12/19/2023 0 Comments Emg als findings![]() ![]() Both reviews are written for the general neurology community.ĪLS incidence and prevalence varies across the globe and estimates are based on different data sources. This review is accompanied by a second more research focused article, which provides an update on complex genetics, pathophysiology, therapeutic development, and exposome science. This review will highlight these emerging discoveries and focus on the most recent ALS advances in diagnosis and prognosis within the past five years. To facilitate earlier diagnosis and improve prognosis, research is on-going into new ALS criteria and scoring systems, as well as emerging diagnostic and prognostic biofluid markers, imaging modalities, and electrophysiological measurements. 3 Additionally, the prognosis of ALS patients remains suboptimal because the determinants of progression are not fully known. The typical median time between initial symptoms and a definitive diagnosis is 10 to 16 months 2 secondary to the rarity and unfamiliarity of disease, incomplete recognition of symptoms, and lack of early and appropriate specialist involvementi. Death from ALS generally occurs within two to four years from diagnosis from respiratory failure, although more slowly progressive forms of the illness occur in a small proportion of patients.ĭiagnosing ALS can be challenging, and the process has remained essentially unchanged in clinical practice in the last decade and no test or tool has replaced clinical history and examination for confirming diagnosis, even with the greater adoption of genetic testing. ALS is now understood as a systems disease and there is significant variation in clinical presentations, including non-motor symptoms, behavioral changes, and cognitive decline, including frontotemporal dementia (FTD). Most patients with spinal onset ALS present with weakness in one body region that spreads over time to the same region on the contralateral side, as well as regions rostral and caudal to the initial onset region. 11 Weakness begins in facial, tongue, and pharyngeal muscles in bulbar onset ALS producing dysarthria and then dysphagia, or in distal upper or lower limb muscles in spinal onset ALS. We hope that improved diagnostic tools for ALS will shorten time to diagnosis, leading to earlier treatment and potentially better outcomes, whereas improved prognostic tools will help ALS patients understand their likely disease course.Īmyotrophic lateral sclerosis (ALS) is a neurodegenerative disease, characterized by progressive, painless muscle weakness due to motor neuron death in the brain, brainstem, and spinal cord. Lastly, new tools like biofluid markers, imaging modalities, and neuromuscular electrophysiological measurements may increase diagnostic and prognostic accuracy. Novel staging and scoring systems can help monitor ALS patients’ disease progression and may potentially serve as clinical trial outcomes. Improved prognosis, such as the ENCALS survival model, could inform the patient and their family about disease course and improve end of life planning. An evolving awareness of ALS genotypes and phenotypes and new ALS criteria, such as the recent Gold Coast criteria, could expedite diagnosis. Earlier diagnosis can improve ALS patient outcomes as timely interventions slow disease progression. Diagnosing ALS can be challenging due to heterogeneity in clinical presentation and overlap with other neurological and neuropsychological conditions. ![]()
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