Revue Neurologique, ISSN 0035-3787, 04/2018, Volume 174, pp. S53 - S53
Journal Article
Soins, ISSN 0038-0814, 06/2008, Volume 53, Issue 726, pp. 44 - 46
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Soins; la revue de reference infirmiere, ISSN 0038-0814, 06/2008, Issue 726, p. 44
Patient Rights - legislation & jurisprudence | Attitude to Health | Physician-Patient Relations - ethics | Humans | Amyotrophic Lateral Sclerosis - diagnosis | Amyotrophic Lateral Sclerosis - psychology | Physician's Role - psychology | Patient Rights - ethics | Self Concept | Defense Mechanisms | France | Truth Disclosure - ethics | Adaptation, Psychological | Communication
Journal Article
Jusqu’à la mort accompagner la vie, ISSN 0768-6625, 2017, Volume 128, Issue 1, pp. 41 - 52
Journal Article
La Presse Medicale, ISSN 0755-4982, 06/2012, Volume 41, Issue 6, pp. 560 - 574
and result from secondary to degeneration of brainstem motor neurons. Recent knowledge argues in favor of the associated . Though muscle atrophy, a paradoxical...
Journal Article
Jusqu’à la mort accompagner la vie, ISSN 0768-6625, 2017, Volume N° 128, Issue 1, p. 41
Journal Article
Medicine Studies, ISSN 1876-4533, 12/2011, Volume 3, Issue 2, pp. 71 - 81
Because our actions change, our responsibility is modified; because our responsibility is modified, we need to question the ethics of the action. Our action is...
Humanities, general | History of Medicine | Announcement of diagnosis | Theory of Medicine/Bioethics | Amyotrophic lateral sclerosis | Anthropological phenomenology | Philosophy of Medicine | Philosophy | Medical law | Paralysis | Analysis
Humanities, general | History of Medicine | Announcement of diagnosis | Theory of Medicine/Bioethics | Amyotrophic lateral sclerosis | Anthropological phenomenology | Philosophy of Medicine | Philosophy | Medical law | Paralysis | Analysis
Journal Article
Journal of Medical Genetics, ISSN 0022-2593, 08/2010, Volume 47, Issue 8, pp. 554 - 560
BackgroundMutations in SOD1, ANG, VAPB, TARDBP and FUS genes have been identified in amyotrophic lateral sclerosis (ALS).MethodsThe relative contributions of...
Superoxide Dismutase - genetics | Age Distribution | Genetic Association Studies | Amyotrophic Lateral Sclerosis - genetics | Humans | Middle Aged | Ribonuclease, Pancreatic - genetics | Vesicular Transport Proteins - genetics | RNA-Binding Protein FUS - genetics | Male | Longevity | DNA-Binding Proteins - genetics | Mutation - genetics | Age of Onset | Aged, 80 and over | Adult | Family | Female | Aged | Amyotrophic Lateral Sclerosis - enzymology | Amyotrophic Lateral Sclerosis - epidemiology | Amyotrophic lateral sclerosis | Development and progression | Genetic aspects | Research | Gene mutations | Identification and classification
Superoxide Dismutase - genetics | Age Distribution | Genetic Association Studies | Amyotrophic Lateral Sclerosis - genetics | Humans | Middle Aged | Ribonuclease, Pancreatic - genetics | Vesicular Transport Proteins - genetics | RNA-Binding Protein FUS - genetics | Male | Longevity | DNA-Binding Proteins - genetics | Mutation - genetics | Age of Onset | Aged, 80 and over | Adult | Family | Female | Aged | Amyotrophic Lateral Sclerosis - enzymology | Amyotrophic Lateral Sclerosis - epidemiology | Amyotrophic lateral sclerosis | Development and progression | Genetic aspects | Research | Gene mutations | Identification and classification
Journal Article
Journal of Medical Genetics, ISSN 0022-2593, 04/2012, Volume 49, Issue 4, pp. 258 - 263
BackgroundExpanded GGGGCC hexanucleotide repeats in the promoter of the C9ORF72 gene have recently been identified in frontotemporal dementia (FTD),...
