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Sensory nerve pathology in amyotrophic lateral sclerosis
T Heads1, M Pollock, A Robertson
1Department of Medicine, University of Otago Medical School, Dunedin, New Zealand.
Acta Neuropathologica
|January 1, 1991
Summary
Amyotrophic lateral sclerosis (ALS) involves sensory nerve pathology, particularly axonal atrophy and demyelination, which worsens with disease duration. This suggests a broader metabolic issue affecting both motor and sensory neurons in ALS patients.
Area of Science:
- Neurology
- Neuroscience
- Pathology
Background:
- Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease primarily affecting motor neurons.
- Sensory nerve involvement in ALS is not fully understood, necessitating detailed morphometric analysis.
Purpose of the Study:
- To assess the consistency and characteristics of sensory nerve pathology in ALS.
- To investigate the correlation between peripheral nerve pathology severity and disease duration in ALS.
- To explore the underlying mechanisms of sensory neuron involvement in ALS.
Main Methods:
- Detailed morphometric analysis of sural nerve biopsies from ALS patients.
- Evaluation of axonal atrophy, myelination status, and fiber diameter distribution.
- Assessment of sodium pump concentration in peripheral nerves.
Main Results:
- Consistent evidence of early axonal atrophy and increased remyelination in ALS sural nerve biopsies.
- A shift towards smaller fiber diameters in the nerve fiber population.
- Significant correlation between the severity of sensory nerve pathology and ALS disease duration.
- Normal peripheral nerve sodium pump concentration in ALS patients.
Conclusions:
- ALS involves an ingravescent dorsal root ganglion neuronopathy affecting large neurons early on.
- Progressive axonal atrophy, secondary demyelination-remyelination, and nerve fiber degeneration are key features.
- Parallel motor and sensory neuron involvement suggests a widespread metabolic disturbance in ALS.