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Survival in two variants of tau-negative frontotemporal lobar degeneration: FTLD-U vs FTLD-MND

K A Josephs1, D S Knopman, J L Whitwell

  • 1Department of Neurology, Mayo Clinic, 200 First St S.W., Rochester, MN 55905, USA. josephs.keith@mayo.edu

Neurology
|August 24, 2005
PubMed

Insights

Frontotemporal lobar degeneration (FTLD) with motor neuron disease (FTLD-MND) has a significantly shorter survival time than FTLD with motor neuron disease type inclusions but without motor neuron disease (FTLD-U). This indicates FTLD-MND is a more aggressive disease.

Area of Science:

  • Neuropathology
  • Neurodegenerative Diseases

Background:

  • Frontotemporal lobar degeneration (FTLD) is pathologically classified into tau-positive and tau-negative subtypes.
  • Tau-negative FTLD encompasses FTLD with motor neuron disease (FTLD-MND) and FTLD with motor neuron disease type inclusions but without motor neuron disease (FTLD-U).

Purpose of the Study:

  • To compare patient outcomes and survival rates between FTLD-MND and FTLD-U.
  • To determine if FTLD-MND represents a more aggressive disease process compared to FTLD-U.

Main Methods:

  • Analysis of patient outcomes.
  • Comparison of survival data between FTLD-MND and FTLD-U cohorts.

Main Results:

  • FTLD-MND patients exhibited significantly shorter survival durations compared to FTLD-U patients.
  • The findings suggest a differential disease aggressiveness between the two tau-negative FTLD subtypes.

Conclusions:

  • FTLD-MND is associated with a more aggressive disease course than FTLD-U.
  • Pathologic classification impacts clinical prognosis in tau-negative FTLD.

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