AMYOTROPHIC-LATERAL-SCLEROSIS | FRONTOTEMPORAL LOBAR DEGENERATION | FUS | HEXANUCLEOTIDE REPEAT | EXPANSION | GENETICS & HEREDITY | Superoxide Dismutase - genetics | Age Factors | Genetic Association Studies | Amyotrophic Lateral Sclerosis - genetics | Humans | Middle Aged | Amyotrophic Lateral Sclerosis - diagnosis | RNA-Binding Protein FUS - genetics | Male | DNA-Binding Proteins - genetics | Proteins - genetics | Young Adult | DNA Repeat Expansion | Phenotype | Aged, 80 and over | Adult | Female | Aged | C9orf72 Protein | Mutation | Superoxide Dismutase-1
AMYOTROPHIC-LATERAL-SCLEROSIS | FRONTOTEMPORAL LOBAR DEGENERATION | FUS | HEXANUCLEOTIDE REPEAT | EXPANSION | GENETICS & HEREDITY | Superoxide Dismutase - genetics | Age Factors | Genetic Association Studies | Amyotrophic Lateral Sclerosis - genetics | Humans | Middle Aged | Amyotrophic Lateral Sclerosis - diagnosis | RNA-Binding Protein FUS - genetics | Male | DNA-Binding Proteins - genetics | Proteins - genetics | Young Adult | DNA Repeat Expansion | Phenotype | Aged, 80 and over | Adult | Female | Aged | C9orf72 Protein | Mutation | Superoxide Dismutase-1
Journal Article
Lancet Neurology, The, ISSN 1474-4422, 2016, Volume 15, Issue 12, pp. 1217 - 1227
Summary Background Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disorder associated with respiratory muscle weakness and respiratory failure....
Neurology | SURVIVAL | STIMULATION | PHRENIC-NERVE | MECHANICAL VENTILATION | ALS | ELECTRODES | MUSCLE | COHORT | EXPERIENCE | NONINVASIVE VENTILATION | CLINICAL NEUROLOGY | Double-Blind Method | Amyotrophic Lateral Sclerosis - therapy | Humans | Middle Aged | Male | Laparoscopy | Respiration Disorders | Respiration, Artificial | Amyotrophic Lateral Sclerosis - complications | Early Termination of Clinical Trials | Electric Stimulation Therapy - methods | Respiratory Insufficiency - prevention & control | Electric Stimulation Therapy - adverse effects | Phrenic Nerve | Female | Aged | Diaphragm - innervation | Diaphragm - physiopathology | Amyotrophic lateral sclerosis | Care and treatment | Nervous system diseases | Neurophysiology | Palliative care | Ventilation | Ostomy | Surgery | Mortality
Neurology | SURVIVAL | STIMULATION | PHRENIC-NERVE | MECHANICAL VENTILATION | ALS | ELECTRODES | MUSCLE | COHORT | EXPERIENCE | NONINVASIVE VENTILATION | CLINICAL NEUROLOGY | Double-Blind Method | Amyotrophic Lateral Sclerosis - therapy | Humans | Middle Aged | Male | Laparoscopy | Respiration Disorders | Respiration, Artificial | Amyotrophic Lateral Sclerosis - complications | Early Termination of Clinical Trials | Electric Stimulation Therapy - methods | Respiratory Insufficiency - prevention & control | Electric Stimulation Therapy - adverse effects | Phrenic Nerve | Female | Aged | Diaphragm - innervation | Diaphragm - physiopathology | Amyotrophic lateral sclerosis | Care and treatment | Nervous system diseases | Neurophysiology | Palliative care | Ventilation | Ostomy | Surgery | Mortality
Journal Article
PLoS ONE, ISSN 1932-6203, 04/2016, Volume 11, Issue 4, p. e0152439
Purpose The mechanisms underlying the topography of motor deficits in spinal muscular atrophy (SMA) remain unknown. We investigated the profile of spinal cord...
