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01.12.2010 | Case Report

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

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

Erschienen in: Journal of Inherited Metabolic Disease | Sonderheft 3/2010

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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.

Auvichayapat N, Auvichayapat P, Watanatorn J, Thamaroj J, Jitpimolmard S (2006) Klüver Bucy syndrome after mycoplasmal bronchitis. Epilepsy behav 8:3220–3322CrossRef

Bucy PC, Klüver H (1955) An anatomical investigation of the temporal lobe in the monkey (Macaca mulatta). J Comp Neurol 103:151–251PubMedCrossRef

Formisano R, Saltuari L, Gerstenbrand F (1995) The presence of Klüver Bucy syndrome as a positive prognostic feature for the remission of traumatic prolonged disturbances of consciousness. Acta Neurol Scand 91:54–57PubMed

Hashimoto NK, Ikeda S (2008) Klüver Bucy syndrome following status epilepticus associated with hepatic encephalopathy. Epilepsy behav 12:337–339PubMedCrossRef

Jha S, Patel R (2004) Kluver Bucy syndrome-An experience with six cases. Neurol India 52:369–371PubMed

Juryńczyk M, Rozniecki J, Zaleski K, Selmaj K (2010) Hypoglycemia as a trigger for the syndrome of acute bilateral basal ganglia lesions in uremia. J Neurol Sci 297:74–75PubMedCrossRef

Lilly R, Cummings JL, Benson DF, Frankel M (1983) The human Klüver-Bucy syndrome. Neurology 33:1141–1145PubMedCrossRef

Melis D, Parenti G, Della Casa R, Sibilio M, Romano A, Di Salle F, Elefante R, Mansi G, Santoro L, Perretti A, Paludetto R, Sequino L, Andria G (2004) Brain damage in glycogen storage disease type I. J Pediatr 144:637–642PubMedCrossRef

Ott BR (1995) Leuprolide treatment of sexual aggression in a patient with dementia and Klüver Bucy syndrome. Clin Neuropharmacol 18:443–447PubMedCrossRef

Pradhan S, Singh MN, Pandey N (1998) Kluver Bucy syndrome in young children. Clin Neurol Neurosurg 100:254–258PubMedCrossRef

Rake JP, Visser G, Labrune P, Leonard JV, Ullrich K, Smit GPA (2002a) Glycogen storage disease type I: diagnosis, management, clinical course and outcome. Results of the European study on glycogen storage disease type I. Eur J Pediatr 161:S20–S34PubMed

Rake JP, Visser G, Labrune P, Leonard JV, Ullrich K, Smit GPA (2002b) Guidelines for management of glycogen storage disase type I- European study on glycogen storage disease type I (ESGSD I). Eur J Pediatr 161:S112–S119PubMedCrossRef

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–S123

Shieh JJ, Pan CJ, Mansfield BC, Chou JY (2003) A glucose-6-phosphate hydrolase, widely expressed outside the liver, can explain age-dependent resolution of hypoglycaemia in glycogen storage disease type Ia. J Biol Chem 278:47098–47103PubMedCrossRef

Slaughter J, Bobo W, Childer MK (1999) selective serotonin reuptake inhibitor treatmentof post-traumatic Klüver-Bucy syndrome. Brain Inj 13:59–62PubMedCrossRef

Tonsgard JH, Harwicke N, Levine SC (1987) Kluver Bucy syndrome in children. Pediatr Neurol 3:162–165PubMedCrossRef

Titel: Klüver Bucy syndrome following hypoglycaemic coma in a patient with glycogen storage disease type Ib
verfasst von: Alix Mollet Boudjemline
Arnaud Isapof
Jean-Bernard Witas
François M. Petit
Vincent Gajdos
Philippe Labrune
Publikationsdatum: 01.12.2010
Verlag: Springer Netherlands
Erschienen in: Journal of Inherited Metabolic Disease / Ausgabe Sonderheft 3/2010
Print ISSN: 0141-8955
Elektronische ISSN: 1573-2665
DOI: https://doi.org/10.1007/s10545-010-9243-y

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