Article
Clinical Neurology
Susanne Spittel, Andre Maier, Dagmar Kettemann, Bertram Walter, Birgit Koch, Kerstin Krause, Jenny Norden, Christoph Muench, Thomas Meyer
Summary: The study found that although NIV represents the standard of care, its utilization rate was low, and TIV was mainly started without preceding NIV. The survival benefit observed in NIV patients was equal to that reported in controlled trials, but the prognosis with TIV varies greatly.
EUROPEAN JOURNAL OF NEUROLOGY
(2021)
Review
Health Care Sciences & Services
Sebastiano Mercadante, Lou'i Al-Husinat
Summary: Amyotrophic lateral sclerosis (ALS) is an incurable neurodegenerative disease. Palliative care principles are crucial in ALS care, and a multidisciplinary medical intervention is essential. Early initiation and advance care planning improve quality of life and prognosis. Cognitive disturbances, psychological distress, pain, sialorrhoea, nutrition, and ventilatory support require intensive supportive care.
JOURNAL OF PAIN AND SYMPTOM MANAGEMENT
(2023)
Article
Biochemistry & Molecular Biology
Yuki Nakayama, Toshio Shimizu, Chiharu Matsuda, Michiko Haraguchi, Kentaro Hayashi, Kota Bokuda, Masahiro Nagao, Akihiro Kawata, Kazushi Takahashi
Summary: The incidence of weight gain in ALS patients who underwent tracheostomy and invasive ventilation (TIV) was investigated. The study found that weight gain and hypoalbuminemia during TIV were associated with the disease stage and may be attributed to the neurodegenerative processes that are peculiar to ALS.
Article
Medicine, General & Internal
Eleonora Volpato, Michele Vitacca, Luciana Ptacinsky, Agata Lax, Salvatore D'Ascenzo, Enrica Bertella, Mara Paneroni, Silvia Grilli, Paolo Banfi
Summary: In ALS patients, home-based adaptation to NIV is as effective as outpatient adaptation in terms of PaCO2, acceptance, and adherence, highlighting the need for further studies on the role of the environment in NIV adherence.
JOURNAL OF CLINICAL MEDICINE
(2022)
Article
Medicine, General & Internal
Jovana Maskovic, Aleksandra Ilic, Vladimir Zugic, Zorica Stevic, Mihailo I. Stjepanovic
Summary: The study evaluates the significance of pulmonary function tests in predicting mortality and defining indications for noninvasive mechanical ventilation (NIMV) in patients with ALS. Results show that patients with spinal form of ALS have higher forced vital capacity (FVC) and forced expiratory volume in 1 s (FEV1) compared to those with bulbar form. Patients with bulbar form have a higher risk of death.
ARCHIVES OF MEDICAL SCIENCE
(2023)
Article
Clinical Neurology
Laura Salzmann, Bernd Alt-Epping, Alfred Simon
Summary: This survey conducted in Germany among neurologists and palliative care practitioners showed that the use of palliative sedation in ALS is widely accepted. There is more concern regarding the use of palliative sedation for psychological symptoms compared to physical symptoms. Refusing artificial nutrition does not affect the acceptance of palliative sedation. Doctors with specialist training in palliative care are more likely to support the use of palliative sedation in ALS.
Article
Rehabilitation
Kristina Gottberg, Charlotte Ytterberg, Petter Sandstedt, Sverker Johansson, Marie Kierkegaard
Summary: Through semi-structured interviews, this study identified challenges faced by next of kin in caring for patients with amyotrophic lateral sclerosis using invasive ventilation via tracheostomy, including prolonging life, coping with daily pressures, and conflicting roles. The importance of involving next of kin throughout the care process and developing specific support interventions to address their needs were emphasized as crucial outcomes of the research.
DISABILITY AND REHABILITATION
(2021)
Article
Clinical Neurology
Kellen H. Quigg, Matthew W. Wilson, Philip J. Choi
Summary: This study aims to investigate the clinical utility of transcutaneous CO2 (TCO2) as a screening tool to identify ALS patients who would benefit from inpatient initiation and titration of NIV. Retrospective analysis of 8 ALS patients admitted urgently for NIV initiation showed that TCO2 measurements can effectively identify respiratory dysfunction and help in timely treatment initiation.