KUGELBERG-WELANDER DISEASE | MULTIPLE-SCLEROSIS | MULTIDISCIPLINARY SCIENCES | CONSTRUCTION | ALS | MOTOR-NEURON | MUSCULAR-ATROPHY | NATURAL-HISTORY | NEUROMUSCULAR-JUNCTIONS | MOUSE MODELS | DEGENERATION | Muscular Atrophy, Spinal - metabolism | Spinal Cord - metabolism | Cervical Cord - pathology | Humans | Male | Survival of Motor Neuron 1 Protein - metabolism | Muscular Atrophy, Spinal - pathology | Case-Control Studies | Magnetic Resonance Imaging | Spinal Cord - pathology | Cervical Cord - metabolism | Survival of Motor Neuron 1 Protein - genetics | Adult | Female | Muscle Strength - physiology | Motor neurons | Physiological aspects | Development and progression | Genetic aspects | Research | Gene expression | Risk factors | Spinal muscular atrophy | Atrophy | Spinal cord | Vertebrae | Magnetic resonance imaging | Correlation analysis | Topography | SMN protein | Muscles | Patients | Skeletal muscle | Life Sciences | Neurons and Cognition
KUGELBERG-WELANDER DISEASE | MULTIPLE-SCLEROSIS | MULTIDISCIPLINARY SCIENCES | CONSTRUCTION | ALS | MOTOR-NEURON | MUSCULAR-ATROPHY | NATURAL-HISTORY | NEUROMUSCULAR-JUNCTIONS | MOUSE MODELS | DEGENERATION | Muscular Atrophy, Spinal - metabolism | Spinal Cord - metabolism | Cervical Cord - pathology | Humans | Male | Survival of Motor Neuron 1 Protein - metabolism | Muscular Atrophy, Spinal - pathology | Case-Control Studies | Magnetic Resonance Imaging | Spinal Cord - pathology | Cervical Cord - metabolism | Survival of Motor Neuron 1 Protein - genetics | Adult | Female | Muscle Strength - physiology | Motor neurons | Physiological aspects | Development and progression | Genetic aspects | Research | Gene expression | Risk factors | Spinal muscular atrophy | Atrophy | Spinal cord | Vertebrae | Magnetic resonance imaging | Correlation analysis | Topography | SMN protein | Muscles | Patients | Skeletal muscle | Life Sciences | Neurons and Cognition
Journal Article
Psychologie et NeuroPsychiatrie du Vieillissement, ISSN 1760-1703, 06/2006, Volume 4, Issue 2, pp. 109 - 119
Journal Article
Journal of Neurology, ISSN 0340-5354, 9/2018, Volume 265, Issue 9, pp. 2125 - 2136
Extrapyramidal deficits are poorly characterised in amyotrophic lateral sclerosis (ALS) despite their contribution to functional disability, increased fall...
Postural instability | Neurology | Neurosciences | Basal ganglia | Medicine & Public Health | Magnetic resonance imaging | Gait impairment | Amyotrophic lateral sclerosis | Neuroradiology | FALLS | WALKING | GAIT | INVOLVEMENT | AMYOTROPHIC-LATERAL-SCLEROSIS | ANTICIPATORY POSTURAL ADJUSTMENTS | CLINICAL NEUROLOGY | SCALE | DOPAMINERGIC DEFICIT | PARKINSONS-DISEASE | Complications and side effects | Care and treatment | Usage | Diagnosis | Research | Risk factors | Gait disorders | Cerebellum | Neuroimaging | Medical imaging | Gait | Computational neuroscience | Neurodegeneration | Brain stem | Cognitive ability | Ganglia | Posture | Life Sciences | Neurons and Cognition
Postural instability | Neurology | Neurosciences | Basal ganglia | Medicine & Public Health | Magnetic resonance imaging | Gait impairment | Amyotrophic lateral sclerosis | Neuroradiology | FALLS | WALKING | GAIT | INVOLVEMENT | AMYOTROPHIC-LATERAL-SCLEROSIS | ANTICIPATORY POSTURAL ADJUSTMENTS | CLINICAL NEUROLOGY | SCALE | DOPAMINERGIC DEFICIT | PARKINSONS-DISEASE | Complications and side effects | Care and treatment | Usage | Diagnosis | Research | Risk factors | Gait disorders | Cerebellum | Neuroimaging | Medical imaging | Gait | Computational neuroscience | Neurodegeneration | Brain stem | Cognitive ability | Ganglia | Posture | Life Sciences | Neurons and Cognition
Journal Article
International Journal of Radiation Oncology, Biology, Physics, ISSN 0360-3016, 2014, Volume 88, Issue 3, pp. 589 - 595
Purpose This study aimed to evaluate the efficiency and the tolerance of radiation therapy (RT) on salivary glands in a large series of amyotrophic lateral...
