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Asymmetrical temporal lobe atrophy with massive neuronal inclusions in multiple system atrophy
K Shibuya1, H Nagatomo, K Iwabuchi
1Department of Neurology and Psychiatry, Numazu Central Hospital, Nakasecho 24-1, Numazu, 410-8575, Shizuoka, Japan.
Journal of the Neurological Sciences
|October 31, 2000
Summary
This study reports a rare case of multiple system atrophy (MSA) with asymmetrical temporal lobe atrophy and unique neuronal inclusions. This finding suggests a potential new variant of MSA with distinct pathological features.
Area of Science:
- Neuropathology
- Neurodegenerative Diseases
Background:
- Multiple System Atrophy (MSA) is a rare neurodegenerative disorder.
- Olivopontocerebellar atrophy (OPCA) is a subtype of MSA characterized by cerebellar and parkinsonian symptoms.
- Temporal lobe atrophy is not a typical feature of MSA.
Observation:
- A 53-year-old Japanese woman presented with cerebellar ataxia, parkinsonism, and early cognitive decline (forgetfulness, disorientation).
- Autopsy revealed MSA with asymmetrical temporal lobe atrophy.
- Intraneuronal globular inclusions were found predominantly in the hippocampus and dentate fascia.
Findings:
- The neuronal inclusions were ubiquitin-positive and argyrophilic, but negative for neurofilament (NF), tau, and paired helical filaments (PHF).
- Ultrastructural analysis showed filamentous structures, ribosome-like granules, mitochondria, and lipofuscin.
- These inclusions differ from Pick bodies, suggesting a distinct pathological entity.
Implications:
- This case represents a rare association of MSA with asymmetrical temporal lobe atrophy and unique intraneuronal inclusions.
- The distinct immunohistochemical and ultrastructural features of the inclusions suggest a potential new variant of MSA.
- Further research is needed to understand the pathogenesis and clinical significance of this MSA variant.