Article
Biochemical Research Methods
Andreia S. Martins, Marta Gromicho, Susana Pinto, Mamede de Carvalho, Sara C. Madeira
Summary: This study proposes using itemset mining and sequential pattern mining to predict the need for non-invasive ventilation treatment in patients with amyotrophic lateral sclerosis. By analyzing static and longitudinal data, disease progression patterns and predictive model features are identified, providing insights for disease management and treatment.
IEEE-ACM TRANSACTIONS ON COMPUTATIONAL BIOLOGY AND BIOINFORMATICS
(2022)
Article
Clinical Neurology
Ryo Morishima, Toshio Shimizu, Hideki Kimura, Kota Bokuda, Takako Saotome, Yuki Nakayama, Kazushi Takahashi
Summary: This study aimed to investigate the usage and dosage of opioids in relieving suffering in patients with amyotrophic lateral sclerosis (ALS). The results showed that patients who used non-invasive ventilation required a significantly higher dose of opioids compared to those without ventilation. Therefore, a multifaceted evaluation and care approach may be necessary for symptom control.
ACTA NEUROLOGICA BELGICA
(2023)
Article
Clinical Neurology
Philippe Corcia, Pascal Lejeune, Patrick Vourc'h, Stephane Beltran, Anne-Sophie Piegay, Helene Blasco, Vincent Meininger
Summary: This study characterized the prototypical phenotype of patients with amyotrophic lateral sclerosis (ALS) associated with PFN1 mutations and identified clinical indications for testing mutations in this gene. The main clinical findings for familial ALS linked to PFN1 were identified as pedigrees with over five cases, an onset age around 50 years, onset in the lower limbs, and the absence of cognitive impairment. The similarities with other ALS mutations prompt a review of ALS classifications based on both phenotype and genotype.
EUROPEAN JOURNAL OF NEUROLOGY
(2023)
Review
Health Care Sciences & Services
Caroline Barry, Emma Larner, Helen Copsey, Matthew Smith, Guy Peryer
Summary: The review summarises recent developments in the use of noninvasive ventilation for people with amyotrophic lateral sclerosis, indicating a modest survival benefit but highlighting the need for further evidence on timing of initiation and impact on cognitive function. Multidisciplinary teams play a key role in supporting patients to consider the potential benefits of NIV.
CURRENT OPINION IN SUPPORTIVE AND PALLIATIVE CARE
(2021)
Article
Geriatrics & Gerontology
Umberto Manera, Maurizio Grassano, Enrico Matteoni, Alessandro Bombaci, Rosario Vasta, Francesca Palumbo, Maria Claudia Torrieri, Paolo Cugnasco, Cristina Moglia, Antonio Canosa, Adriano Chio, Andrea Calvo
Summary: Respiratory failure is a common cause of death in ALS patients, and its occurrence varies among patients with different phenotypic features. Early predictors of respiratory failure are important to initiate NIV. In this study, serum chloride values at diagnosis were evaluated as prognostic biomarkers for overall survival and NIV adaptation in ALS patients. Analysis showed a significant correlation between serum chloride and various clinical features, and serum chloride value at diagnosis was found to significantly influence survival and time to NIV start. Serum chloride analyzed at diagnosis can serve as a low-cost marker for predicting respiratory decompensation in ALS patients.
FRONTIERS IN AGING NEUROSCIENCE
(2023)
Article
Clinical Neurology
Miguel Oliveira Santos, Marta Gromicho, Susana Pinto, Michael Swash, Mamede de Carvalho
Summary: This study analyzed a cohort of 74 amyotrophic lateral sclerosis (ALS) patients with dysphagia under continuous non-invasive ventilation treatment. The results showed that gastrostomy was not associated with survival rate for patients with mild to moderate dysphagia. However, disease duration and progression rate were predictors of survival for patients with normal swallowing ability.
