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Primary lateral sclerosis: a neuropsychological study

R J Caselli1, B E Smith, D Osborne

  • 1Department of Neurology, Mayo Clinic Scottsdale, AZ 85259, USA.

Neurology
|November 1, 1995
PubMed
Summary
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Eight of nine patients with primary lateral sclerosis (PLS) showed mild cognitive impairment, particularly in frontal lobe functions and memory. Cognitive testing may aid in localizing neurological deficits in PLS.

Area of Science:

  • Neuroscience
  • Neurology

Background:

  • Primary lateral sclerosis (PLS) is a rare, progressive motor neuron disease.
  • Cognitive function in PLS is not well-characterized.

Purpose of the Study:

  • To investigate the cognitive profiles of patients diagnosed with primary lateral sclerosis.
  • To explore potential links between cognitive impairment and frontal lobe dysfunction in PLS.

Main Methods:

  • Cognitive testing was administered to nine patients with clinically and radiologically confirmed PLS.
  • Neuropsychological tests sensitive to frontal lobe functions and memory were utilized.

Main Results:

  • No patients presented with dementia.
  • Eight out of nine patients exhibited mild cognitive impairment.

Related Experiment Videos

  • Impairments were most prominent in tests assessing frontal lobe functions, followed by memory-related tests.

    • Conclusions:

    • Cognitive testing can be a valuable tool for identifying cortical involvement in progressive spasticity syndromes like PLS.
    • Findings suggest potential nosological relationships between PLS and other frontal lobe degenerative disorders, including frontotemporal dementia.