Abstract
Cotton wool plaques (CWP) are large, ball-like plaques lacking dense amyloid cores that displace adjacent structures. They were first described in a Finnish kindred with early-onset Alzheimer disease (AD) with spastic paraparesis due to a presenilin-I Δ9 mutation. We describe a case of sporadic late-onset AD with numerous neocortical CWP as well as severe amyloid angiopathy and marked leukoencephalopathy, compared with 16 cases of late-onset AD with similar degrees of amyloid angiopathy and leukoencephalopathy. The cases were studied with histologic methods and with single and double immunostaining for beta-amyloid (Aβ), paired helical filaments-tau (PHF-tau), neurofilament (NF), glial fibrillary acidic protein (GFAP), HLA-DR, and amyloid precursor protein (APP). We found that CWP were well-circumscribed amyloid deposits infiltrated by ramified microglia and surrounded by dystrophic neurites that were immunopositive for APP, but only weakly for NF and PHF-tau. Aβ1-12 was diffuse throughout the CWP, while Aβ37-42 was peripherally located and Aβ20-40 more centrally located. Two of the 16 late-onset AD cases also had CWP, but they were also admixed with diffuse plaques and plaques with dense amyloid cores. Pyramidal tract degeneration was not a consistent finding or a prominent feature in any case. The results suggest that CWP are not specific for early-onset familial AD with spastic paraparesis.
Original language | English (US) |
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Pages (from-to) | 1051-1061 |
Number of pages | 11 |
Journal | Journal of Neuropathology and Experimental Neurology |
Volume | 60 |
Issue number | 11 |
DOIs | |
State | Published - 2001 |
Keywords
- Alzheimer disease
- Amyloid angiopathy
- Cotton wool plaques
- Leukoencephalopathy
- Presenilin
- Spastic paraparesis
ASJC Scopus subject areas
- General Medicine