Difficulties in Differential Diagnostics of neurodegenerative disorders.

What is it about?

Multiple system atrophy (MSA) is a psoradic adult-onset disease onset and progressive neurodegenerative disorder that may manifest with diverse clinical features. Although definite diagnosis of the MSA can only be made by confirming neuropathological evidence, the diagnosis can be supported by clinical and imaging features. In this report, we present a woman with primary symptoms such as rapidly progressive aphasia, mild limb ataxia and autonomic dysfunction. After 2 years of the disease progression, she developed global aphasia, parkinsonism syndrome, cerebellar ataxia, pyramidal signs and severe mental decline. She had no response to dopaminergic treatment. Differential diagnosis was complicated because of atypical early disease presentation with dominant progressive aphasia and imaging findings, and was performed with frontotemporal dementia, corticobasal degeneration, dementia with Lewy bodies and progressive supranuclear palsy. In our case, all neurological signs and symptoms as well as the disease course are more typical for multiple system atrophy, despite the imaging findings and cognitive decline which developed in the late stage of the disease.

Featured Image

Why is it important?

Clinically differentiating MSA from progressive supranuclear palsy and other similar neurodegenerative disorders may be challenging in all stages of the disease, especially with atypical disease presentation. The diagnostic criteria for MSA have only been validated retrospectively. Although definite diagnosis of the MSA can only be made by confirming neuropathological evidence, the diagnosis can be supported by clinical and imaging features.

Perspectives