Kluver Bucy Syndrome: A Rare Neurological Disease with Several Challenges

Abstract

Kluver Bucy Syndrome is an extremely rare disorder with a diverse presentation that can be confused with certain common diseases such as Korsakoff’s psychosis, Pick’s disease, and Alzheimer’s dementia. The most common risk factors overall and in children include Herpes simplex encephalitis. In adults, temporal lobe stroke subsequent to several possible etiologies is the second most common cause. Almost any condition that affects the brain (majorly the temporal lobe) can lead to Kluver Bucy Syndrome, making it more difficult to find out the underlying cause. The syndrome is named after Paul Bucy and Heinrich Kluver who discovered it in the monkeys. The various manifestations occur because of the involvement of the amygdala and hippocampus in the temporal lobe and include amnesia which may be anterograde or retrograde, hyperphagia, hyperorality, hypersexuality, visual agnosia, and hypermetamorphosis. The limbic system may get affected causing the above manifestations too. Management of the Kluver Bucy Syndrome is challenging and needs more research as pharmacotherapy and behavioural therapies are the only known interventions to treat patients with KBS currently with not so good outcomes as the delay in the diagnosis makes it not respond to the medications quite a number of times. The diagnosis is mainly clinical and early detection and management of the underlying cause could help provide a good prognosis. In this Review article, we review the conditions leading to Kluver Bucy syndrome, its pathophysiology, clinical features, and the major challenges in its diagnosis.