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Motor neuron disease and frontotemporal lobar degeneration: a tale of two disorders linked to TDP-43

Lauren B Elman1, Leo McCluskey, Murray Grossman

  • 1Department of Neurology, University of Pennsylvania School of Medicine, Philadelphia, PA, USA. elmanl@uphs.upenn.edu

Neuro-Signals
|December 22, 2007
PubMed

Insights

Motor neuron disease (MND) is increasingly linked to cognitive impairment, with up to half of patients experiencing difficulties. This review highlights the fundamental connection between MND and frontotemporal lobar degeneration (FTLD).

Area of Science:

  • Neuroscience
  • Neurology
  • Pathology

Background:

  • Motor neuron disease (MND) was traditionally associated solely with motor weakness.
  • Emerging evidence indicates significant cognitive deficits in a substantial portion of MND patients.
  • A subset of MND patients exhibit dementia clinically and pathologically similar to frontotemporal lobar degeneration (FTLD).

Purpose of the Study:

  • To review the growing body of evidence linking Motor Neuron Disease (MND) and Frontotemporal Lobar Degeneration (FTLD).
  • To discuss the presence and nature of cognitive difficulties and dementia in MND patients.
  • To explore the shared pathological hallmarks between MND and FTLD.

Main Methods:

  • Review of existing literature on cognitive function in MND.
  • Analysis of neuroimaging studies comparing MND and FTLD.
  • Examination of biochemical and histopathological findings, particularly TDP-43 inclusions.

Main Results:

  • Cognitive difficulties are identified in up to 50% of individuals with MND.
  • Approximately 5-10% of MND patients present with dementia resembling FTLD.
  • Neuroimaging reveals abnormalities consistent with FTLD in MND patients.
  • Identical ubiquinated TDP-43 inclusions are observed in both MND and FTLD neuropathology.

Conclusions:

  • There is a fundamental pathological and clinical link between Motor Neuron Disease and Frontotemporal Lobar Degeneration.
  • Cognitive impairment is a significant feature of MND, extending beyond motor symptoms.
  • Shared TDP-43 proteinopathy underscores the close relationship between these neurodegenerative disorders.

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