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https://www.readbyqxmd.com/read/29159848/speech-deterioration-in-amyotrophic-lateral-sclerosis-als-after-manifestation-of-bulbar-symptoms
#1
Tanja Makkonen, Hanna Ruottinen, Riitta Puhto, Mika Helminen, Johanna Palmio
BACKGROUND: The symptoms and their progression in amyotrophic lateral sclerosis (ALS) are typically studied after the diagnosis has been confirmed. However, many people with ALS already have severe dysarthria and loss of adequate speech at the time of diagnosis. Speech-and-language therapy interventions should be targeted timely based on communicative need in ALS. AIMS: To investigate how long natural speech will remain functional and to identify the changes in the speech of persons with ALS...
November 21, 2017: International Journal of Language & Communication Disorders
https://www.readbyqxmd.com/read/29134445/ptosis-and-bulbar-onset-an-unusual-phenotype-of-familial-als
#2
Fabiola De Marchi, L Corrado, E Bersano, M F Sarnelli, V Solara, S D'Alfonso, R Cantello, L Mazzini
Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disorder of upper and lower motor neurons that usually spare the oculomotor nerves. Here, we describe a case of two siblings with a familial bulbar-onset ALS both with ptosis manifested at the onset of the disease.
November 13, 2017: Neurological Sciences
https://www.readbyqxmd.com/read/29126546/changes-in-lingual-alveolar-contact-pressure-during-speech-over-six-months-in-amyotrophic-lateral-sclerosis
#3
Jeff Searl, Stephanie Knollhoff
PURPOSE: This study compared lingual-alveolar contact pressures during consonant (LACP-Sp) production between adults with and without amyotrophic lateral sclerosis (ALS) over three visits within six months and assessed the relationship between LACP-Sp and speech intelligibility. METHODS: Ten people with ALS and nine without read sentences with lingual-alveolar consonants embedded as LACP-Sp was recorded. Recordings were done at study entry, then three and six months after entry...
November 2017: Journal of Communication Disorders
https://www.readbyqxmd.com/read/29113448/can-oxidation-reduction-potential-of-cerebrospinal-fluid-be-a-monitoring-biomarker-in-amyotrophic-lateral-sclerosis
#4
Milos Opacic, Zorica Stevic, Vladimir Bascarevic, Miroslav Zivic, Mihajlo Spasic, Ivan Spasojevic
The monitoring of progression in amyotrophic lateral sclerosis (ALS) relies on clinical outcome measures that take months to interpret, such as revised ALS functional rating scale (ALSFRS-R) score, with no approved biomarkers. A number of clinical studies have documented the involvement of oxidative stress in ALS pathology. Pertinent to this, we propose to evaluate oxidation-reduction potential (ORP) of cerebrospinal fluid (CSF) as a potential indicator of ALS progression. The case/control study included 24 patients with neurological non-neurodegenerative disorders (controls), and 82 ALS patients with different degrees of disease (ALSFRS-R score: 21-47)...
November 7, 2017: Antioxidants & Redox Signaling
https://www.readbyqxmd.com/read/29084402/health-status-perspectives-in-amyotrophic-lateral-sclerosis
#5
Susana Pinto, Mamede de Carvalho
BACKGROUND/AIMS: The global perception of the health status (HS) of amyotrophic lateral sclerosis (ALS) patients before the initial diagnosis has not been addressed previously. METHODS: We recorded the following at the first visit, before diagnostic information: (1) visual analog scale (VAS) of the EQ-5D; (2) the revised ALS functional rating scale (ALSFRS- R), bulbar (ALSFRSb), upper limb (ALSFRSul), lower limb (ALSFRSll), and respiratory (RofALSFRS-R) subscores; and (3) forced and slow vital capacities...
October 31, 2017: Neuro-degenerative Diseases
https://www.readbyqxmd.com/read/29082510/brain-signal-intensity-changes-as-biomarkers-in-amyotrophic-lateral-sclerosis
#6
Juan F Vázquez-Costa, Miguel Mazón, Joan Carreres-Polo, David Hervás, Jordi Pérez-Tur, Luis Martí-Bonmatí, Teresa Sevilla
OBJECTIVES: To evaluate the contribution of the demographical, clinical, analytical and genetic factors to brain signal intensity changes in T2-weighted MR images in amyotrophic lateral sclerosis (ALS) patients and controls. METHODS: Susceptibility-weighted and FLAIR sequences were obtained in a 3T MR scanner. Iron-related hypointensities in the motor cortex (IRhMC) and hyperintensities of the corticospinal tract (HCT) were qualitatively scored. Age, gender, family history and clinical variables were recorded...
