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Related Experiment Videos

The auditory startle response in progressive supranuclear palsy

J C Rothwell1, M Vidailhet, P D Thompson

  • 1Institute of Neurology, MRC Human Movement and Balance Unit, London, United Kingdom.

Journal of Neural Transmission. Supplementum
|January 1, 1994
PubMed
Summary

The auditory startle response originates in the brainstem and spinal cord. Progressive supranuclear palsy patients lack this response, while Parkinson's disease patients show a delayed startle, suggesting basal ganglia influence.

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Area of Science:

  • Neuroscience
  • Neurology
  • Clinical Electrophysiology

Background:

  • The auditory startle response involves brainstem and spinal pathways.
  • Its neurophysiological underpinnings are not fully elucidated.
  • Understanding its alterations in neurodegenerative diseases is crucial.

Purpose of the Study:

  • To investigate the neuroanatomical origin of the human auditory startle response using EMG.
  • To examine the auditory startle response in patients with progressive supranuclear palsy (PSP) and Parkinson's disease (PD).
  • To explore the role of the basal ganglia in modulating the startle reflex.

Main Methods:

  • Electromyography (EMG) was used to record muscle activity during auditory startle.
  • Auditory startle responses were assessed in healthy individuals, PSP patients, and PD patients.

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  • EMG characteristics, including onset latency and amplitude, were analyzed.

    • Main Results:

    • Normal auditory startle response originates in the pontine reticular formation and travels via a slow spinal pathway.
    • Patients with PSP showed reduced or absent startle responses, indicating pontine reticular formation pathology.
    • PD patients exhibited normal startle amplitude but delayed onset, unaffected by L-dopa treatment.

    Conclusions:

    • The findings support a pontine origin and slow spinal conduction for the human auditory startle reflex.
    • PSP is associated with damage to the lower pontine reticular formation, disrupting the startle pathway.
    • The delayed startle in PD may result from altered basal ganglia output to brainstem centers.