Arch Neurol. 2009 Jan;66(1):125-9. doi: 10.1001/archneurol.2008.517.Alzheimer abnormalities of the amygdala with Klüver-Bucy syndrome symptoms: an amygdaloid variant of Alzheimer disease.Kile SJ1, Ellis WG, Olichney JM, Farias S, DeCarli C.Author information 1Department of Neurology, University of California at Davis,, Arch Neurol. 2009 Jan;, 2009 Jan 20
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.