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Cerebral white matter tau-positive granular glial pathology as a characteristic pathological feature in long
Taku Homma1, Yoko Mochizuki2, Shinsuke Tobisawa3
1Department of Neuropathology, Tokyo Metropolitan Neurological Hospital, Fuchu, Tokyo, Japan; Department of Pathology, Saitama Medical University International Medical Center, Hidaka, Saitama, Japan; Division of Human Pathology, Department of Pathology and Microbiology, Nihon University School of Medicine, Itabashi, Tokyo, Japan.
Introduction:
It is unclear whether tau-positive granular glial pathology is a characteristic feature of MSA. We aimed to analyse the prevalence and significance of tau-positive granular glial pathology in MSA.
Methods:
Fourteen MSA cases were clinicopathologically investigated, focusing on tau-positive granular glial pathology in the frontal and temporal white matter and putamen.
Results:
In five MSA cases, the temporal white matter showed AT8-positive granular glial pathology; this pathology was detected in the frontal white matter in three cases. AT8-positive granular glia in the white matter were associated with long disease duration with long-term tube feeding and/or long-term tracheotomy. Alpha-synuclein-positive glial cytoplasmic inclusion intensity was not associated with AT8-positive granular glial pathology. The tau isoform of AT8-positive granular glia in the cerebral white matter exhibited three-repeat, not four-repeat, tau. Ten MSA patients showed tau-positive granular glial pathology in the putamen; the tau isoform was predominantly three-repeat tau and four-repeat tau in cases with disease duration ≥13 years and < 13 years, respectively.
Conclusions:
Tau-positive granular glia in the putamen is a characteristic pathological feature of MSA. Tau-positive granular glia appear in the cerebral white matter in MSA patients and are associated with long disease duration with long-term tube feeding and/or long-term tracheotomy.
Insights
Tau-positive granular glial pathology is a characteristic feature in the putamen of Multiple System Atrophy (MSA) cases. This pathology also appears in the white matter of MSA patients, correlating with longer disease duration.
Area of Science:
- Neuropathology
- Neurodegenerative Diseases
Background:
- The presence of tau-positive granular glial pathology in Multiple System Atrophy (MSA) remains unclear.
- Investigating this pathology is crucial for understanding MSA's diverse pathological features.
Purpose of the Study:
- To determine the prevalence and significance of tau-positive granular glial pathology in MSA.
- To analyze the tau isoform composition and clinical correlations of this pathology.
Main Methods:
- Clinicopathological investigation of fourteen MSA cases.
- Focus on tau-positive granular glial pathology in the frontal and temporal white matter and putamen.
- Immunohistochemical analysis for tau (AT8) and alpha-synuclein.
Main Results:
- Tau-positive granular glial pathology was found in the putamen of ten MSA cases and in the white matter of five cases.
- White matter pathology correlated with longer disease duration, long-term tube feeding, and/or tracheotomy.
- Predominantly three-repeat tau was observed in white matter granular glia, while putaminal glia showed a mix of three-repeat and four-repeat tau, dependent on disease duration.
Conclusions:
- Tau-positive granular glia in the putamen is a characteristic pathological feature of MSA.
- Tau-positive granular glia in the cerebral white matter of MSA patients is associated with prolonged disease duration and supportive care.
- The tau isoform composition varies between white matter and putamen, and with disease duration.
