesthesia of the left hand. Embolic stroke of undetermined source (ESUS) was diagnosed and warfarin was administered. However, transient ischemic attack recurred upon admission to our hospital. Paroxysmal atrial fibrillation and cerebral arterial stenotic lesions were absent. Transesophageal echocardiography revealed a mobile hyperechoic structure on the aortic valve indicating papillary fibroelastoma. She was diagnosed with a brain embolism due to the intracardiac tumor which was surgically excised and pathologically confirmed as papillary fibroelastoma...
December 28, 2016: Rinshō Shinkeigaku, Clinical Neurology
https://www.readbyqxmd.com/read/28033738/the-production-effect-in-adults-with-dysarthria-improving-long-term-verbal-memory-by-vocal-production
#10
Michal Icht, Orly Bergerzon-Biton, Yaniv Mama
People show better memory for words read aloud relative to words read silently, the Production Effect (PE). Vocalisation at study makes the produced (aloud) words more distinct than the non-produced (silent) words, hence more memorable. Such encoding distinctiveness is related to the additional processing of aloud words that is later used during retrieval. This study investigated the PE in dysarthric adults, characterised by speech production difficulties. Their memory performance (recognition) was compared to a group of healthy adults...
December 29, 2016: Neuropsychological Rehabilitation
https://www.readbyqxmd.com/read/28018469/type-i-chiari-malformation-presenting-orthostatic-syncope-who-treated-with-decompressive-surgery
#11
Hyun-Seung Shin, Jeong A Kim, Dong-Seok Kim, Joon Soo Lee
Chiari malformations are a congenital anomaly of the hindbrain. The most common, Chiari malformation type I (CM-I), is characterized by herniation of the cerebellar tonsils extending at least 3 mm below the plane of the foramen magnum. Consequently, CM-I is associated with hydrocephalus and symptoms involving compression of the cervicomedullary junction by ectopic tonsils. Several studies have reported the clinical symptoms associated with CM-I, including suboccipital headache, weakness in the upper extremities, facial numbness, loss of temperature sensation, ataxia, diplopia, dysarthria, dysphagia, vomiting, vertigo, nystagmus, and tinnitus...
November 2016: Korean Journal of Pediatrics
https://www.readbyqxmd.com/read/28018166/motor-dysfunctions-and-neuropathology-in-mouse-models-of-spinocerebellar-ataxia-type-2-a-comprehensive-review
#12
REVIEW
João M Da Conceição Alves-Cruzeiro, Liliana Mendonça, Luís Pereira de Almeida, Clévio Nóbrega
Spinocerebellar ataxia type 2 (SCA2) is an autosomal dominant ataxia caused by an expansion of CAG repeats in the exon 1 of the gene ATXN2, conferring a gain of toxic function that triggers the appearance of the disease phenotype. SCA2 is characterized by several symptoms including progressive gait ataxia and dysarthria, slow saccadic eye movements, sleep disturbances, cognitive impairments, and psychological dysfunctions such as insomnia and depression, among others. The available treatments rely on palliative care, which mitigate some of the major symptoms but ultimately fail to block the disease progression...
2016: Frontiers in Neuroscience
https://www.readbyqxmd.com/read/28017257/autosomal-recessive-cerebellar-ataxia-type-1-mimicking-multiple-sclerosis-a-report-of-two-siblings-with-a-novel-mutation-in-syne1-gene-in-a-saudi-family
#13
Hussein Algahtani, Yousef Marzouk, Raghad Algahtani, Sali Salman, Bader Shirah
Autosomal Recessive Cerebellar Ataxia type 1 (ARCA1), also known as recessive ataxia of Beauce, is an adult onset pure cerebellar ataxia that typically presents with cerebellar ataxia and/or dysarthria. A mutation in the synaptic nuclear envelope protein 1 (SYNE1) gene that is located on chromosome 6p25 results in premature termination of the protein. It was first reported in 2007 as the first identified gene responsible for a recessively inherited pure cerebellar ataxia. In this article, we are presenting two brothers with ARCA1 who were misdiagnosed and treated as multiple sclerosis for more than a decade...
January 15, 2017: Journal of the Neurological Sciences
https://www.readbyqxmd.com/read/28009766/urgent-bypass-surgery-following-failed-endovascular-treatment-in-acute-symptomatic-stroke-patient-with-mca-occlusion
#14
Chang Yeob Lee, Chang Hyun Kim, Chang-Young Lee, Sung-Il Sohn, Jeong-Ho Hong
INTRODUCTION: Although the benefits of extracranial-intracranial bypass surgery remain controversial, there is some surgical rationale for the augmentation of cerebral blood flow in cases of acute ischemic stroke with hemodynamic instability. CASE REPORT: We report a case of a 62-year-old woman who suddenly developed right hemiplegia and global aphasia. Initial magnetic resonance imaging and magnetic resonance angiography revealed a small acute ischemic lesion in left parietal lobe with occlusion at the left middle cerebral artery...
