BACKGROUND: Neurofibrillary tangles and beta-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.
BACKGROUND: Neurofibrillary tangles and beta-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.
Authors: D Neary; J S Snowden; L Gustafson; U Passant; D Stuss; S Black; M Freedman; A Kertesz; P H Robert; M Albert; K Boone; B L Miller; J Cummings; D F Benson Journal: Neurology Date: 1998-12 Impact factor: 9.910
Authors: Norman L Foster; Judith L Heidebrink; Christopher M Clark; William J Jagust; Steven E Arnold; Nancy R Barbas; Charles S DeCarli; R Scott Turner; Robert A Koeppe; Roger Higdon; Satoshi Minoshima Journal: Brain Date: 2007-08-18 Impact factor: 13.501
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Authors: Alberto Jaramillo-Jimenez; Lasse M Giil; Diego A Tovar-Rios; Miguel Germán Borda; Daniel Ferreira; Kolbjørn Brønnick; Ketil Oppedal; Dag Aarsland Journal: Front Neurol Date: 2021-07-07 Impact factor: 4.003