Objective: To identify a novel physiologic marker of bulbar motor involvement in amyotrophic lateral sclerosis (ALS) based on jaw muscle synergies.
Methods: Muscle synergies were extracted from the surface electromyographic recordings of five jaw muscles during speech in 11 individuals at the prodromal or symptomatic stages of bulbar ALS and 10 neurologically-healthy controls, using non-negative matrix factorization. The disrupted muscle synergies in ALS were identified; their efficacy in differentiating individuals with ALS from healthy controls and relation to the previously-reported kinematic and functional speech deficits were determined.
Results: An antagonist synergy (i.e., masseter × digastric) was significantly disrupted in ALS, which differentiated individuals with ALS from healthy controls with 82% sensitivity and 90% specificity. Such a disruption occurred prodromally and was associated with slowed jaw movement and reduced speaking rate across the range of severity.
Conclusions: The disruption of jaw antagonist synergy in ALS likely reflects the impact of impaired neural drive on the coordinative functioning of bulbar muscles, which may be used to assess bulbar motor involvement.
Significance: Jaw antagonist synergy shows promise as a novel physiologic marker of bulbar motor involvement in ALS, which has the potential to serve as a quantitative measurement tool for bulbar assessment.
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http://dx.doi.org/10.1016/j.clinph.2020.09.030 | DOI Listing |
Brain Commun
January 2025
Neuromuscular Department, Motor Neuron Disease Centre, Queen Square Institute of Neurology, University College London, London WC1N 3BG, UK.
Neuroinflammation impacts on the progression of amyotrophic lateral sclerosis (ALS), a fatal neurodegenerative disorder. Specialized pro-resolving mediators trigger the resolution of inflammation. We investigate the specialized pro-resolving mediator blood profile and their receptors' expression in peripheral blood mononuclear cells in relation to survival in ALS.
View Article and Find Full Text PDFBMC Neurol
January 2025
Department of Neurology, Wessex Neurological Centre, University Hospital Southampton, Southampton, UK.
Brain Sci
November 2024
Clinical Neuroanatomy, Department of Neurology, University Hospital Ulm, 89081 Ulm, Germany.
Creativity and the production of artwork can have an impact on the course and treatment of comorbid severe mental illness and neurodegeneration. We report on a 70-year-old male patient with highly original artistic behavior, who suffered from lifelong recurrent major depression and subsequently developed symptoms of progressive bulbar palsy (PBP). In the context of a systematic literature review, we detail the patient's personal and artistic biographies and portray artwork from his artistic portfolio together with his disease history, clinical examination, psychopathological and neuropsychological evaluations, blood and cerebrospinal fluid analyses, neuroimaging, neurophysiological testing, and psychotherapeutic treatment.
View Article and Find Full Text PDFMol Neurobiol
January 2025
Department of Translational Medicine, Università del Piemonte Orientale, Novara, Italy.
Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disorder that primarily affects the motor neurons in the brain and spinal cord. While the exact cause of ALS is not fully understood, a combination of genetic and environmental factors is believed to contribute to its development. Growth arrest-specific 6 (Gas6), a vitamin K-dependent protein, has been recognized to enhance oligodendrocytes and neurons' survival and is associated with different kinds of (neuro)inflammatory conditions.
View Article and Find Full Text PDFJ Neurol Sci
December 2024
Department of Neurology, Seoul National University Hospital, Seoul, Republic of Korea; Department of Translational Medicine, Seoul National University College of Medicine, Seoul, Republic of Korea; Biomedical Research Institute, Seoul National University Hospital, Seoul, Republic of Korea; Wide River Institute of Immunology, Seoul National University, Hongcheon, Republic of Korea; Neuroscience Research Institute, Seoul National University College of Medicine, Seoul, Republic of Korea. Electronic address:
Facial onset sensory and motor neuronopathy (FOSMN) syndrome is a rare neurodegenerative disorder initially characterized by facial sensory deficits, which later progress to motor deficits in a rostral-caudal distribution. This study investigated the prevalence, clinical features, and prognosis of FOSMN syndrome and compared these aspects with those of bulbar-onset amyotrophic lateral sclerosis (ALS) within a single institutional cohort of motor neuron diseases. We identified four patients with FOSMN syndrome who had been misclassified as having bulbar-onset ALS, representing approximately 2 % of such ALS cases.
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