January 2017: Neurologist
https://www.readbyqxmd.com/read/28006860/paraneoplastic-cerebellar-degeneration-and-lambert-eaton-myasthenia-in-a-patient-with-merkel-cell-carcinoma-and-voltage-gated-calcium-channel-antibodies
#15
Lucia Pavolucci, Giulia Giannini, Maria Pia Giannoccaro, Maria Pia Foschini, Bethan Lang, Patrizia Avoni, Paolo Tinuper, Angela Vincent, Rocco Liguori
INTRODUCTION: Merkel cell carcinoma is a rare cutaneous, aggressive tumor. Although it shares many neuroendocrine features with small cell lung carcinoma, it has only occasionally been reported with paraneoplastic neurological syndromes. METHODS: A healthy 67 year-old man developed acute ataxia, vertigo, and nausea. Subsequently he also developed dysarthria, diplopia, xerostomia, fatigability and progressive anorexia. He underwent a full diagnostic workup and was found to have a high titer of voltage-gated calcium channel antibodies in serum and cerebrospinal fluid, neurophysiological findings compatible with Lambert-Eaton myasthenia and neurological signs compatible with cerebellar degeneration...
December 22, 2016: Muscle & Nerve
https://www.readbyqxmd.com/read/28002479/recovernow-feasibility-of-a-mobile-tablet-based-rehabilitation-intervention-to-treat-post-stroke-communication-deficits-in-the-acute-care-setting
#16
Karen H Mallet, Rany M Shamloul, Dale Corbett, Hillel M Finestone, Simon Hatcher, Jim Lumsden, Franco Momoli, Michel C F Shamy, Grant Stotts, Richard H Swartz, Christine Yang, Dar Dowlatshahi
BACKGROUND: Approximately 40% of patients diagnosed with stroke experience some degree of aphasia. With limited health care resources, patients' access to speech and language therapies is often delayed. We propose using mobile-platform technology to initiate early speech-language therapy in the acute care setting. For this pilot, our objective was to assess the feasibility of a tablet-based speech-language therapy for patients with communication deficits following acute stroke. METHODS: We enrolled consecutive patients admitted with a stroke and communication deficits with NIHSS score ≥1 on the best language and/or dysarthria parameters...
2016: PloS One
https://www.readbyqxmd.com/read/28000909/-language-disorders-in-acute-cerebellitis-beyond-dysarthria
#17
D Barragan-Martinez, N Nunez-Enamorado, M Berenguer-Potenciano, N Villora-Morcillo, A Martinez de Aragon, A Camacho-Salas
INTRODUCTION: Acute cerebellitis is one of the main causes of cerebellar syndrome in infancy. Among the wide range of manifestations, headache and ataxia being the most predominant, we can find other less frequent, although nonetheless interesting, ones, such as language disorders, which go beyond the well-known cerebellar dysarthria. The different combinations in which the symptoms can appear, especially when not accompanied by ataxia, make the condition a real challenge for the clinician...
January 1, 2017: Revista de Neurologia
https://www.readbyqxmd.com/read/27994357/clinical-correlates-of-leukoaraiosis-a-study-of-175-patients
#18
Rustom S Wadia, Sandesh K Ghiya, Joshita Singh, Santosh M Sontakke, Vishwas Bharadwaj, Rahul V Sonawane, Yogesh P Bade, K Shrikanth, Nikhil Goli, Rohit Singh Chauhan, Nilesh A Nadkarni
BACKGROUND: In India, the correlates of leukoaraiosis (LA) have not been widely reported. This study was designed to investigate the factors which correlate with LA. MATERIALS AND METHODS: We included patients with LA who consented for the study and graded their severity on the basis of Fazekas scale. We excluded patients with LA who did not consent/cooperate for the study as also patients with other white matter changes which mimic LA. RESULTS: LA is a common and under-rated cause of disability...
October 2016: Annals of Indian Academy of Neurology
https://www.readbyqxmd.com/read/27992342/representation-learning-based-assistive-system-for-persons-with-dysarthria
#19
S Chandrakala, N Rajeswari
An assistive system for persons with vocal impairment due to dysarthria converts dysarthric speech to normal speech or text. Because of the articulatory deficits, dysarthric speech recognition needs a robust learning technique. Representation learning is significant for complex tasks such as dysarthric speech recognition. We focus on robust representation for dysarthric speech recognition that involves recognizing sequential patterns of varying length utterances. We propose a hybrid framework that uses a generative learning based data representation with a discriminative learning based classifier...
December 13, 2016: IEEE Transactions on Neural Systems and Rehabilitation Engineering
https://www.readbyqxmd.com/read/27990316/subthalamus-stimulation-in-parkinson-disease-accounting-for-the-bilaterality-of-contacts
#20
Jean-Jacques Lemaire, Bruno Pereira, Philippe Derost, François Vassal, Miguel Ulla, Dominique Morand, Guillaume Coll, Jean Gabrillargues, Ana Marques, Bérangère Debilly, Jérôme Coste, Franck Durif
BACKGROUND: Deep brain stimulation (DBS) in Parkinson's disease uses bi-hemispheric high-frequency stimulation within the subthalamus, however, the specific impacts of bilaterality of DBS are still not clear. Thus, we aimed to study the individual-level clinical impact of locations of right-left contact pair-up accounting for each subthalamic nucleus (STN) anatomy. METHODS: Contact locations and effects at 1 year were studied retrospectively in an unselected series of 53 patients operated between 2004 and 2010...
2016: Surgical Neurology International
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