Alzheimer's disease with spastic paresis and cotton wool type plaques.
We reviewed Alzheimer's cases with spastic paresis and cotton wool type plaques in five Japanese and nine Caucasian cases. Most were early onset familial Alzheimer's disease with presenilin 1 mutations. The cotton wool type plaques were related to extremely high production of A beta 42, due mainly to presenilin 1 mutations and low immune responses. Cotton wool plaques were numerous in the entire central nervous system, including basal ganglia, brainstem and even in spinal cord. Cotton wool type plaques were composed of slightly electron dense synaptic structures, but amyloid fibrils were rarely found. Such a high accumulation of A beta 42 may cause degeneration of the pyramidal tract and basal ganglia from an early stage of Alzheimer's disease.
Tabira
Takeshi
T
National Institute for Longevity Sciences, Obu, Aichi, Japan. tabira@nils.go.jp
Chui
De Hua
DH
Nakayama
Hiroshi
H
Kuroda
Shigetoshi
S
Shibuya
Makoto
M
D000328:Adult; D000544:Alzheimer Disease / Q000150:complications* / Q000235:genetics / Q000473:pathology*; D016229:Amyloid beta-Peptides / Q000032:analysis / Q000235:genetics; D002490:Central Nervous System / Q000473:pathology* / Q000503:physiopathology; D005260:Female; D006801:Humans; D008297:Male; D008565:Membrane Proteins / Q000172:deficiency / Q000235:genetics; D008875:Middle Aged; D009154:Mutation / Q000235:genetics; D020336:Paraparesis, Spastic / Q000235:genetics* / Q000473:pathology* / Q000503:physiopathology; D010446:Peptide Fragments / Q000032:analysis / Q000235:genetics; D058225:Plaque, Amyloid / Q000235:genetics / Q000473:pathology*; D053764:Presenilin-1
D016229:Amyloid beta-Peptides; D008565:Membrane Proteins; C508853:PSEN1 protein, human; D010446:Peptide Fragments; D053764:Presenilin-1; C075222:amyloid beta-protein (1-42)