TY - JOUR
T1 - Alzheimer abnormalities of the amygdala with Klüver-Bucy syndrome symptoms
T2 - An amygdaloid variant of Alzheimer disease
AU - Kile, Shawn J.
AU - Ellis, William G.
AU - Olichney, John M
AU - Tomaszewski Farias, Sarah E
AU - DeCarli, Charles
PY - 2009/1
Y1 - 2009/1
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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U2 - 10.1001/archneurol.2008.517
DO - 10.1001/archneurol.2008.517
M3 - Article
C2 - 19139311
AN - SCOPUS:58449131941
VL - 66
SP - 125
EP - 129
JO - Archives of Neurology
JF - Archives of Neurology
SN - 0003-9942
IS - 1
ER -