Amyotrophic lateral sclerosis (ALS) is classified as a multigenic, multifactorial, and heterogeneous neurodegenerative/neuroinflammatory diseasethat slays especially upper and lower motor neurons controlling voluntary muscle activity. After the insurgence that is characterized bytypical symptoms such as weakness in the limbs and muscle twitches, the disease rapidly evolves into progressive muscle atrophy, paralysis, andlastly death occurring by respiratory failure usually within 2-4 years of diagnosis. ALS is now understood as a multisystem and broadspectrummotor neuron disease largely variable in presentation and outcomes, also showing extra-motor deficits such as extrapyramidal, thalamic, cerebellar, and sensory nerve abnormalities, in addition to comorbid cognitivebehavioral instabilities and psychiatric symptoms.Approximately 15% of ALS patients are also reported suffering from frontotemporal dementia. Concomitant immunological irregularities andgut dysbiosis are often observed. The autonomic nervous system is also involved in the disease, since patients die of sudden death when they lose their ability to compensate for cardiorespiratory arrest. It clearly emerges that a disease like ALS impacts multiple systems throughout the body, and it is not just a neurological disorder.
Amyotrophic lateral sclerosis disease burden: doing better at getting better
Volonte' C;
2023
Abstract
Amyotrophic lateral sclerosis (ALS) is classified as a multigenic, multifactorial, and heterogeneous neurodegenerative/neuroinflammatory diseasethat slays especially upper and lower motor neurons controlling voluntary muscle activity. After the insurgence that is characterized bytypical symptoms such as weakness in the limbs and muscle twitches, the disease rapidly evolves into progressive muscle atrophy, paralysis, andlastly death occurring by respiratory failure usually within 2-4 years of diagnosis. ALS is now understood as a multisystem and broadspectrummotor neuron disease largely variable in presentation and outcomes, also showing extra-motor deficits such as extrapyramidal, thalamic, cerebellar, and sensory nerve abnormalities, in addition to comorbid cognitivebehavioral instabilities and psychiatric symptoms.Approximately 15% of ALS patients are also reported suffering from frontotemporal dementia. Concomitant immunological irregularities andgut dysbiosis are often observed. The autonomic nervous system is also involved in the disease, since patients die of sudden death when they lose their ability to compensate for cardiorespiratory arrest. It clearly emerges that a disease like ALS impacts multiple systems throughout the body, and it is not just a neurological disorder.File | Dimensione | Formato | |
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