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Updated: Jun 21, 2026

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

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

Published on: March 4, 2014

Thymic involution, a co-morbidity factor in amyotrophic lateral sclerosis.

Akop Seksenyan¹, Noga Ron-Harel, David Azoulay

¹Maxine-Dunitz Neurosurgical Institute, Cedars-Sinai Medical Center, Los Angeles, CA, USA.

Journal of Cellular and Molecular Medicine

|August 5, 2009

Summary

Amyotrophic lateral sclerosis (ALS) is linked to T-cell malfunction. Studies show reduced thymic function and T-cell activity in ALS patients and mouse models, suggesting a potential therapeutic target.

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Area of Science:

Neuroimmunology
Immunology
Cellular Biology

Background:

Amyotrophic lateral sclerosis (ALS) involves rapid motor neuron loss.
CD4(+) T cells are crucial for central nervous system maintenance and repair.
Previous research indicates a protective role for CD4(+) T cells in ALS mouse models.

Purpose of the Study:

To investigate the hypothesis that T-cell malfunction occurs in parallel with motor neuron dysfunction in ALS.
To assess thymic function as a measure of peripheral T-cell availability in ALS models and patients.

Main Methods:

Assessed thymic function in mSOD1 (G93A) mice and human ALS patients.
Measured T-cell receptor rearrangement excision circles (TRECs) in blood.
Analyzed thymic progenitor-cell content and histology in mice.
Examined gene expression and apoptosis markers in peripheral blood mononuclear cells (PBMCs).

Main Results:

Reduced thymic progenitor cells and abnormal thymic histology were observed in mSOD1 mice.
ALS patients showed decreased thymic output (reduced TRECs) and a restricted T-cell repertoire.
Increased pro-apoptotic BAX/BCL2 ratio and reduced expression of T-cell activity genes (CD80, CD86, IFNG, IL18) were found in ALS patient PBMCs.

Conclusions:

Thymic dysfunction and T-cell abnormalities are present in both human ALS patients and an animal model.
These immune system defects may be co-pathological factors in ALS, irrespective of the underlying cause.
Findings suggest potential therapeutic strategies targeting thymic defects and T-cell deficiencies in ALS treatment.