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ALS dysphagia

Gonçalo Nunes, Carla Adriana Santos, Miguel Grunho, Jorge Fonseca
BACKGROUND: Dysphagia is common in amyotrophic lateral sclerosis (ALS) and may result in malnutrition. Endoscopic gastrostomy (PEG) is recommended when oral feeding is unsafe. This work aims to assess the effectiveness and safety of PEG feeding on improving nutritional and prognostic parameters in ALS patients. METHODS: Observational and retrospective study using records from ALS patients referred for gastrostomy. Age, gender and mortality data were collected. NRS 2002, body mass index (BMI), serum albumin, transferrin and total cholesterol were recorded at the time of PEG (T0) and repeated after 3 months (T3)...
September 20, 2016: Nutrición Hospitalaria: Organo Oficial de la Sociedad Española de Nutrición Parenteral y Enteral
Bruno Fattori, Gabriele Siciliano, Valentina Mancini, Luca Bastiani, Paolo Bongioanni, Elena Caldarazzo Ienco, Maria R Barillari, Salvatore O Romeo, Andrea Nacci
OBJECTIVE: Our aim was to evaluate the relationship between the disease severity of Amyotrophic Lateral Sclerosis (ALS) and the following parameters of Fiberoptic Endoscopic Evaluation of Swallowing (FEES): premature spillage, post-swallowing residue and aspiration. METHODS: We studied 202 patients (95 women and 107 men) with ALS; of these, 136 had spinal and 66 had bulbar onset. They were analyzed according to the Amyotrophic Lateral Sclerosis Functioning Rating Scale (ALSFRS) and the b-ALSFRS subscale (bulbar scale)...
August 25, 2016: Auris, Nasus, Larynx
Caitlin S Jackson-Tarlton, Timothy J Benstead, Steve Doucette
Amyotrophic lateral sclerosis (ALS) is associated with nutritional deficits. Gastrostomy tubes are often inserted in patients with ALS to supplement or replace oral intake. The aim of this study was to better understand the practices of gastrostomy tube insertion in patients with ALS. Pre-collected de-identified data were obtained from the Canadian Neuromuscular Disease Registry (CNDR). Feeding tube status was compared with markers of dysphagia, respiratory compromise, and weight status in both univariate and multivariate analysis by employing odds ratios...
August 18, 2016: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
Haris Hakeem, Masood Uz Zaman, Sara Khan
INTRODUCTION: Amyotrophic lateral sclerosis (ALS) carries a grim prognosis. Various ALS mimics have been reported and should be excluded before confirming this diagnosis. METHODS: We report the case of a 61-year-old man who presented with progressively worsening limb weakness and dysphagia. His examination showed mixed upper and lower motor neuron signs without sensory impairment. ALS was suspected, however atypical diffuse pain prompted diagnostic work-up to exclude other causes...
August 12, 2016: Muscle & Nerve
Andrea Nacci, Jacopo Matteucci, Salvatore Osvaldo Romeo, Stefania Santopadre, Maria Denise Cavaliere, Maria Rosaria Barillari, Stefano Berrettini, Bruno Fattori
OBJECTIVE: To perform a retrospective evaluation of the incidence of complications and adverse events during fiberoptic endoscopic evaluation of swallowing (FEES) in 2,820 examinations. PATIENTS AND METHODS: Subjects included in- and outpatients at Pisa University Hospital referred for FEES by other physicians due to dysphagia symptoms. Neurologic diseases were the most commonly diagnosed conditions in the patients tested (48.3%). Informed consent was obtained from all subjects...
2016: Folia Phoniatrica et Logopaedica
Mansi Pankaj Jani, Geeta Bharat Gore
BACKGROUND: Motor neurone disease also commonly known as Amyotrophic Lateral Sclerosis (ALS) is a neurological condition which affects various motor functions of the body. Dysphagia (disordered swallowing) is commonly seen in patients with ALS having bulbar symptoms. OBJECTIVES: Research reveals presence of dysphagia in patients with ALS at various stages of swallowing using instrumental assessment. However, very few studies have been done focussing on clinical profiling of swallowing in these patients...
June 27, 2016: NeuroRehabilitation
Javier Riancho, Pablo Lozano-Cuesta, Ana Santurtún, Pascual Sánchez-Juan, José Manuel López-Vega, José Berciano, José Miguel Polo
BACKGROUND: In the last years different studies have reported an increase of amyotrophic lateral sclerosis (ALS) incidence, highlighting the role of the environment in this disease. This prompted us to review ALS cases diagnosed at our hospital in the last decade and to compare them with a previous ALS series reported in our region 30 years ago. METHODS: We reviewed those ALS cases diagnosed at our centre between 2004 and 2013. Subsequently, we compared them with the previous series regarding clinical and epidemiological features...
