title: Klüver Bucy syndrome following hypoglycaemic coma in a patient with glycogen storage disease type Ib.
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.
authors.affiliation: APHP, Service de Pédiatrie, Hôpital Antoine Béclère, Clamart, France.
authors.forename: Alix Mollet
authors.initials: AM
authors.lastname: Boudjemline
authors.forename: Arnaud
authors.initials: A
authors.lastname: Isapof
authors.forename: Jean-Bernard
authors.initials: JB
authors.lastname: Witas
authors.forename: François M
authors.initials: FM
authors.lastname: Petit
authors.forename: Vincent
authors.initials: V
authors.lastname: Gajdos
authors.forename: Philippe
authors.initials: P
authors.lastname: Labrune
mesh_terms: D000328:Adult; D015415:Biomarkers / Q000097:blood; D001786:Blood Glucose / Q000378:metabolism; D003128:Coma / Q000175:diagnosis / Q000209:etiology*; D005215:Fasting / Q000097:blood; D005953:Glycogen Storage Disease Type I / Q000150:complications* / Q000175:diagnosis / Q000628:therapy; D006801:Humans; D007003:Hypoglycemia / Q000097:blood / Q000175:diagnosis / Q000209:etiology* / Q000628:therapy; D020232:Kluver-Bucy Syndrome / Q000175:diagnosis / Q000209:etiology* / Q000523:psychology / Q000628:therapy; D008279:Magnetic Resonance Imaging; D008297:Male; D012141:Respiratory Tract Infections / Q000150:complications* / Q000175:diagnosis / Q000628:therapy; D012720:Severity of Illness Index; D016896:Treatment Outcome
chemical_list: D015415:Biomarkers; D001786:Blood Glucose