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Related Experiment Video

Updated: Jan 10, 2026

An Instrumented Pull Test to Characterize Postural Responses

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Startle Reflex in Primary Lateral Sclerosis (PLS): A Comparison With Amyotrophic Lateral Sclerosis (ALS).

Grace E Jang¹, Ikjae Lee¹, Jinsy A Andrews²

¹Department of Neurology, Columbia University Irving Medical Center, New York, New York, USA.

|November 29, 2025

Summary

Startle reflex (SR) is significantly more prevalent in primary lateral sclerosis (PLS) than in amyotrophic lateral sclerosis (ALS). This suggests SR may be a novel clinical marker for PLS and upper motor neuron-predominant ALS.

Keywords:

amyotrophic lateral sclerosis (ALS)primary lateral sclerosis (PLS)startle reflex

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Area of Science:

Neurology
Neuroscience
Clinical Research

Background:

Limited data exists on startle reflex (SR) in primary lateral sclerosis (PLS).
Understanding SR in PLS is crucial for differential diagnosis and disease characterization.
Comparison with amyotrophic lateral sclerosis (ALS) can provide valuable insights.

Purpose of the Study:

To investigate the presence and prevalence of SR in PLS.
To compare SR findings between PLS and ALS.
To explore potential clinical markers for these motor neuron diseases.

Main Methods:

Cross-sectional study involving 46 PLS and 54 ALS participants.
Structured interviews were used to assess SR.
Fisher's exact test and multivariable linear regression analyzed prevalence and associations.

Main Results:

SR prevalence was markedly higher in PLS (93.5%) compared to ALS (20.4%).
In ALS, SR was observed in all upper motor neuron (UMN)-predominant cases (100%).
Younger age, shorter disease duration, and PLS diagnosis correlated with more frequent SR.

Conclusions:

SR is significantly more common in PLS than ALS.
Predominant UMN involvement appears key for SR in both conditions.
SR may serve as a novel clinical marker in PLS and UMN-predominant ALS, requiring further validation.