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Neuropathologic characteristics of familial Alzheimer disease : cotton wool plaques and neuronal migration

Research Project

Project/Area Number 16500226
Research Category

Grant-in-Aid for Scientific Research (C)

Allocation TypeSingle-year Grants
Section一般
Research Field Nerve anatomy/Neuropathology
Research InstitutionKeio University

Principal Investigator

TAKAO Masaki  Keio University, School of Medicine, Assistant Professor, 医学部, 講師 (50245487)

Project Period (FY) 2004 – 2006
Project Status Completed (Fiscal Year 2006)
Budget Amount *help
¥2,300,000 (Direct Cost: ¥2,300,000)
Fiscal Year 2006: ¥400,000 (Direct Cost: ¥400,000)
Fiscal Year 2005: ¥700,000 (Direct Cost: ¥700,000)
Fiscal Year 2004: ¥1,200,000 (Direct Cost: ¥1,200,000)
KeywordsAlzheimer disease / amyloid beta / tau / cotton wool plaque / senile plaque / neuronal migration / Presenilin 1 / Alzheimer病 / cotton wool plaque
Research Abstract

Cotton wool plaque (CWP) is a pathologic alteration initially reported in association with variant Alzheimer disease (early onset of dementia and spastic paraparesis) caused by a deletion of exon 9 in the Presenilin 1 (PSEN1) gene. The aim of this study is to report the results of clinical and neuropathologic characteristics of eight individuals affected with early onset of Alzheimer disease (EOAD) and 'cotton wool plaques (CWPs)' in association with mutations L166P, G217D, V261F, V261I, P264L and A431E in the Presenilin 1 (PSEN1) gene. In order to appraise the clinical characteristics of EOAD-CWPs, we analyzed several symptoms that include spastic paraparesis. In addition, the frequency of CWPs and neuritic plaques (NPs) was assessed using the semi quantification of the CERAD methodology in the multiple anatomical regions such as cerebral cortex, subcortical nucleus, cerebellum and brainstem. Each section was stained using hematoxylin and eosin, Bielschowsky and/or Bodian methods as w … More ell as immunolabeled using antibodies raised against Aβ, tau and α-synuclein. In addition, neuronal migration abnormalities were analyzed in the cerebral white matter. All cases were characterized clinically by early onset of dementia, however spastic paraparesis was only found in cases with L166P and V261F mutation. Neuropathologically, numerous CWPs were seen in the cerebral cortex, striatum and amygdala. The frequency of CWPs was significantly higher than that of NPs in five mutations (L166P, G217D, V261F, V261I, A431E). In some instances, abnormal neurons were seen in the cerebral white matter. Besides dementia, clinical presentation is heterogeneous and spastic paraparesis is not obligatory symptoms in EOAD-CWPs. The distribution of CWPs differs from that of NPs in which it extends to the striatum and midbrain. If CWPs are present, they are generally severest modality of plaques in the cerebrum. Our results may provide the information of clinical and neuropathologic heterogeneities of EOAD-CWPs. (297 words) Less

Report

(4 results)
  • 2006 Annual Research Report   Final Research Report Summary
  • 2005 Annual Research Report
  • 2004 Annual Research Report
  • Research Products

    (23 results)

All 2006 2005

All Journal Article (21 results) Book (2 results)

  • [Journal Article] Corticobasal degeneration as cause of progressive non-fluent aphasia : a clinical,radiological and pathological study of an autopsy case.2006

    • Author(s)
      Takao M, et al.
    • Journal Title

      Neuropathology 26・6

      Pages: 569-578

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2006 Final Research Report Summary
  • [Journal Article] 【認知症の動的神経病理】アルファシヌクレイノパチー2006

    • Author(s)
      高尾昌樹, Ghetti B
    • Journal Title

      臨床検査Brain Pathol 50・10

      Pages: 1130-1136

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2006 Final Research Report Summary
  • [Journal Article] Familial spastic paraplegia of two siblings - clinical and neuropathologic analysis2006

    • Author(s)
      Takao M, et al.
    • Journal Title

      Brain Pathol 16・S

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2006 Final Research Report Summary
  • [Journal Article] Presenilin1の新規S170F変異を伴う,20代発症のLewy小体を伴う家族性アルツハイマー病(抄訳,解説)2006

    • Author(s)
      高尾昌樹
    • Journal Title

      Brain & Nerve 15

      Pages: 5-5

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2006 Final Research Report Summary
  • [Journal Article] A case report of possible myotonic dystrophy without DM1 OR DM2 gene mutations. Clinical and pathologic analyses2006

    • Author(s)
      Takao M, et al.
    • Journal Title

      Brain Pathol 16・S

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2006 Final Research Report Summary
  • [Journal Article] ラクナ梗塞の病理2006

    • Author(s)
      高尾昌樹, 厚東篤生
    • Journal Title

      日本臨床 64増刊

      Pages: 134-138

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2006 Final Research Report Summary
  • [Journal Article] Corticobasal degeneration as cause of progressive non-fluent aphasia : a clinical, radiological and pathological study of an autopsy case.2006

