Affiliation:
1. Department of Neurology National Hospital Organization Osaka Toneyama Medical Center Toyonaka Japan
2. Department of Neurology Higashiosaka City Medical Center Higashiosaka Japan
3. Department of Molecular Genetics, Brain Research Institute Niigata University Niigata Japan
4. Department of Neurology, Graduate School of Medicine Osaka University Suita Japan
Abstract
We report a case of argyrophilic grain disease (AGD) with unique clinical and pathological presentations. A 52‐year‐old man presented with spastic quadriparesis, bulbar palsy, and mild cognitive decline. His condition deteriorated rapidly and he died of pneumonia three years from onset. Pathologically, neuronal degeneration was involved severely in the amygdala, ambient gyrus, midbrain tegmentum, and reticular formation. The neurons of the temporal lobe, cingulate gyrus, brainstem, and spinal gray matter were also lost moderately. There was diffuse 4‐repeat tau‐pathology with argyrophilic grains. There were pretangles, globose‐type neurofibrillary tangles, and coiled bodies in the cerebral cortices, basal ganglia, thalami, brainstem, and the spinal cord except for the cerebellar cortices. There was no pathologic mutation in MAPT.
Funder
Japan Agency for Medical Research and Development
Subject
Neurology (clinical),General Medicine,Pathology and Forensic Medicine
Cited by
1 articles.
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