Journal of Inherited Metabolic Disease

, Volume 33, Supplement 3, pp 477–480 | Cite as

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

  • Alix Mollet Boudjemline
  • Arnaud Isapof
  • Jean-Bernard Witas
  • François M. Petit
  • Vincent Gajdos
  • Philippe Labrune
Case Report


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.


Methylphenidate Severe Hypoglycaemia Glycogen Storage Disease Glycogen Storage Disease Type Anosognosia 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.


  1. Auvichayapat N, Auvichayapat P, Watanatorn J, Thamaroj J, Jitpimolmard S (2006) Klüver Bucy syndrome after mycoplasmal bronchitis. Epilepsy behav 8:3220–3322CrossRefGoogle Scholar
  2. Bucy PC, Klüver H (1955) An anatomical investigation of the temporal lobe in the monkey (Macaca mulatta). J Comp Neurol 103:151–251PubMedCrossRefGoogle Scholar
  3. 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 91:54–57PubMedGoogle Scholar
  4. Hashimoto NK, Ikeda S (2008) Klüver Bucy syndrome following status epilepticus associated with hepatic encephalopathy. Epilepsy behav 12:337–339PubMedCrossRefGoogle Scholar
  5. Jha S, Patel R (2004) Kluver Bucy syndrome-An experience with six cases. Neurol India 52:369–371PubMedGoogle Scholar
  6. 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 297:74–75PubMedCrossRefGoogle Scholar
  7. Lilly R, Cummings JL, Benson DF, Frankel M (1983) The human Klüver-Bucy syndrome. Neurology 33:1141–1145PubMedCrossRefGoogle Scholar
  8. 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 144:637–642PubMedCrossRefGoogle Scholar
  9. Ott BR (1995) Leuprolide treatment of sexual aggression in a patient with dementia and Klüver Bucy syndrome. Clin Neuropharmacol 18:443–447PubMedCrossRefGoogle Scholar
  10. Pradhan S, Singh MN, Pandey N (1998) Kluver Bucy syndrome in young children. Clin Neurol Neurosurg 100:254–258PubMedCrossRefGoogle Scholar
  11. 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–S34PubMedGoogle Scholar
  12. 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–S119PubMedCrossRefGoogle Scholar
  13. 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
  14. 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 278:47098–47103PubMedCrossRefGoogle Scholar
  15. Slaughter J, Bobo W, Childer MK (1999) selective serotonin reuptake inhibitor treatmentof post-traumatic Klüver-Bucy syndrome. Brain Inj 13:59–62PubMedCrossRefGoogle Scholar
  16. Tonsgard JH, Harwicke N, Levine SC (1987) Kluver Bucy syndrome in children. Pediatr Neurol 3:162–165PubMedCrossRefGoogle Scholar

Copyright information

© SSIEM and Springer 2010

Authors and Affiliations

  • Alix Mollet Boudjemline
    • 1
  • Arnaud Isapof
    • 1
  • Jean-Bernard Witas
    • 5
  • François M. Petit
    • 4
  • Vincent Gajdos
    • 1
  • Philippe Labrune
    • 1
    • 2
    • 3
    • 6
  1. 1.APHP, Service de PédiatrieHôpital Antoine BéclèreClamartFrance
  2. 2.Univ-Paris Sud, UFR Kremlin BicêtreClamartFrance
  3. 3.INSERM U 948, CHU NantesNantesFrance
  4. 4.APHP, Laboratoire de biochimie et biologie moléculaireHôpital Antoine BéclèreClamartFrance
  5. 5.CMPR Centre du ChâteauSoisy sur SeineFrance
  6. 6.Centre de Référence des Maladies Héréditaires du Métabolisme Hépatique, Service de PédiatrieHôpital Antoine BéclèreClamart cedexFrance

Personalised recommendations