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Whisker-signaled Eyeblink Classical Conditioning in Head-fixed Mice
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Blink reflex in primary lateral sclerosis.

D G Podikoglou1, T G Avramidis, A L Papadimitriou

  • 1Department of Neurology, Red Cross Hospital, Athens, Greece. danisgr@teledomenet.gr

Journal of Neurology, Neurosurgery, and Psychiatry
|June 18, 2004
PubMed
Summary

Primary lateral sclerosis (PLS) shows abnormal blink reflex (BR) circuits, unlike amyotrophic lateral sclerosis (ALS) where BR progressively disappears. This study differentiates PLS pathophysiology and aids in tracking ALS progression.

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Area of Science:

  • Neuroscience
  • Neurology

Background:

  • Primary lateral sclerosis (PLS) is a rare motor neuron disease.
  • Understanding PLS pathophysiology is crucial for diagnosis and management.

Purpose of the Study:

  • To investigate potential changes in the blink reflex (BR) circuit in patients with primary lateral sclerosis (PLS).
  • To differentiate PLS from amyotrophic lateral sclerosis (ALS) using BR.
  • To explore BR as a potential marker for disease progression.

Main Methods:

  • Blink reflex (BR) was assessed in 10 PLS patients, 10 ALS patients, and 30 healthy controls.
  • Evoked potential studies were used to analyze BR.
  • Patients were followed annually for 5 years.

Main Results:

  • Both PLS and ALS groups exhibited significantly lower R(2) and R(2)' amplitudes compared to healthy controls (p<0.001).
  • R(1) amplitude/latency and R(2)/R(2)' latencies were normal in all patient groups.
  • PLS patients showed no significant changes in BR over 5 years, while ALS patients demonstrated progressive BR disappearance.

Conclusions:

  • The blink reflex (BR) is demonstrably abnormal in primary lateral sclerosis (PLS).
  • BR alterations offer insights into PLS pathophysiology.
  • BR serves as a valuable marker for monitoring amyotrophic lateral sclerosis (ALS) progression.