Dominantly inherited dementia and parkinsonism, with non-Alzheimer amyloid plaques: A new neurogenetic disorder

R. N. Rosenberg, J. B. Green, C. L. White, D. R. Sparkman, S. J. DeArmond, J. J. Kepes

Research output: Contribution to journalArticle

13 Citations (Scopus)

Abstract

A family is described in which a dominant form of inheritance, probably autosomal dominant, expresses severe dementia and parkinsonism as the major clinical features. Neuropathological correlates in two autopsied members of this family consisted of extracellular hyaline eosinophilic, congophilic amyloid plaques in decreasing order of frequency in the cerebral cortex, basal ganglia, thalamus, and substantia nigra, and atrophy and gliosis of the basal ganglia and substantia nigra. The extracellular plaques did not stain with antibody raised against the prion protein nor with two separate anti-amyloid A4 antibodies. The combination of dominantly inherited dementia with parkinsonism and extracellular plaques in this distribution that are amyloid and prion protein antibody negative has not been previously reported and thus may represent a new neurological genetic disorder.

Original languageEnglish (US)
Pages (from-to)152-158
Number of pages7
JournalAnnals of Neurology
Volume25
Issue number2
StatePublished - 1989

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Parkinsonian Disorders
Substantia Nigra
Basal Ganglia
Dementia
Antibodies
Amyloidogenic Proteins
Inborn Genetic Diseases
Hyalin
Gliosis
Amyloid Plaques
Nervous System Diseases
Thalamus
Amyloid
Cerebral Cortex
Atrophy
Coloring Agents
Dementia-Parkinsonism With Non-Alzheimer Amyloid Plaques
Prion Proteins

ASJC Scopus subject areas

  • Neuroscience(all)

Cite this

Dominantly inherited dementia and parkinsonism, with non-Alzheimer amyloid plaques : A new neurogenetic disorder. / Rosenberg, R. N.; Green, J. B.; White, C. L.; Sparkman, D. R.; DeArmond, S. J.; Kepes, J. J.

In: Annals of Neurology, Vol. 25, No. 2, 1989, p. 152-158.

Research output: Contribution to journalArticle

Rosenberg, R. N. ; Green, J. B. ; White, C. L. ; Sparkman, D. R. ; DeArmond, S. J. ; Kepes, J. J. / Dominantly inherited dementia and parkinsonism, with non-Alzheimer amyloid plaques : A new neurogenetic disorder. In: Annals of Neurology. 1989 ; Vol. 25, No. 2. pp. 152-158.
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