Acta Neuropathologica

, Volume 106, Issue 4, pp 348–356

Variability and heterogeneity in Alzheimer's disease with cotton wool plaques: a clinicopathological study of four autopsy cases

Authors

    • Department of NeuropsychiatryOkayama University Graduate School of Medicine and Dentistry
  • Seishi Terada
    • Department of NeuropsychiatryOkayama University Graduate School of Medicine and Dentistry
  • Hideki Ishizu
    • Department of NeuropsychiatryOkayama University Graduate School of Medicine and Dentistry
    • Department of Laboratory MedicineZikei Institute of Psychiatry
  • Hiroshi Ujike
    • Department of NeuropsychiatryOkayama University Graduate School of Medicine and Dentistry
  • Takeshi Ishihara
    • Department of NeuropsychiatryOkayama University Graduate School of Medicine and Dentistry
  • Masuyuki Namba
    • Department of Laboratory MedicineZikei Institute of Psychiatry
  • Yasuaki Hayashi
    • Department of NeurologyNational Okayama Medical Center
  • Tetsuya Nishinaka
    • Okayama Prefectural Hospital
  • Reiko Namba
    • Clinical Research Institute and Department of NeurologyNational Minami-Okayama Hospital
  • Hanae Nakashima
    • Department of NeuropsychiatryOkayama University Graduate School of Medicine and Dentistry
  • Kenji Uéda
    • Department of Neural PlasticityTokyo Institute of Psychiatry
  • Frédéric Checler
    • Institut de Pharmacologie Moléculaire et Cellulaire, UMR6097, Centre National de la Recherche ScientifiqueUniverisité de Nice-Sophia Antipolis
  • Shigetoshi Kuroda
    • Department of NeuropsychiatryOkayama University Graduate School of Medicine and Dentistry
Regular Paper

DOI: 10.1007/s00401-003-0737-7

Cite this article as:
Yokota, O., Terada, S., Ishizu, H. et al. Acta Neuropathol (2003) 106: 348. doi:10.1007/s00401-003-0737-7

Abstract

We describe three cases of early- (cases 1–3, 28–39 years) and one of late-onset (case 4, 76 years) Alzheimer's disease (AD) with 'cotton wool' plaques (CWPs) but without a family history indicating autosomal dominant inheritance. The early-onset cases, but not the late-onset case, showed remarkable aggression, disinhibition, and impulsiveness. Spastic paraparesis was observed in only one early-onset case. Hematoxylin-eosin-stained sections showed numerous CWPs, especially in the temporal cortex, in all cases. Bielschowsky-stained sections showed neurofibrillary tangles and minor neuritic changes surrounding the CWPs in three cases, but not in case 2. Gallyas-Braak-stained sections showed weak argyrophilia in homogeneous material of the CWPs in cases 2 and 4. Quantitative analysis demonstrated that Aβ42 was deposited more predominantly than Aβ40 in three cases. However, in case 2, approximately twice as much Aβ40 as Aβ42 was deposited. Tau immunostaining demonstrated neuritic changes in three cases, but not in case 2. α-Synuclein-positive Lewy bodies (LBs) and astrocytic lesions containing non-Aβ component of AD amyloid (NAC), a central fragment of α-synuclein, were found in case 3. In conclusion, (1) a frontal lobe syndrome-like personality change may be one of the characteristic clinical features of early-onset CWP-AD, (2) the deposition pattern of Aβ40 and Aβ42 in CWP-AD is more variable than that of presenilin-1-linked cases, (3) Aβ deposition can result in development of dementia without tau pathology, and (4) CWP-AD with LBs and several other neurodegenerative disorders with LBs share a common process involving α-synuclein and NAC deposition.

Keywords

Alzheimer's diseaseα-SynucleinCotton wool plaqueTau

Copyright information

© Springer-Verlag 2003