JOURNAL OF THE NEUROLOGICAL SCIENCES
(2022)
Article
Clinical Neurology
Miguel Oliveira Santos, Marta Gromicho, Susana Pinto, Michael Swash, Mamede de Carvalho
Summary: For ALS patients dependent on continuous NIV without feeding tube, the survival rate is similar between those with different levels of swallowing function. Disease duration and progression rate are important factors to consider when discussing gastrostomy.
JOURNAL OF THE NEUROLOGICAL SCIENCES
(2022)
Article
Clinical Neurology
Giorgia Querin, Timothee Lenglet, Rabab Debs, Tanya Stojkovic, Anthony Behin, Francois Salachas, Nadine Le Forestier, Maria del Mar Amador, Lucette Lacomblez, Vincent Meininger, Gaelle Bruneteau, Pascal Laforet, Sophie Blancho, Veronique Marchand-Pauvert, Peter Bede, Jean-Yves Hogrel, Pierre-Francois Pradat
CLINICAL NEUROPHYSIOLOGY
(2018)
Article
Respiratory System
Raquel Guimaraes-Costa, Thomas Similowski, Isabelle Rivals, Capucine Morelot-Panzini, Marie-Cecile Nierat, Mai Thao Bui, David Akbar, Christian Straus, Norma Beatriz Romero, Patrick Pierre Michel, Fabrice Menegaux, Francois Salachas, Jesus Gonzalez-Bermejo, Gaelle Bruneteau, Maria Del Mar Amador, Jean Christophe Antoine, Marie Christine Arne-Bes, Valerie Attali, Katell Beauvais, Veronique Brunaud Danel, Jean-Philippe Camdessanche, William Camu, Laurence Carluer, Julien Cassereau, Maud Chapart, Bruno Chenuel, Nathalie Chereau, Pascal Cintas, Pierre Clavelou, Philippe Corda, Andre Cornette, Philippe Couratier, Isabelle Court-Fortune, Antoine Cuvelier, Christophe Delclaux, Claude Desnuelle, Sophie Di Maria, Catherine Fargeot, Brigitte Fauroux, Armelle Finet-Monnier, Marie Celine Fleury, Carmen Gaillat, Marjolaine Georges, Maurice Giroud, Annick Greil, Muriel Guerrier, Nathalie Guy, Didier Hannequin, Valerie Juillien, Raul Juntas-Morales, Romain Kessler, Yvan Kolev, Julien Lagarde, Geraldine Lautrette, Pierre Le Cam, Nadine LeForestier, Gwendal Lemasson, Thimothee Lenglet, Pascal Magro, Anne Mallart, Olivier Martinaud, Vincent Meininger, Nicole Meslier, Pierre Moirot, Capucine Morelot-Panzini, Yann Nadjar, Guillaume Nicolas, Myriam Niel-Duriez, Severine Noullet, Nicolas Pageot, Thierry Perez, Christophe Perrin, Odile Pillet, Sophie Pittion, Jean Pouget, Pierre-Franos Pradat, Helene Prigent, Claudio Rabec, Jean-Damien Ricard, Norma Romero, Catherine Royer, Barbara Schaup, Kamila Sedkaoui, Marie-Helene Soriani, Marie-Laure Tanguy, Christine Tranchant, Nadia Vandenberghe, Annie Vershueren, Fausto Viader, Anne-Cecile Wienalek-Bachelet
EUROPEAN RESPIRATORY JOURNAL
(2019)
Article
Neuroimaging
Giorgia Querin, Mohamed-Mounir El Mendili, Timothee Lenglet, Anthony Behin, Tanya Stojkovic, Francois Salachas, David Devos, Nadine Le Forestier, Maria del Mar Amador, Rabab Debs, Lucette Lacomblez, Vincent Meninger, Gaelle Bruneteau, Julien Cohen-Adad, Stephane Lehericy, Pascal Laforet, Sophie Blancho, Habib Benali, Martin Catala, Menghan Li, Veronique Marchand-Pauvert, Jean-Yves Hogrel, Peter Bede, Pierre-Francois Pradat
NEUROIMAGE-CLINICAL
(2019)
Article
Critical Care Medicine
Julien Mayaux, Jerome Lambert, Capucine Morelot-Panzini, Jesus Gonzalez-Bermejo, Julie Delemazure, Claudia Llontop, Gaelle Bruneteau, Francois Salachas, Martin Dres, Alexandre Demoule, Thomas Similowski
