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Multiple system atrophy with severe involvement of the motor cortical areas and cerebral white matter
K Wakabayashi1, T Ikeuchi, A Ishikawa
1Brain Disease Research Centre, Brain Research Institute, Niigata University, Japan. koichi@bri.niigata-u.ac.jp
Abstract:
We report multiple system atrophy (MSA) of 14 years' duration in a 75-year-old woman. Postmortem examination revealed pathological changes typical of MSA. Furthermore, neuronal loss with astrocytosis in the primary motor and premotor cortices, especially in the fifth and sixth layers, and extensive myelin and axonal loss in the frontal and parietal white matter were evident. There were numerous ubiquitin-positive oligodendroglial inclusions, which are characteristic of MSA, in these cortical and white matter lesions. These findings suggest that the motor cortical areas and cerebral white matter are sites of significant involvement in the MSA disease process and that inclusion-bearing oligodendroglial alterations contribute to the white matter degeneration.
Insights
Multiple System Atrophy (MSA) involves significant motor cortex and white matter degeneration, characterized by neuronal loss and ubiquitin-positive inclusions. These pathological changes highlight key areas affected in this neurodegenerative disease.
Area of Science:
- Neuroscience
- Neuropathology
Background:
- Multiple System Atrophy (MSA) is a progressive neurodegenerative disorder.
- Understanding the specific pathological changes in MSA is crucial for diagnosis and treatment.
Observation:
- A 75-year-old woman with a 14-year history of MSA underwent postmortem examination.
- Pathological examination revealed characteristic features of MSA.
Findings:
- Significant neuronal loss and astrocytosis were observed in the primary motor and premotor cortices, particularly in deeper layers.
- Extensive myelin and axonal loss were noted in the frontal and parietal white matter.
- Numerous ubiquitin-positive oligodendroglial inclusions, a hallmark of MSA, were present in affected cortical and white matter areas.
Implications:
- The motor cortex and cerebral white matter are significantly impacted in the MSA disease process.
- Oligodendroglial inclusions contribute to white matter degeneration in MSA.
- These findings enhance our understanding of MSA neuropathology.