October 29, 2017: Acta Neurologica Scandinavica
https://www.readbyqxmd.com/read/29073458/resting-state-fmri-correlates-of-theory-of-mind-impairment-in-amyotrophic-lateral-sclerosis
#7
Francesca Trojsi, Federica Di Nardo, Gabriella Santangelo, Mattia Siciliano, Cinzia Femiano, Carla Passaniti, Giuseppina Caiazzo, Michele Fratello, Mario Cirillo, Maria Rosaria Monsurrò, Fabrizio Esposito, Gioacchino Tedeschi
Theory of Mind (ToM), the ability to recognize thoughts and emotions of another, may be one of the cognitive domains affected in amyotrophic lateral sclerosis (ALS), a neurodegenerative disease now recognized as a multi-system disorder. The present study aimed to identify early dysfunctions of brain resting state functional magnetic resonance imaging (RS-fMRI) networks in a group of ALS patients longitudinally explored for impairment of "cognitive" and "affective" ToM subcomponents. RS-fMRI connectivity was investigated in a group of 21 patients with ALS (i...
October 3, 2017: Cortex; a Journal Devoted to the Study of the Nervous System and Behavior
https://www.readbyqxmd.com/read/29067136/amyotrophic-lateral-sclerosis-an-acupuncture-approach
#8
Poovadan Sudhakaran
Background: Amyotrophic lateral sclerosis (ALS) is an adult onset neurodegenerative disorder that has no curative treatment and is usually fatal. Modern medicine treatment is mostly supportive. Acupuncture has much more to offer by way of symptomatic relief and improving quality of life (QoL). Useful points for the treatment of bulbar paralysis, paralysis of upper and lower extremities, and correction of underlying Disharmony Patterns are discussed and an illustrative case is presented to demonstrate the acupuncture approach for treating ALS...
October 1, 2017: Medical Acupuncture
https://www.readbyqxmd.com/read/29055436/brain-white-matter-demyelinating-lesions-and-amyotrophic-lateral-sclerosis-in-a-patient-with-c9orf72-hexanucleotide-repeat-expansion
#9
Miguel Oliveira Santos, Inês Caldeira, Marta Gromicho, Ana Pronto-Laborinho, Mamede de Carvalho
A hexanucleotide repeat expansion in the C9orf72 gene is associated with amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration. It has been described before four patients with multiple sclerosis (MS) and C9orf72-ALS. However, C9orf72 positivity is not associated with increased risk of MS. Inflammatory pathways related to NF-κB have been linked to ALS and MS, and appear to be important in C9orf72-ALS patients. A 42-year-old woman presented with progressive bulbar symptoms for 9 months. Neurological examination disclosed spastic dysarthria, atrophic tongue with fasciculations, brisk jaw and limb tendon reflexes, and bilateral Hoffman sign...
October 2017: Multiple Sclerosis and related Disorders
https://www.readbyqxmd.com/read/29055222/the-cerebrospinal-fluid-values-of-advanced-oxidation-protein-products-and-total-thiol-content-in-patients-with-amyotrophic-lateral-sclerosis
#10
Gordana Djordjevic, Srdjan Ljubisavljevic, Srdjan Sretenovic, Gordana Kocic, Ivana Stojanovic, Svetlana Stojanovic
OBJECTIVES: Amyotrophic lateral sclerosis (ALS) is recognized as a progressive neurodegenerative disorder of unknown origin. Oxidative stress (OS) is considered as one of the most challenging hypothesis in the disease pathogenesis. The aim of this study was to contribute to the understanding of what extent there is involvement of OS in ALS. PATIENTS AND METHODS: We assessed Advanced Oxidation Protein Products (AOPP) and total thiol (-SH) groups in cerebrospinal fluid (CSF) of 24 ALS patients (13 of them presented with spinal form while 11 patients had bulbar form) and 20 controls (CG)...
October 18, 2017: Clinical Neurology and Neurosurgery
https://www.readbyqxmd.com/read/29054915/prevalence-of-sleep-apnoea-and-capnographic-detection-of-nocturnal-hypoventilation-in-amyotrophic-lateral-sclerosis
#11
Matthias Boentert, Christian Glatz, Cornelia Helmle, Angelika Okegwo, Peter Young
OBJECTIVE: This retrospective study aimed to investigate whether overnight oxymetry and early morning blood gas analysis predict nocturnal hypoventilation (NH) as reflected by night-time hypercapnia in patients with amyotrophic lateral sclerosis (ALS). In addition, prevalence and clinical determinants of sleep apnoea in ALS were evaluated. METHODS: In 250 patients with non-ventilated ALS, transcutaneous capnometry was performed along with polysomnography or polygraphy and early morning blood gases...