    • Author(s)
      Takao M, et al.
    • Journal Title

      Neuropathology 26

      Pages: 569-578

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      2006 Final Research Report Summary
  • [Journal Article] Alpha-synucleinopathy2006

    • Author(s)
      Takao M, Ghetti B
    • Journal Title

      J Med Technology 50

      Pages: 1130-1136

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      2006 Final Research Report Summary
  • [Journal Article] Familial spastic paraplegia of two siblings - clinical and neuropathologic analysis2006

    • Author(s)
      Takao M, Akiyama H, Kaburagi H, Ogata K.
    • Journal Title

      Brain Pathol 16

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      2006 Final Research Report Summary
  • [Journal Article] Translation and comment for (Novel presenilin 1 mutation (S170F) causing Alzheimer disease with Lewy bodies in the third decade of life)2006

    • Author(s)
      Takao M.
    • Journal Title

      Brain & Nerve 15:5

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      2006 Final Research Report Summary
  • [Journal Article] A case report of possible myotonic dystrophy without DM1 OR DM2 gene mutations. Clinical and pathologic analyses2006

    • Author(s)
      Takao M, Sato H, Suzuki N, Nishino I, Yoshikawa T, Ishihara T.
    • Journal Title

      Brain Pathol 16

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      2006 Final Research Report Summary
  • [Journal Article] Pathology of lacunar infarction2006

    • Author(s)
      Takao M, Koto A.
    • Journal Title

      Nippon Rinsho 64 Suppl 8

      Pages: 134-8

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      2006 Final Research Report Summary
  • [Journal Article] FTDP-17 and Pick diease. In Diagnosis of Alzheimer Disease ed by Murayama S.2006

    • Author(s)
      Takao M, Ghetti B.
    • Pages
      187-198
    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      2006 Final Research Report Summary
  • [Journal Article] Corticobasal degeneration as cause of progressive non-fluent aphasia : a clinical, radiological and pathological study of an autopsy case.2006

    • Author(s)
      Takao M, et al.
    • Journal Title

      Neuropathology 26・6

      Pages: 569-578

    • Related Report
      2006 Annual Research Report
  • [Journal Article] 【認知症の動的神経病理】アルファシヌクレイノパチー2006

    • Author(s)
      高尾昌樹
    • Journal Title

      臨床検査 50・10

      Pages: 1130-1136

    • Related Report
      2006 Annual Research Report
  • [Journal Article] Familial spastic paraplegia of two siblings-clinical and neuropathologic analysis.2006

    • Author(s)
      Takao M, et al.
    • Journal Title

      Brain Pathol 16・S

      Pages: 64-64

    • Related Report
      2006 Annual Research Report
  • [Journal Article] Presenilin 1の新規S170F変異を伴う,20代発症のLewy小体を伴う家族性アルツハイマー病2006

    • Author(s)
      高尾昌樹
    • Journal Title

      Brain & Nerve 15

      Pages: 5-5

    • Related Report
      2006 Annual Research Report
  • [Journal Article] A case report of myopathy with unusual clinical and pathologic presentation.2006

    • Author(s)
      Sato H, Takao M et al.
    • Journal Title

      Neuropathology 26

    • Related Report
      2006 Annual Research Report
  • [Journal Article] Amino-terminally truncated Aβ peptide species are the main component of cotton wool plaques.2005

    • Author(s)
      Miravalle L, Calero M, Takao M et al.
    • Journal Title

      Biochemistry 44

      Pages: 10810-10821

    • Related Report
      2005 Annual Research Report
  • [Journal Article] Gerstmann-Straussler-Scheinker disease associated with the prnp a117v-129v mutation : neuropathology of an asymptomatic gene carrier and five clinically affected individuals from a family2005

    • Author(s)
      Takao M, Piccardo P, et al.
    • Journal Title

      J Neuropath Exp Neurol 64

      Pages: 433-433

    • Related Report
      2005 Annual Research Report
  • [Journal Article] Neuropathologic phenotype of Alzheimer disease associated with Presenilin 1 gene Y115C and M146L mutation2005

    • Author(s)
      Takao M, Murrell JR et al.
    • Journal Title

      Neuropathology 25

    • Related Report
      2005 Annual Research Report
  • [Book] FTDP-17とPick病 pp187-198(アルツハイマー病診断)2006

    • Author(s)
      高尾昌樹, Ghetti B
    • Total Pages
      234
    • Publisher
      真興交易出版
    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2006 Final Research Report Summary
  • [Book] FTDP-17とPick病 アルツハイマー病診断(村山繁雄編)2006

    • Author(s)
      高尾昌樹
    • Total Pages
      234
    • Publisher
      真興交易(株)医書出版部
    • Related Report
      2006 Annual Research Report

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Published: 2004-04-01   Modified: 2016-04-21  

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