2016: Neuro-degenerative Diseases
Nazan Simsek Erdem, Kamil Karaali, Ali Ünal, Ferah Kızılay, Candan Öğüş, Hilmi Uysal
The aim of the study is to determine the association between respiratory swallow patterns in amyotrophic lateral sclerosis (ALS) patients. Furthermore, it aims to clarify the role of the dysphagia limit in defining the relationship between swallowing disorders and respiratory disorders. Functional rating scales were used to describe swallowing and respiratory function. Swallowing was observed using the dysphagia limit. Dysphagia limit is the volume at which a second or more swallows are required to swallow the whole bolus...
May 5, 2016: Acta Neurologica Belgica
Daichi Yokoi, Naoki Atsuta, Hazuki Watanabe, Ryoichi Nakamura, Akihiro Hirakawa, Mizuki Ito, Hirohisa Watanabe, Masahisa Katsuno, Yuishin Izumi, Mitsuya Morita, Akira Taniguchi, Masaya Oda, Koji Abe, Kouichi Mizoguchi, Osamu Kano, Satoshi Kuwabara, Ryuji Kaji, Gen Sobue
The clinical courses of sporadic amyotrophic lateral sclerosis (ALS) show extensive variability. Our objective was to elucidate how age of onset influences the progression of regional symptoms and functional losses in sporadic ALS. We included 648 patients with sporadic ALS from a multicenter prospective ALS cohort. We investigated the distribution of initial symptoms and analyzed the time from onset to events affecting activities of daily living (ADL) as well as the longitudinal changes in each regional functional rating score among four groups with different ages of onset...
June 2016: Journal of Neurology
Timothy Benstead, Caitlin Jackson-Tarlton, Desmond Leddin
BACKGROUND: Amyotrophic lateral sclerosis (ALS) is a rapidly progressing degenerative motor neuron disease that results in significant muscle weakness. Defects in energy metabolism and difficulties in swallowing eventually lead to a reduction in body mass. Weight loss exacerbates symptoms and serves as an independent negative prognostic factor. Percutaneous endoscopic gastrostomy (PEG) is often inserted in patients with ALS to either supplement or replace oral feeding. However, the criteria for PEG placement and timing of insertion are important clinical decisions that have not been fully studied...
April 4, 2016: Canadian Journal of Neurological Sciences. le Journal Canadien des Sciences Neurologiques
Lauren Tabor, Joy Gaziano, Stephanie Watts, Raele Robison, Emily K Plowman
Although it is known that dysphagia contributes to significant malnutrition, pneumonia, and mortality in amyotrophic lateral sclerosis (ALS), it remains unclear how swallowing impairment impacts quality of life in this vulnerable patient population. The aim of the current study was to (1) delineate swallow-related quality of life (SR-QOL) profiles in individuals with ALS and (2) evaluate relationships between SR-QOL, degree of swallowing impairment, and ALS global disease progression. Eighty-one ALS patients underwent a standardized videofluoroscopic swallow study and completed the swallowing quality of life (SWAL-QOL) instrument and ALS functional rating scale-revised (ALSFRS-R)...
June 2016: Dysphagia
J G Weikamp, D A X Schinagl, C C P Verstappen, H J Schelhaas, B J M de Swart, J G Kalf
OBJECTIVES: Botulinum neurotoxin (BoNT) injections in the salivary glands and radiotherapy (RT) on these glands are commonly used to alleviate severe drooling in patients with amyotrophic lateral sclerosis (ALS). This study compares BoNT type A with RT based on patient-rated evaluations. MATERIALS & METHODS: A prospective randomized controlled pilot study to compare RT (n = 10; on the parotid and the posterior part of the submandibular glands) with BoNT-A treatment (n = 10; in the parotid glands only, because of the risk of increasing oropharyngeal weakness) in patients with ALS...
September 2016: Acta Neurologica Scandinavica
Emily K Plowman, Stephanie A Watts, Raele Robison, Lauren Tabor, Charles Dion, Joy Gaziano, Tuan Vu, Clifton Gooch
Dysphagia and aspiration are prevalent in amyotrophic lateral sclerosis (ALS) and contribute to malnutrition, aspiration pneumonia, and death. Early detection of at risk individuals is critical to ensure maintenance of safe oral intake and optimal pulmonary function. We therefore aimed to determine the discriminant ability of voluntary cough airflow measures in detecting penetration/aspiration status in ALS patients. Seventy individuals with ALS (El-Escorial criteria) completed voluntary cough spirometry testing and underwent a standardized videofluoroscopic swallowing evaluation (VFSE)...
June 2016: Dysphagia
Carolin Dahlke, Darius Saberi, Bastian Ott, Beate Brand-Saberi, Thomas Schmitt-John, Carsten Theiss
BACKGROUND: Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disorder of the upper and lower motor neurons, characterized by rapid progressive weakness, muscle atrophy, dysarthria, dysphagia, and dyspnea. Whereas the exact cause of ALS remains uncertain, the wobbler mouse (phenotype WR; genotype wr/wr) equally develops a progressive degeneration of motor neurons in the spinal cord and motor cortex with striking similarities to sporadic human ALS, suggesting the possibility of a common pathway to cell death...