October 20, 2017: Journal of Neurology, Neurosurgery, and Psychiatry
https://www.readbyqxmd.com/read/29054330/tongue-strength-in-patients-with-subacute-myelo-optico-neuropathy
#12
Satoshi Yamashita, Tatsuya Nakama, Mitsuharu Ueda, Shoji Honda, En Kimura, Masaaki Konagaya, Yukio Ando
Subacute myelo-optico-neuropathy (SMON) is a neurodegenerative disease that may be caused by overdose or prolonged oral administration of clioquinol. Recently, dysphagia has attracted attention as a complication of SMON. To investigate lingual control in SMON, we examined patients with SMON using assessments of maximum tongue pressure, compared with dysphagia-related diseases, such as sporadic inclusion body myositis (sIBM) and amyotrophic lateral sclerosis (ALS), and healthy volunteer. The mean maximum tongue pressure (Pmax) in patients with SMON was 14...
October 17, 2017: Journal of Clinical Neuroscience: Official Journal of the Neurosurgical Society of Australasia
https://www.readbyqxmd.com/read/29037065/patterns-of-symptom-development-in-patients-with-motor-neuron-disease
#13
Renée Walhout, Esther Verstraete, Martijn P Van Den Heuvel, Jan H Veldink, Leonard H Van Den Berg
OBJECTIVE: To investigate whether symptom development in motor neuron disease (MND) is a random or organized process. METHODS: Six hundred patients with amyotrophic lateral sclerosis (ALS), upper motor neuron (UMN) or lower motor neuron (LMN) phenotypes were invited for a questionnaire concerning symptom development. A binomial test was used to examine distribution of symptoms from site of onset. Development of symptoms over time was evaluated by Kaplan-Meier analysis...
October 16, 2017: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
https://www.readbyqxmd.com/read/29028499/fasciculations-in-nerve-and-muscle-disorders-a-prospective-study-of-muscle-ultrasound-compared-to-electromyography
#14
M T Johansson, H R Ellegaard, H Tankisi, A Fuglsang-Frederiksen, E Qerama
OBJECTIVES: We examined the clinical utility of muscle ultrasound (MUS) in detecting fasciculations in patients with nerve and muscle disorders (NMD) and investigated the impact on diagnostic sensitivity when combining electromyography (EMG) and MUS. METHODS: We included 58 consecutive patients suspected to have NMD and 38 healthy subjects (HS). Patients and HS underwent MUS in 14 skeletal and two bulbar muscles and the video recordings of the MUS were anonymised...
September 22, 2017: Clinical Neurophysiology: Official Journal of the International Federation of Clinical Neurophysiology
https://www.readbyqxmd.com/read/28982219/mechanical-ventilation-for-amyotrophic-lateral-sclerosis-motor-neuron-disease
#15
REVIEW
Aleksandar Radunovic, Djillali Annane, Muhammad K Rafiq, Ruth Brassington, Naveed Mustfa
BACKGROUND: Amyotrophic lateral sclerosis (ALS), also known as motor neuron disease, is a fatal neurodegenerative disease. Neuromuscular respiratory failure is the most common cause of death, which usually occurs within two to five years of the disease onset. Supporting respiratory function with mechanical ventilation may improve survival and quality of life. This is the second update of a review first published in 2009. OBJECTIVES: To assess the effects of mechanical ventilation (tracheostomy-assisted ventilation and non-invasive ventilation (NIV)) on survival, functional measures of disease progression, and quality of life in ALS, and to evaluate adverse events related to the intervention...
October 6, 2017: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/28980485/sympathetic-vascular-response-to-facial-cooling-is-increased-in-flail-phenotypes-of-amyotrophic-lateral-sclerosis
#16
Marcin Tutaj, MaŁgorzata Miller, Barbara Tomik, Aleksandra Golenia, Agnieszka Stanuszek, Katarzyna BŁońska, Agnieszka SŁowik
OBJECTIVE: To assess cardiovascular responses to cold face test (CFT) in patients with classic-onset ALS (bulbar or limb onset, ALS-C) and in patients with flail arm and flail leg phenotypes (FA/FL). METHODS: In 18 ALS-C, eight FA/FL patients and 10 age-matched controls we continuously monitored heart rate (HR), systolic (SBP), diastolic (DBP) and mean blood pressure (MBP) during two-minute baseline and one-minute cold stimulus application. HR and BP responses to CFT were calculated as differences between the peak responses and baseline values (dHR, dSBP, dDBP, dMBP), as percent changes from baseline (dHR%, dSBP%, dDBP%, dMBP%), and also latencies and durations of HR and BP responses were assessed (LatHR, tHR, LatBP, tBP)...
