Primary angiitis of the central nervous system and Alzheimer's disease

Clinically and pathologically evident in a single patient

Research output: Contribution to journalArticle

Abstract

Two years after being successfully treated for biopsy confirmed primary angiitis of the central nervous system (PACNS), a 69-year-old woman presented with cognitive decline. In contrast to her first presentation, her altered mental function developed gradually, was not associated with headache or abnormal cerebrospinal fluid analysis, and did not improve, with immunosuppression. Reevaluation of her original brain biopsy not only confirmed the presence of PACNS, but also revealed neuritic plaques and neurofibrillary tangles, suggesting a concurrent diagnosis of Alzheimer's disease. Cerebral angiogram did not suggest vasculitis and magnetic resonance imaging showed generalized cerebral atrophy supporting the diagnosis of Alzheimer's. This case illustrates that Alzheimer's dementia and PACNS can coexist in a single patient and that Alzheimer's disease should be considered when a patient with successfully treated PACNS presents with cognitive decline months or years after initial diagnosis.

Original languageEnglish (US)
Pages (from-to)2935-2937
Number of pages3
JournalJournal of Rheumatology
Volume27
Issue number12
StatePublished - 2000

Fingerprint

Central Nervous System Diseases
Alzheimer Disease
Biopsy
Neurofibrillary Tangles
Amyloid Plaques
Vasculitis
Immunosuppression
Atrophy
Headache
Cerebrospinal Fluid
Angiography
Magnetic Resonance Imaging
Primary angiitis of the central nervous system
Brain
Cognitive Dysfunction

Keywords

  • Alzheimer's disease
  • Amyloid angiopathy
  • Granulomatous vasculitis
  • Primary angiitis of the central nervous system

ASJC Scopus subject areas

  • Rheumatology
  • Immunology

Cite this

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title = "Primary angiitis of the central nervous system and Alzheimer's disease: Clinically and pathologically evident in a single patient",
abstract = "Two years after being successfully treated for biopsy confirmed primary angiitis of the central nervous system (PACNS), a 69-year-old woman presented with cognitive decline. In contrast to her first presentation, her altered mental function developed gradually, was not associated with headache or abnormal cerebrospinal fluid analysis, and did not improve, with immunosuppression. Reevaluation of her original brain biopsy not only confirmed the presence of PACNS, but also revealed neuritic plaques and neurofibrillary tangles, suggesting a concurrent diagnosis of Alzheimer's disease. Cerebral angiogram did not suggest vasculitis and magnetic resonance imaging showed generalized cerebral atrophy supporting the diagnosis of Alzheimer's. This case illustrates that Alzheimer's dementia and PACNS can coexist in a single patient and that Alzheimer's disease should be considered when a patient with successfully treated PACNS presents with cognitive decline months or years after initial diagnosis.",
keywords = "Alzheimer's disease, Amyloid angiopathy, Granulomatous vasculitis, Primary angiitis of the central nervous system",
author = "Daniel Brotman and Eberhart, {Charles G} and Burger, {P. C.} and McArthur, {Justin Charles} and Hellmann, {David B}",
year = "2000",
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T2 - Clinically and pathologically evident in a single patient

AU - Brotman, Daniel

AU - Eberhart, Charles G

AU - Burger, P. C.

AU - McArthur, Justin Charles

AU - Hellmann, David B

PY - 2000

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N2 - Two years after being successfully treated for biopsy confirmed primary angiitis of the central nervous system (PACNS), a 69-year-old woman presented with cognitive decline. In contrast to her first presentation, her altered mental function developed gradually, was not associated with headache or abnormal cerebrospinal fluid analysis, and did not improve, with immunosuppression. Reevaluation of her original brain biopsy not only confirmed the presence of PACNS, but also revealed neuritic plaques and neurofibrillary tangles, suggesting a concurrent diagnosis of Alzheimer's disease. Cerebral angiogram did not suggest vasculitis and magnetic resonance imaging showed generalized cerebral atrophy supporting the diagnosis of Alzheimer's. This case illustrates that Alzheimer's dementia and PACNS can coexist in a single patient and that Alzheimer's disease should be considered when a patient with successfully treated PACNS presents with cognitive decline months or years after initial diagnosis.

AB - Two years after being successfully treated for biopsy confirmed primary angiitis of the central nervous system (PACNS), a 69-year-old woman presented with cognitive decline. In contrast to her first presentation, her altered mental function developed gradually, was not associated with headache or abnormal cerebrospinal fluid analysis, and did not improve, with immunosuppression. Reevaluation of her original brain biopsy not only confirmed the presence of PACNS, but also revealed neuritic plaques and neurofibrillary tangles, suggesting a concurrent diagnosis of Alzheimer's disease. Cerebral angiogram did not suggest vasculitis and magnetic resonance imaging showed generalized cerebral atrophy supporting the diagnosis of Alzheimer's. This case illustrates that Alzheimer's dementia and PACNS can coexist in a single patient and that Alzheimer's disease should be considered when a patient with successfully treated PACNS presents with cognitive decline months or years after initial diagnosis.

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