Alzheimer abnormalities of the amygdala with Klüver-Bucy syndrome symptoms: An amygdaloid variant of Alzheimer disease

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Abstract

Background: Neurofibrillary tangles and β-amyloid plaques have been observed in the amygdala in Alzheimer disease. A disproportionate abundance of this abnormality in the amygdala may cause behavioral symptoms similar to Klüver-Bucy syndrome. Objectives: To describe an atypical behavioral presentation of Alzheimer disease and to review the literature on the subject. Design: Case study. Setting: Outpatient specialty clinic. Patient: A 70-year-old man with progressive behavioral symptoms of hyperorality, hypersexuality, hypermetamorphosis, visual agnosia, hyperphagia, and apathy who died at age 77 of asphyxiation on a foreign object. Main Outcome Measures: Clinical symptomatology, brain imaging, and neuropathology. Results: The pathologic diagnosis was Alzheimer disease with abundant tangles and plaques in the lateral amygdala. Conclusions: This case represents a variant of Alzheimer disease with prominent amygdala abnormalities and a Klüver-Bucy phenotype that was misdiagnosed as frontotemporal dementia. Clinical and imaging findings that may aid in accurate diagnosis are reviewed.

Original languageEnglish (US)
Pages (from-to)125-129
Number of pages5
JournalArchives of Neurology
Volume66
Issue number1
DOIs
StatePublished - Jan 2009

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Amygdala
Alzheimer Disease
Behavioral Symptoms
Agnosia
Apathy
Frontotemporal Dementia
Hyperphagia
Neurofibrillary Tangles
Asphyxia
Amyloid Plaques
Ambulatory Care Facilities
Foreign Bodies
Diagnostic Errors
Neuroimaging
Outcome Assessment (Health Care)
Phenotype
Alzheimer's Disease
Syndrome
Alzheimer

ASJC Scopus subject areas

  • Clinical Neurology

Cite this

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title = "Alzheimer abnormalities of the amygdala with Kl{\"u}ver-Bucy syndrome symptoms: An amygdaloid variant of Alzheimer disease",
abstract = "Background: Neurofibrillary tangles and β-amyloid plaques have been observed in the amygdala in Alzheimer disease. A disproportionate abundance of this abnormality in the amygdala may cause behavioral symptoms similar to Kl{\"u}ver-Bucy syndrome. Objectives: To describe an atypical behavioral presentation of Alzheimer disease and to review the literature on the subject. Design: Case study. Setting: Outpatient specialty clinic. Patient: A 70-year-old man with progressive behavioral symptoms of hyperorality, hypersexuality, hypermetamorphosis, visual agnosia, hyperphagia, and apathy who died at age 77 of asphyxiation on a foreign object. Main Outcome Measures: Clinical symptomatology, brain imaging, and neuropathology. Results: The pathologic diagnosis was Alzheimer disease with abundant tangles and plaques in the lateral amygdala. Conclusions: This case represents a variant of Alzheimer disease with prominent amygdala abnormalities and a Kl{\"u}ver-Bucy phenotype that was misdiagnosed as frontotemporal dementia. Clinical and imaging findings that may aid in accurate diagnosis are reviewed.",
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AU - Kile, Shawn J.

AU - Ellis, William G.

AU - Olichney, John M

AU - Tomaszewski Farias, Sarah E

AU - DeCarli, Charles

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N2 - Background: Neurofibrillary tangles and β-amyloid plaques have been observed in the amygdala in Alzheimer disease. A disproportionate abundance of this abnormality in the amygdala may cause behavioral symptoms similar to Klüver-Bucy syndrome. Objectives: To describe an atypical behavioral presentation of Alzheimer disease and to review the literature on the subject. Design: Case study. Setting: Outpatient specialty clinic. Patient: A 70-year-old man with progressive behavioral symptoms of hyperorality, hypersexuality, hypermetamorphosis, visual agnosia, hyperphagia, and apathy who died at age 77 of asphyxiation on a foreign object. Main Outcome Measures: Clinical symptomatology, brain imaging, and neuropathology. Results: The pathologic diagnosis was Alzheimer disease with abundant tangles and plaques in the lateral amygdala. Conclusions: This case represents a variant of Alzheimer disease with prominent amygdala abnormalities and a Klüver-Bucy phenotype that was misdiagnosed as frontotemporal dementia. Clinical and imaging findings that may aid in accurate diagnosis are reviewed.

AB - Background: Neurofibrillary tangles and β-amyloid plaques have been observed in the amygdala in Alzheimer disease. A disproportionate abundance of this abnormality in the amygdala may cause behavioral symptoms similar to Klüver-Bucy syndrome. Objectives: To describe an atypical behavioral presentation of Alzheimer disease and to review the literature on the subject. Design: Case study. Setting: Outpatient specialty clinic. Patient: A 70-year-old man with progressive behavioral symptoms of hyperorality, hypersexuality, hypermetamorphosis, visual agnosia, hyperphagia, and apathy who died at age 77 of asphyxiation on a foreign object. Main Outcome Measures: Clinical symptomatology, brain imaging, and neuropathology. Results: The pathologic diagnosis was Alzheimer disease with abundant tangles and plaques in the lateral amygdala. Conclusions: This case represents a variant of Alzheimer disease with prominent amygdala abnormalities and a Klüver-Bucy phenotype that was misdiagnosed as frontotemporal dementia. Clinical and imaging findings that may aid in accurate diagnosis are reviewed.

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