Amyotrophic lateral sclerosis restricted to motor deficits represents a phenotypic variant of disease

Takeaway

  • Amyotrophic lateral sclerosis that only has motor deficits may represent a phenotypic variant of amyotrophic lateral sclerosis, rather than a progression of disease.

Why this matters

  • Substantial overlap in clinical, genetic, and pathological features has been reported between amyotrophic lateral sclerosis and behavioral variant of frontotemporal dementia. Across the disease spectrum, people with amyotrophic lateral sclerosis exhibit mild cognitive and/or behavioral symptoms but it is unclear if this is a distinct phenotype or natural progression.

  • This novel study used innovative magnetic resonance imaging approaches to assess distinctive patterns of network disruption. It provides a comprehensive characterization of the neural correlates across the clinical spectrum of amyotrophic lateral sclerosis-frontotemporal dementia which will facilitate disease classification.