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Updated: May 23, 2025

Utility of Dissociated Intrinsic Hand Muscle Atrophy in the Diagnosis of Amyotrophic Lateral Sclerosis
Published on: March 4, 2014
[Challenges in Delivering the Diagnosis of Multiple System Atrophy]
1Department of Neurology, Graduate School of Medicine, Chiba University.
Abstract:
Multiple system atrophy (MSA) is a progressive, incurable neurodegenerative disease characterized by the risk of sudden death and various symptoms, including autonomic and cognitive dysfunction, as well as motor symptoms such as cerebellar ataxia and parkinsonism. These clinical features make the diagnosis of MSA challenging for neurologists. Our questionnaire survey on the delivery of an MSA diagnosis revealed that 92.3% of the participating neurologists found it difficult to deliver the diagnosis. However, 82.8% perceived explaining the risk of sudden death to be challenging. Factors independently associated with difficulties in delivering a diagnosis included perceived challenges in the differential diagnosis of MSA, conveying information about the risk of sudden death, and explaining the importance of the family's decision-making process in life-prolonging treatment. Further research is required to develop guidelines for delivering the diagnosis of MSA.
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