2015: Journal of Neuroinflammation
E K Plowman, L C Tabor, R Robison, J Gaziano, C Dion, S A Watts, T Vu, C Gooch
BACKGROUND: Oropharyngeal dysphagia is prevalent in individuals with amyotrophic lateral sclerosis (ALS) leading to malnutrition, aspiration pneumonia, and death. These factors necessitate early detection of at-risk patients to prolong maintenance of safe oral intake and pulmonary function. This study aimed to evaluate the discriminant ability of the Eating Assessment Tool (EAT-10) to identify ALS patients with unsafe airway protection during swallowing. METHODS: Seventy ALS patients completed the EAT-10 survey and underwent a standardized videofluoroscopic evaluation of swallowing...
January 2016: Neurogastroenterology and Motility: the Official Journal of the European Gastrointestinal Motility Society
Masahiro Nakamori, Naohisa Hosomi, Sachiko Takaki, Masaya Oda, Aya Hiraoka, Mineka Yoshikawa, Hayato Matsushima, Kazuhide Ochi, Kazuhiro Tsuga, Hirofumi Maruyama, Yuishin Izumi, Masayasu Matsumoto
OBJECTIVE: Dysphagia is a critical issue in amyotrophic lateral sclerosis (ALS) patients. An evaluation of swallowing function is important for assessing the risk of aspiration. We investigated the validity of tongue sonography compared with videofluoroscopic examination for ALS patients. METHODS: We investigated 18 ALS patients. Nine subjects underwent repeated investigations. All of the subjects underwent tongue sonography and videofluoroscopic examination. Additionally, tongue sonography was evaluated in 18 age- and sex-matched healthy volunteers...
February 2016: Clinical Neurophysiology: Official Journal of the International Federation of Clinical Neurophysiology
Daniel A de Luis, Olatz Izaola, Beatriz de la Fuente, Paloma Muñoz-Calero, Angeles Franco-Lopez
INTRODUCTION: neurodegenerative diseases cause changes in the level of consciousness or swallowing mechanisms that often necessitate a specialized nutritional support. OBJECTIVE: review the risk of malnutrition and its treatment in patients with cerebral vascular disease, Parkinson's disease, dementia and amyotrophic lateral sclerosis. DEVELOPMENT: degenerative neurological diseases are one of the main indications for nutritional support in our country...
2015: Nutrición Hospitalaria: Organo Oficial de la Sociedad Española de Nutrición Parenteral y Enteral
Hiroshige Taniguchi, Hideaki Nakayama, Kazuhiro Hori, Masatoyo Nishizawa, Makoto Inoue, Takayoshi Shimohata
The prevalence of esophageal involvement and its impact on clinical manifestations in patients with multiple system atrophy (MSA) remains unknown. We recruited 16 consecutive patients with dysphagia associated with MSA (MSA group) and 16 consecutive patients with dysphagia associated with amyotrophic lateral sclerosis (ALS group). We assessed the presence or absence of food stagnation within the esophagus using videofluorography. Food stagnation within the esophagus was observed in 16 patients (100 %; 7 severe, 9 mild) in the MSA group and in 4 patients (25 %; 4 mild) in the ALS group (P < 0...
December 2015: Dysphagia
Muhammad K Rafiq, Ellen Lee, Michael Bradburn, Christopher J McDermott, Pamela J Shaw
Patients with ALS may have insufficientenergy substrates, due to dysphagia and hypermetabolism, which adversely affects the prognosis. Hyperlipidaemia has been reported to be associated with ALS and to represent a significant prognostic factor for survival in ALS. The aim of this study was to examine the prevalence of dyslipidaemia among a cohort of patients with ALS and how the lipid profile of patients with ALS influenced the prognosis. This was a prospective observational cohort study comprising 512 ALS patients, recruited for the TRO19622 (Olesoxime) investigational medicinal product trial...
2015: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
Ayako Wada, Michiyuki Kawakami, Meigen Liu, Eri Otaka, Atsuko Nishimura, Fumio Liu, Tomoyoshi Otsuka
Dysphagia is one of the most critical problems in patients with progressive neuromuscular diseases. However, clinically useful and practical scales to evaluate dysphagia are limited. Therefore, the aim of this study was to develop such a scale. An 8-stage Neuromuscular Disease Swallowing Status Scale (NdSSS) was developed and tested for its inter- and intrarater reliabilities, concurrent validity, and responsiveness. The NdSSS was used to evaluate 134 patients with Duchenne muscular dystrophy (DMD) and 84 patients with amyotrophic lateral sclerosis (ALS)...
October 2015: Journal of Neurology
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