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Utility of Dissociated Intrinsic Hand Muscle Atrophy in the Diagnosis of Amyotrophic Lateral Sclerosis

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ALS With and Without Upper Motor Neuron Signs: A Comparative Study Supporting the Gold Coast Criteria.

Hee-Jae Jung^1,2, E-Nae Cheong³, Jungmin So⁴

¹Department of Neurology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Republic of Korea.

Annals of Clinical and Translational Neurology

|December 15, 2025

Summary

Amyotrophic lateral sclerosis (ALS) patients without upper motor neuron (UMN) signs share similar characteristics and prognoses to those with UMN signs. These findings support the Gold Coast criteria for diagnosing ALS, even without UMN signs.

Keywords:

Gold Coast criteria amyotrophic lateral sclerosis upper motor neuron

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Area of Science:

Neurology
Neuroscience
Clinical Medicine

Background:

The Gold Coast criteria allow amyotrophic lateral sclerosis (ALS) diagnosis without upper motor neuron (UMN) signs.
Uncertainty exists regarding the clinical characteristics and prognoses of ALS patients with (ALSwUMN) versus without (ALSwoUMN) UMN signs.

Purpose of the Study:

To compare clinical features, disease progression, electrophysiological findings, biomarker profiles, imaging parameters, and survival between ALSwUMN and ALSwoUMN groups.
To evaluate the validity of the Gold Coast criteria in diagnosing ALS without UMN signs.

Main Methods:

Classified 71 ALS patients into ALSwUMN (n=51) and ALSwoUMN (n=20) groups based on Gold Coast criteria.
Assessed clinical data, motor evoked potentials (MEPs), serum biomarkers (cardiac Troponin T, neurofilament light chain, GFAP, BDNF), and brain imaging (cortical thickness, white matter volume).
Analyzed survival using Kaplan-Meier method.

Main Results:

Broadly similar clinical features, disease progression, and biomarker profiles were observed between groups.
Abnormal MEPs were more frequent in ALSwUMN (94.0%) than ALSwoUMN (63.2%).
Both groups showed cortical thinning; ALSwUMN had additional brainstem atrophy, while ALSwoUMN showed distinct cortical thinning patterns. No significant survival difference was found.

Conclusions:

ALSwoUMN patients exhibit comparable clinical profiles and outcomes to ALSwUMN patients.
Minor electrophysiological and imaging differences do not significantly alter prognosis.
Findings support the inclusion of ALSwoUMN within the ALS spectrum under the Gold Coast criteria.