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ALS - Motor Neuron Disease: Mechanism and Development of New Therapies
15:48

ALS - Motor Neuron Disease: Mechanism and Development of New Therapies

Published on: July 29, 2007

Clinically undetected motor neuron disease in pathologically proven frontotemporal lobar degeneration with motor

Keith A Josephs1, Joseph E Parisi, David S Knopman

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

Archives of Neurology
|April 12, 2006
PubMed
Abstract

Insights

Clinical diagnosis of frontotemporal lobar degeneration with motor neuron disease (FTLD-MND) is challenging. Many pathologically confirmed FTLD-MND cases show dementia signs, but motor neuron disease signs are less consistently detected, influenced by pathological spectrum and hippocampal sclerosis.

Area of Science:

  • Neurology
  • Pathology
  • Neuroscience

Background:

  • Frontotemporal lobar degeneration with motor neuron disease (FTLD-MND) is a complex neuropathological entity.
  • Clinical detection of both dementia and motor neuron disease in pathologically confirmed FTLD-MND requires assessment.

Purpose of the Study:

  • To evaluate clinical diagnostic accuracy for FTLD-MND.
  • To identify reasons for potential misdiagnosis in FTLD-MND.

Main Methods:

  • Retrospective review of medical records for pathologically confirmed FTLD-MND cases.
  • Semiquantitative analysis of motor and extramotor pathological findings.

Main Results:

  • All 17 FTLD-MND cases exhibited clinical frontotemporal dementia; only 10 (59%) showed clinical motor neuron disease.
  • Hippocampal sclerosis was more frequent in cases lacking clinical motor neuron disease (P<.01).
  • Milder neuronal loss, gliosis, and corticospinal tract degeneration were noted in cases without clinical motor neuron disease.

Conclusions:

  • Clinical diagnostic sensitivity for FTLD-MND elements is modest.
  • FTLD-MND heterogeneity and mild motor neuron degeneration, especially with hippocampal sclerosis, complicate clinical diagnosis.

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