Amyotrophic lateral sclerosis
Authors
Discussion
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Implication
Clinical review highlights non-motor neuraxis involvement and limited disease-modifying therapies in amyotrophic lateral sclerosis, indicating a need for broader neuroprotective interventions.
Key Points
- To review the neuropathology, genetic underpinnings, and therapeutic landscape of amyotrophic lateral sclerosis.
- Synthesized neuropathological and neuroimaging evidence evaluating motor and non-motor neuraxis involvement in amyotrophic lateral sclerosis.
- Assessed clinical management strategies, comparing approved disease-modifying therapies with supportive symptomatic interventions.
- Amyotrophic lateral sclerosis involves the degeneration of upper and lower motor neurons, with evidence showing pathology extending into the non-motor neuraxis.
- A subset of cases have familial origins linked to neuronal gene mutations, whereas the pathogenic drivers of sporadic disease remain largely unexplained.
- Only two disease-modifying drugs are available to decelerate progression, requiring patient care to rely primarily on symptomatic treatments for spasticity and dysarthria.
Cite This Study
Hardiman et al. (2017) studied this question.