Radiology | Hematology, Oncology and Palliative Medicine | MANAGEMENT | SIALORRHEA | ONCOLOGY | DISEASE | ALS | RADIOTHERAPY | CANCER | RADIOLOGY, NUCLEAR MEDICINE & MEDICAL IMAGING | BOTULINUM-TOXIN | Radiotherapy Dosage | Prospective Studies | Humans | Middle Aged | Male | Photons - therapeutic use | Amyotrophic Lateral Sclerosis - complications | Submandibular Gland | Parotid Gland - radiation effects | Sialorrhea - etiology | Statistics, Nonparametric | Aged, 80 and over | Adult | Female | Remission Induction - methods | Aged | Sialorrhea - radiotherapy | Sects | Medical research | Nervous system diseases | Care and treatment | Nuclear radiation | Medicine, Experimental | Amyotrophic lateral sclerosis | Radiotherapy | Index Medicus | LINEAR ACCELERATORS | PATIENTS | NERVOUS SYSTEM DISEASES | SALIVARY GLANDS | RADIOLOGY AND NUCLEAR MEDICINE | RADIATION DOSES | TOXICITY | SIDE EFFECTS | EFFICIENCY
Radiology | Hematology, Oncology and Palliative Medicine | MANAGEMENT | SIALORRHEA | ONCOLOGY | DISEASE | ALS | RADIOTHERAPY | CANCER | RADIOLOGY, NUCLEAR MEDICINE & MEDICAL IMAGING | BOTULINUM-TOXIN | Radiotherapy Dosage | Prospective Studies | Humans | Middle Aged | Male | Photons - therapeutic use | Amyotrophic Lateral Sclerosis - complications | Submandibular Gland | Parotid Gland - radiation effects | Sialorrhea - etiology | Statistics, Nonparametric | Aged, 80 and over | Adult | Female | Remission Induction - methods | Aged | Sialorrhea - radiotherapy | Sects | Medical research | Nervous system diseases | Care and treatment | Nuclear radiation | Medicine, Experimental | Amyotrophic lateral sclerosis | Radiotherapy | Index Medicus | LINEAR ACCELERATORS | PATIENTS | NERVOUS SYSTEM DISEASES | SALIVARY GLANDS | RADIOLOGY AND NUCLEAR MEDICINE | RADIATION DOSES | TOXICITY | SIDE EFFECTS | EFFICIENCY
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Full Text
The motor unit number index (MUNIX) profile of patients with adult spinal muscular atrophy
Clinical Neurophysiology, ISSN 1388-2457, 11/2018, Volume 129, Issue 11, pp. 2333 - 2340
Objective of this study is the comprehensive characterisation of motor unit (MU) loss in type III and IV Spinal Muscular Atrophy (SMA) using motor unit number...
