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Frontotemporal lobar degeneration without lobar atrophy.

Keith A Josephs1, Jennifer L Whitwell, Clifford R Jack

  • 1Department of Neurology, Division of Behavioral Neurology and Movement Disorders, Mayo Clinic, Rochester, MN 55905, USA. josephs.keith@mayo.edu

Archives of Neurology
|November 15, 2006
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Summary

Frontotemporal lobar degeneration with ubiquitin-only-immunoreactive neuronal inclusions (FTLD-U) cases with hippocampal sclerosis (HpScl) show significant frontal and temporal lobe atrophy. Cases without HpScl exhibit minimal cortical atrophy, suggesting distinct disease pathways.

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Area of Science:

  • Neurology
  • Neuroscience
  • Pathology

Background:

  • Frontotemporal lobar degeneration with ubiquitin-only-immunoreactive neuronal inclusions (FTLD-U) is a common form of frontotemporal dementia.
  • Hippocampal sclerosis (HpScl), characterized by neuronal loss in the hippocampus, is observed in many FTLD-U cases.

Purpose of the Study:

  • To investigate clinical and magnetic resonance imaging (MRI) correlates of HpScl in FTLD-U.
  • To differentiate FTLD-U cases with and without HpScl based on structural imaging and pathology.

Main Methods:

  • Retrospective review of 24 FTLD-U cases with detailed clinical and pathological data.
  • Voxel-based morphometry (VBM) used to assess gray matter atrophy patterns.
  • Cases were categorized based on the presence or absence of HpScl.

Main Results:

  • No significant differences in demographics or clinical features between FTLD-U cases with and without HpScl.
  • VBM revealed minimal cortical atrophy in FTLD-U cases without HpScl.
  • FTLD-U cases with HpScl demonstrated moderate to severe frontal and temporal lobe atrophy, consistent with histopathology.

Conclusions:

  • FTLD-U with and without HpScl exhibit distinct patterns of cortical atrophy on structural imaging.
  • FTLD-U cases lacking HpScl show minimal atrophy, challenging current FTLD staging.
  • These differences suggest potentially different underlying genetic or environmental factors in FTLD-U subtypes.