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Alzheimer's disease with spastic paresis and cotton wool type plaques
Author(s) -
Tabira Takeshi,
Chui De Hua,
Nakayama Hiroshi,
Kuroda Shigetoshi,
Shibuya Makoto
Publication year - 2002
Publication title -
journal of neuroscience research
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.72
H-Index - 160
eISSN - 1097-4547
pISSN - 0360-4012
DOI - 10.1002/jnr.10392
Subject(s) - paresis , pathology , alzheimer's disease , amyloid (mycology) , basal ganglia , presenilin , medicine , disease , anatomy , neuroscience , central nervous system , biology , surgery
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 Aβ42, 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 Aβ42 may cause degeneration of the pyramidal tract and basal ganglia from an early stage of Alzheimer's disease. © 2002 Wiley‐Liss, Inc.