Heart Vessels and Transplantation (Aug 2024)
Amyotrophic lateral sclerosis in a combination with frontotemporal dementia: A clinical case study
Abstract
Objective: Amyotrophic lateral sclerosis (ALS) is a devastating neurodegenerative disease affecting both central and peripheral motor neurons, presenting a significant challenge in modern neurology. This article discusses the multifactorial nature of ALS, its prevalence, and the absence of effective treatment. Clinical manifestations include damage to upper and lower motor neurons, with varied courses influenced by genetic mutations. Atypical forms, such as ALS with frontotemporal dementia, pose diagnostic challenges due to cognitive symptoms that may precede motor impairments. The study emphasizes the importance of neurophysiological and cognitive diagnostic methods in identifying associated conditions. Case presentation: The article presents a clinical observation of a 51-year-old patient with a rare combination of ALS and frontotemporal dementia. The patient exhibited a progressive decline in motor function, cognitive impairments, and behavioral changes. Detailed clinical descriptions, including neurological and neuropsychological assessments, highlight the complexity of the case. Instrumental studies, such as magnetic resonance imaging and electomyography, revealed atrophy in frontotemporal areas and anterior horn involvement, corroborating the diagnosis. Treatment involved edaravone and B vitamins, resulting in a modest improvement in motor function. Conclusion: The case underscores the rarity of combined ALS and frontotemporal degeneration, prompting further research and dynamic monitoring. While current interventions can slow disease progression, effective treatments for ALS remain elusive. The coexistence of frontotemporal dementia complicates the disease course, necessitating ongoing clinical attention and research efforts.
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