Biomarkers | MUNIX | SMA type III | SMA type IV | Motor unit loss | MUSIX | HEALTHY-SUBJECTS | ALS | STRENGTH | AMYOTROPHIC-LATERAL-SCLEROSIS | NATURAL-HISTORY | NEUROSCIENCES | REPRODUCIBILITY | CLINICAL NEUROLOGY | CHILDREN | NEURON LOSS | DISEASE | MUSCLES | Disability Evaluation | Humans | Middle Aged | Muscle, Skeletal - physiopathology | Adult | Female | Male | Muscle, Skeletal - innervation | Recruitment, Neurophysiological | Muscle Strength | Spinal Muscular Atrophies of Childhood - pathology | Spinal muscular atrophy | Life Sciences | Human health and pathology
Biomarkers | MUNIX | SMA type III | SMA type IV | Motor unit loss | MUSIX | HEALTHY-SUBJECTS | ALS | STRENGTH | AMYOTROPHIC-LATERAL-SCLEROSIS | NATURAL-HISTORY | NEUROSCIENCES | REPRODUCIBILITY | CLINICAL NEUROLOGY | CHILDREN | NEURON LOSS | DISEASE | MUSCLES | Disability Evaluation | Humans | Middle Aged | Muscle, Skeletal - physiopathology | Adult | Female | Male | Muscle, Skeletal - innervation | Recruitment, Neurophysiological | Muscle Strength | Spinal Muscular Atrophies of Childhood - pathology | Spinal muscular atrophy | Life Sciences | Human health and pathology
Journal Article
Neurobiology of Aging, ISSN 0197-4580, 2011, Volume 32, Issue 3, pp. 557.e11 - 557.e13
Abstract Mutations in OPTN gene encoding optineurin have recently been identified at the homozygote and heterozygote state in Japanese families with slowly...
Neurology | Internal Medicine | Familial ALS | Amyotrophic lateral sclerosis | R96L OPTN mutation | Motor neuron disease | Genetic analysis | OPEN-ANGLE GLAUCOMA | OPTINEURIN | MUTATIONS | NEUROSCIENCES | GERIATRICS & GERONTOLOGY | France - epidemiology | Glaucoma, Open-Angle - genetics | Amyotrophic Lateral Sclerosis - genetics | Humans | Computational Biology | Transcription Factor TFIIIA - genetics | Exons - genetics | Family Health | Genotype | Male | Mutation - genetics | Genetic Testing - methods | Transcription Factor TFIIIA - metabolism | Animals | Amyotrophic Lateral Sclerosis - metabolism | Female | Glaucoma | Family | Genetic aspects | Ophthalmology | Analysis
Neurology | Internal Medicine | Familial ALS | Amyotrophic lateral sclerosis | R96L OPTN mutation | Motor neuron disease | Genetic analysis | OPEN-ANGLE GLAUCOMA | OPTINEURIN | MUTATIONS | NEUROSCIENCES | GERIATRICS & GERONTOLOGY | France - epidemiology | Glaucoma, Open-Angle - genetics | Amyotrophic Lateral Sclerosis - genetics | Humans | Computational Biology | Transcription Factor TFIIIA - genetics | Exons - genetics | Family Health | Genotype | Male | Mutation - genetics | Genetic Testing - methods | Transcription Factor TFIIIA - metabolism | Animals | Amyotrophic Lateral Sclerosis - metabolism | Female | Glaucoma | Family | Genetic aspects | Ophthalmology | Analysis
Journal Article
PloS one, ISSN 1932-6203, 2016, Volume 11, Issue 12, p. e0167886
Journal Article
PLOS ONE, ISSN 1932-6203, 12/2016, Volume 11, Issue 12, p. e0152439
Journal Article
Frontiers in neuroscience, ISSN 1662-4548, 2019, Volume 13, p. 538
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disorder causing a progressive motor weakness of all voluntary muscles, whose progression challenges...
Amyotrophic lateral sclerosis | Nervous system diseases | Caregivers | Handwriting | Neurodegenerative diseases | Muscles | Eye movements | Patients | Quality of life | Apathy | Smooth pursuit eye movements | Writing | Communication | Dementia | Life Sciences | Neurons and Cognition | pilot clinical study | smooth-pursuit eye movements | motor learning | amyotrophic lateral sclerosis | assisted communication devices
Amyotrophic lateral sclerosis | Nervous system diseases | Caregivers | Handwriting | Neurodegenerative diseases | Muscles | Eye movements | Patients | Quality of life | Apathy | Smooth pursuit eye movements | Writing | Communication | Dementia | Life Sciences | Neurons and Cognition | pilot clinical study | smooth-pursuit eye movements | motor learning | amyotrophic lateral sclerosis | assisted communication devices
Journal Article