Non-Alzheimer dementia with status spongiosus and neuronal cells lossshowing unusual perineuronal structures and point mutation at 129codon of prion protein
M. Hayashi
Bok Engelsk 1997
| Utgitt | 1997
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| Omfang | Side 55- 59
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| Opplysninger | The subject presented with intellectual decline followed byprogressive muscle weakness of the bilateral upper limbs when he was60 years old. He had a point mutation (methionin-valine) at 129 prionprotein codon. He died at the age of 63 and necropsy revealedbilateral frontal lobe atrophy. The frontal cortex showed neuronalcell loss in layers II and III with spongiform change. Reusche silverimpregnation technique for beta-peptide combined with ubiquitinimmunostaining revealed perineuronal structures encirclingdegenerated neurons and ubiquitin-immunoreactive (IR) dot-likedeposits. They were distributed particularly in the temporalneocortex and entorhinal cortex. They differed from either classicsenile or diffuse plaque by the absence of amyloid core in the centerand of amyloid fibrils. Ubiquitin-IR materials were also found asneuronal inclusions in the hippocampal granular cells. Nigraldegeneration and neuronal loss in the hypoglossal nerve nucleus andin the anterior horn of the spinal cord were also found and spinalcord motoneurons had Bunina body inclusions. The clinical featuresand pathological findings were consistent with non-Alzheimer dementiawith status spongiosus and neuronal cell loss. The unusualperineuronal structures found in our case might be a specificcellular pathology of dementia of the frontal lobe type.
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| Emner |
