Alzheimer abnormalities of the amygdala with Klüver-Bucy syndrome symptoms: an amygdaloid variant of Alzheimer disease.
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.


OBJECTIVE
To describe an atypical behavioral presentation of Alzheimer disease and to review the literature on the subject.


METHODS
Case study.


METHODS
Outpatient specialty clinic.


METHODS
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.


METHODS
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.
Kile
Shawn J
SJ
Department of Neurology, University of California at Davis, 4860 Y St, Ste 3700, Sacramento, CA 95817, USA.
Ellis
William G
WG
Olichney
John M
JM
Farias
Sarah
S
DeCarli
Charles
C
D000368:Aged; D000544:Alzheimer Disease / Q000150:complications* / Q000473:pathology* / Q000503:physiopathology; D000679:Amygdala / Q000000981:diagnostic imaging / Q000473:pathology* / Q000503:physiopathology; D001344:Autopsy; D003704:Dementia / Q000175:diagnosis; D003937:Diagnosis, Differential; D003951:Diagnostic Errors / Q000517:prevention & control; D018450:Disease Progression; D017809:Fatal Outcome; D006801:Humans; D020232:Kluver-Bucy Syndrome / Q000209:etiology* / Q000473:pathology* / Q000503:physiopathology; D008279:Magnetic Resonance Imaging; D008297:Male; D016874:Neurofibrillary Tangles / Q000473:pathology; D009483:Neuropsychological Tests; D058225:Plaque, Amyloid / Q000473:pathology; D049268:Positron-Emission Tomography; D012189:Retrospective Studies