October 5, 2017: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
https://www.readbyqxmd.com/read/28914354/comorbidity-of-dementia-with-amyotrophic-lateral-sclerosis-als-insights-from-a-large-multicenter-italian-cohort
#17
Francesca Trojsi, Mattia Siciliano, Cinzia Femiano, Gabriella Santangelo, Christian Lunetta, Andrea Calvo, Cristina Moglia, Kalliopi Marinou, Nicola Ticozzi, Gianluca Drago Ferrante, Carlo Scialò, Gianni Sorarù, Amelia Conte, Yuri M Falzone, Rosanna Tortelli, Massimo Russo, Valeria Ada Sansone, Adriano Chiò, Gabriele Mora, Barbara Poletti, Paolo Volanti, Claudia Caponnetto, Giorgia Querin, Mario Sabatelli, Nilo Riva, Giancarlo Logroscino, Sonia Messina, Antonio Fasano, Maria Rosaria Monsurrò, Gioacchino Tedeschi, Jessica Mandrioli
To assess the association, at diagnosis, between amyotrophic lateral sclerosis (ALS) and dementia in a large cohort of well-characterized Italian patients. We investigated the phenotypic profile of 1638 incident patients with definite, probable or laboratory-supported probable ALS, diagnosed from January 2009 to December 2013 in 13 Italian Referral Centers, located in 10 Italian Regions, and classified in two independent subsamples accounting for presence or not of dementia. The collected ALS features, including survival and other follow-up data, were compared between the two subgroups using a one-way analysis of variance and Chi-square test, as appropriate, logistic regression models and Kaplan-Meier survival analysis...
November 2017: Journal of Neurology
https://www.readbyqxmd.com/read/28884711/-clinical-polymorphism-of-amyotrophic-lateral-sclerosis
#18
E A Kovrazhkina, O D Razinskaya, L V Gubsky
AIM: To clarify clinical polymorphism of amyotrophic lateral sclerosis (ALS). MATERIAL AND METHODS: The study was based on records of a hospital personalized register. Ninety-four patients, aged from 25 to 81 years, diagnosed with ALS according to El Escorial criteria were included. Electromyography and, if necessary, transcranial magnetic stimulation and magnetic-resonance tomography were used to confirm the diagnosis. Disease progression was assessed with the ARSFRS...
2017: Zhurnal Nevrologii i Psikhiatrii Imeni S.S. Korsakova
https://www.readbyqxmd.com/read/28873374/characteristics-and-prognosis-of-oldest-old-subjects-with-amyotrophic-lateral-sclerosis
#19
Meira Dandaba, Philippe Couratier, Anaïs Labrunie, Marie Nicol, Bello Hamidou, Marie Raymondeau, Giancarlo Logroscino, Pierre Marie Preux, Benoît Marin
BACKGROUND: Amyotrophic Lateral Sclerosis (ALS) is an age-related neurodegenerative disease with unclear characteristics and prognosis in the oldest old (80 years and over). The aim of this study was to compare the oldest old and younger ALS patients in terms of clinical and socio-demographic characteristics, and prognosis. METHODS: ALS incident cases from the register of ALS in Limousin (FRALim), diagnosed between January 2000 and July 2013, were included. Descriptive and comparative analyses by age group were carried out...
2017: Neuroepidemiology
https://www.readbyqxmd.com/read/28872913/post-hoc-analysis-of-randomised-placebo-controlled-double-blind-study-mci186-19-of-edaravone-mci-186-in-amyotrophic-lateral-sclerosis
#20
Koji Takei, Fumihiro Takahashi, Shawn Liu, Kikumi Tsuda, Joseph Palumbo
Post-hoc analyses of the ALS Functional Rating Scale-Revised (ALSFRS-R) score data, the primary endpoint in the 24-week double-blind placebo-controlled study of edaravone (MCI186-19, NCT01492686), were performed to confirm statistical robustness of the result. The previously reported original analysis had used a last observation carried forward (LOCF) method and also excluded patients with fewer than three completed treatment cycles. The post-hoc sensitivity analyses used different statistical methods as follows: 1) including all patients regardless of treatment cycles received (ALL LOCF); 2) a mixed model for repeated measurements (MMRM) analysis; and 3) the Combined Assessment of Function and Survival (CAFS) endpoint...
October 2017: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
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