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Updated: May 27, 2026
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Diffusion Tensor Magnetic Resonance Imaging in the Analysis of Neurodegenerative Diseases
Published on: July 28, 2013
Right temporal variant frontotemporal dementia with motor neuron disease
Elizabeth A Coon1, Jennifer L Whitwell, Joseph E Parisi
1Department of Neurology, Behavioral Neurology, Mayo Clinic, Rochester, MN 55905, USA. coon.elizabeth@mayo.edu
Summary
Frontotemporal dementia with motor neuron disease (FTD-MND) can present with severe right temporal lobe atrophy. This suggests a potential new subtype, right temporal variant FTD-MND, requiring further study.
Area of Science:
- Neurology
- Neuroscience
- Pathology
Background:
- Frontotemporal dementia (FTD) subtypes exhibit distinct atrophy patterns.
- Right temporal variant FTD is linked to behavioral and semantic deficits.
- The anatomical basis for FTD with motor neuron disease (FTD-MND) with right temporal atrophy was previously undescribed.
Observation:
- This study identified FTD-MND patients with predominant right temporal lobe atrophy.
- Neuroimaging revealed significant right temporal atrophy, corticospinal tract degeneration, and hypometabolism.
- Pathological examination of two patients showed TDP-43 type 3 pathology.
Findings:
- A correlation between FTD-MND and severe right temporal lobe atrophy was observed.
- Patients presented with varied behavioral and aphasic symptoms despite similar atrophy patterns.
- TDP-43 pathology was predominant in the examined cases.
Implications:
- This suggests a potential 'right temporal variant FTD-MND' subtype.
- Further research is needed to fully characterize this FTD-MND presentation.
- Understanding these patterns aids in refining FTD diagnoses and understanding disease mechanisms.
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