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An Amygdaloid Variant of Alzheimer Disease

Shawn J. Kile, MD; William G. Ellis, MD; John M. Olichney, MD; Sarah Farias, PhD; Charles DeCarli, MD

Arch Neurol. 2009;66(1):125-129.

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.

Author Affilations: Department of Neurology, University of California at Davis, Sacramento.

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