Klüver Bucy syndrome following hypoglycaemic coma in a patient with glycogen storage disease type Ib
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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.
KeywordsMethylphenidate Severe Hypoglycaemia Glycogen Storage Disease Glycogen Storage Disease Type Anosognosia
- Rake JP, Visser G, Labrune P, Leonard JV, Moses S, Ullrich K, Wendel U, Smit GPA (2002c) Consensus guidelines for management of glycogen storage disease type Ib – European study on glycogen storage disease type I. Eur J Pediatr 61:S120–S123Google Scholar