Alzheimer Abnormalities of the Amygdala With Klüver-Bucy Syndrome Symptoms: An Amygdaloid Variant of Alzheimer Disease


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Abstract

BackgroundNeurofibrillary 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.ObjectivesTo describe an atypical behavioral presentation of Alzheimer disease and to review the literature on the subject.DesignCase study.SettingOutpatient specialty clinic.PatientA 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 MeasuresClinical symptomatology, brain imaging, and neuropathology.ResultsThe pathologic diagnosis was Alzheimer disease with abundant tangles and plaques in the lateral amygdala.ConclusionsThis 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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