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01-12-2010 | Case Report

Klüver Bucy syndrome following hypoglycaemic coma in a patient with glycogen storage disease type Ib

Authors: Alix Mollet Boudjemline, Arnaud Isapof, Jean-Bernard Witas, François M. Petit, Vincent Gajdos, Philippe Labrune

Published in: Journal of Inherited Metabolic Disease | Special Issue 3/2010

Abstract

Patients with type I glycogen storage disease (GSD) have poor tolerance to fasting, sometimes less than 3 hours during infancy. Even though most patients are able, as they get older, to tolerate a longer fasting period, they are at permanent risk for fast-induced hypoglycaemia, even in adulthood. Klüver Bucy syndrome, is characterized by psychic blindness (inability to recognize familiar objects), hypermetamorphosis (strong tendency to react to visual stimulus), increased oral exploration, placidity, indiscriminate hyper-sexuality and change in dietary habits. In this case report, we describe the development of Klüver Bucy syndrome in a 28-year-old man with type Ib GSD, following prolonged and severe hypoglycaemia triggered by a common respiratory infection.

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Title: Klüver Bucy syndrome following hypoglycaemic coma in a patient with glycogen storage disease type Ib
Authors: Alix Mollet Boudjemline
Arnaud Isapof
Jean-Bernard Witas
François M. Petit
Vincent Gajdos
Philippe Labrune
Publication date: 01-12-2010
Publisher: Springer Netherlands
Published in: Journal of Inherited Metabolic Disease / Issue Special Issue 3/2010
Print ISSN: 0141-8955
Electronic ISSN: 1573-2665
DOI: https://doi.org/10.1007/s10545-010-9243-y

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