JOURNAL OF CRITICAL CARE
(2019)
Article
Neurosciences
Timothee Lenglet, Jonathan Mirault, Marie Veyrat-Masson, Aurelie Funkiewiez, Maria del Mar Amador, Gaelle Bruneteau, Nadine Le Forestier, Pierre-Francois Pradat, Francois Salachasl, Yannick Vacher, Lucette Lacomblez, Jean Lorenceau
FRONTIERS IN NEUROSCIENCE
(2019)
Letter
Critical Care Medicine
Raquel Guimaraes-Costa, Marie-Cecile Nierat, Isabelle Rivals, Capucine Morelot-Panzini, Norma Beatriz Romero, Fabrice Menegaux, Francois Salachas, Jesus Gonzalez-Bermejo, Thomas Similowski, Gaelle Bruneteau
AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE
(2019)
Letter
Respiratory System
Mathilde Oranger, Jesus Gonzalez-Bermejo, Philippine Dacosta-Noble, Claudia Llontop, Antoine Guerder, Valery Trosini-Desert, Morgane Faure, Mathieu Raux, Maxens Decavele, Alexandre Demoule, Capucine Morelot-Panzini, Thomas Similowski
EUROPEAN RESPIRATORY JOURNAL
(2020)
Article
Clinical Neurology
M. Mongin, R. Debs, N. Le Forestier, T. Lenglet, F. Salachas, P-F Pradat, M. D. M. Amador, G. Bruneteau, B. Degos
REVUE NEUROLOGIQUE
(2020)
Article
Clinical Neurology
C. Delorme, O. Paccoud, A. Kas, A. Hesters, S. Bombois, P. Shambrook, A. Boullet, D. Doukhi, L. Le Guennec, N. Godefroy, R. Maatoug, P. Fossati, B. Millet, V. Navarro, G. Bruneteau, S. Demeret, V. Pourcher
EUROPEAN JOURNAL OF NEUROLOGY
(2020)
Article
Multidisciplinary Sciences
Adele Hesters, Maria del Mar Amador, Rabab Debs, Nadine Le Forestier, Timothee Lenglet, Pierre-Francois Pradat, Francois Salachas, Morgane Faure, Maria-Alejandra Galarza Jimenez, Jesus Gonzalez-Bermejo, Capucine Morelot, Gaelle Bruneteau
SCIENTIFIC REPORTS
(2020)
Article
Clinical Neurology
Giorgia Querin, Timothee Lenglet, Rabab Debs, Tanya Stojkovic, Anthony Behin, Francois Salachas, Nadine Le Forestier, Maria Del Mar Amador, Gaelle Bruneteau, Pascal Laforet, Sophie Blancho, Veronique Marchand-Pauvert, Peter Bede, Jean-Yves Hogrel, Pierre-Francois Pradat
Summary: Quantitative strength testing, SMAFRS, and MUNIX are effective in capturing disease progression in adult SMA patients. Composite multimodal scores increase predictive value and may reduce sample size requirements in clinical trials.
JOURNAL OF NEUROLOGY
(2021)
Review
Medicine, General & Internal
Jean-Michel Arnal, Mathilde Oranger, Jesus Gonzalez-Bermejo
Summary: Non-invasive ventilation (NIV) is commonly used for patients with nocturnal hypoventilation caused by chronic respiratory failure. Monitoring NIV is important to optimize ventilator settings and detect common problems. This review discusses the accuracy and limitations of ventilator data, as well as the use of nocturnal oximetry and capnography. Polygraphy and polysomnography are recommended in specific cases to characterize upper airway obstruction. Telemonitoring of the ventilator is also a useful tool. A basic monitoring package is recommended for all patients, complemented by advanced monitoring for specific cases.
JOURNAL OF CLINICAL MEDICINE
(2023)
Article
Respiratory System
Leo Grassion, Jesus Gonzalez-Bermejo, Isabelle Arnulf, Maxime Patout