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Alpha-synuclein in motor neuron disease: an immunohistologic study.
M J Doherty1, T D Bird, J B Leverenz
1Department of Neurology, University of Washington, Seattle, Washington, USA.
Acta Neuropathologica
|December 3, 2003
Summary
Alpha-synuclein (ASN) abnormalities are present in motor neuron disorders (MND), including amyotrophic lateral sclerosis (ALS). Increased ASN staining was found in brain and spinal tissues, suggesting a potential role in MND pathogenesis.
Area of Science:
- Neuroscience
- Neuropathology
Background:
- Alpha-synuclein (ASN) is linked to Lewy body disorders like Parkinson's disease.
- Lewy body-like inclusions and potential ASN abnormalities have been reported in amyotrophic lateral sclerosis (ALS).
Purpose of the Study:
- To investigate alpha-synuclein (ASN) immunoreactivity in spinal and brain tissues of individuals with progressive motor neuron disorders (MND).
Main Methods:
- Tissue samples from MND patients and controls were stained for ASN.
- A blinded, semiquantitative analysis assessed ASN staining for spheroids, neuronal inclusions, glial staining, and white matter changes.
- Clinical records were reviewed to confirm diagnoses based on El-Escariol Criteria for ALS.
Main Results:
- MND cases showed significantly increased ASN staining in spheroids and glial cells in both gray and white matter.
- Abnormal ASN staining of corticospinal tract axonal fibers and myelin was also significant.
- No significant difference in the detection of ASN-positive neuronal inclusions was observed between groups.
Conclusions:
- Significant alpha-synuclein (ASN) abnormalities are present in motor neuron disorders (MND).
- These findings suggest a potential role for ASN in the pathogenesis of MND, although the exact association remains unclear.