Mesiotemporal Disconnection and Hypoactivity in Klüver-Bucy Syndrome: Case Series and Literature Review

Objective: Klüver-Bucy syndrome (KBS) is often perceived as rare and limited to cases with bilateral amygdala destruction. In fact, various alternate mechanisms may be involved, warranting exploration of the syndrome’s presentation, pathophysiology, prognosis, and management.

Data Sources: Clinical management and the electronic medical records were examined for 2 patients diagnosed with partial KBS (ICD-10 F07.0) after experiencing ≥ 3 of the following: placidity, indiscriminate dietary behavior, hyperorality, hypersexuality, visual agnosia, and hypermetamorphosis. A literature search was performed in April 2015 by using the keyword Kluver-Bucy in PubMed and Ovid databases for English language publications since inception. Additionally, the authors reviewed the reference list of these publications in order to identify additional reports.

Study Selection: Studies were included if they had information about presentation, pathophysiology, syndrome treatment or management, and course of KBS.

Data Extraction: Information about our KBS cases was obtained by reviewing electronic medical records and by direct observation of the patients. A total of 186 (PubMed) and 137 (Ovid) publications were identified in each database. We ultimately reviewed 109 articles containing information about KBS, finding 51 publications addressing relevant aspects of this syndrome.

Results: The first case demonstrates KBS secondary to mesiotemporal structural atrophy, and the second illustrates transient KBS due to functional, postictal, hypoactivity within such structures. Literature review and discussion regarding both prognosis and treatment of KBS follows.

Conclusions: Klüver-Bucy syndrome may be underreported due to a limited understanding of the syndrome as one necessitating bilateral amygdaloid destruction. The syndrome can be seen with damage/hypofunction of the hippocampal-amygdaloid complex and its projections. The prognosis of KBS is variable, and its treatment is based on a combination of environmental and pharmacologic measures.