Blood–spinal cord barrier breakdown and pericyte reductions in amyotrophic lateral sclerosis

Acta Neuropathol, 2013 · DOI: 10.1007/s00401-012-1039-8 · Published: September 1, 2012

Simple Explanation

The blood–brain barrier and blood–spinal cord barrier (BSCB) limit the entry of plasma components and erythrocytes into the central nervous system (CNS). Pericytes play a key role in maintaining blood–CNS barriers. The BSCB is damaged in patients with amyotrophic lateral sclerosis (ALS).

Study Duration

Not specified

Participants

11 ALS patients and 5 non-neurodegenerative disorders controls

Evidence Level

Level 3: Retrospective Human Tissue Analysis

Key Findings

1
ALS patients exhibit perivascular deposits of erythrocyte-derived hemoglobin and hemosiderin in the cervical spinal cord.
2
Spinal cord parenchymal accumulation of plasma-derived immunoglobulin G, ﬁbrin and thrombin was demonstrated in ALS, but not controls.
3
ALS patients showed a significant reduction in pericyte number in the spinal cord compared to controls.

Research Summary

This study demonstrates BSCB disruption in ALS patients, leading to erythrocyte extravasation and accumulation of erythrocyte-derived products in the spinal cord. The research also found an association between BSCB damage and a reduction in pericyte populations in human ALS spinal cord tissue specimens. These findings suggest that vascular dysfunction may play a significant role in ALS pathogenesis.

Practical Implications

Diagnostic Potential

Detection of BSCB disruption and pericyte loss could serve as potential biomarkers for ALS diagnosis or disease progression.

Therapeutic Targets

Targeting vascular dysfunction and promoting BSCB integrity and pericyte survival may offer new therapeutic avenues for ALS.

MRI Interpretation

MRI studies in ALS patients should be interpreted with caution due to the small size of spinal cord hemosiderin deposits, which may limit the sensitivity and specificity of conventional T2*-weighted MRI imaging pulse sequences.

Study Limitations

1
Post-mortem tissue analysis may reflect end-stage processes.
2
The study acknowledges the relatively small sample size.
3
The study raises the question of whether similar findings occur in the motor cortex and affected brainstem motor nuclei, which remains to be seen.

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