Literature DB >> 3263613

Regional brain glucose utilization in adenylosuccinase-deficient patients measured by positron emission tomography.

A G De Volder1, J Jaeken, G Van den Berghe, A Bol, C Michel, M Cogneau, A M Goffinet.   

Abstract

Regional brain glucose utilization was investigated by positron emission tomography with fluorodeoxyglucose in three children with adenylosuccinase deficiency. A consistent pattern was found in the three patients, namely a marked decrease of fluorodeoxyglucose uptake in all gray structures, with the exception of the cerebellum, which was minimally affected. Anomalies predominated in the cerebral cortex, particularly in the anterior regions; they were less pronounced in thalamus and basal ganglia. The observations suggest that positron emission tomography may be a useful tool for the localization of the deleterious effects of metabolic diseases and for the investigation of their pathophysiologic mechanisms.

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Year:  1988        PMID: 3263613     DOI: 10.1203/00006450-198808000-00020

Source DB:  PubMed          Journal:  Pediatr Res        ISSN: 0031-3998            Impact factor:   3.756


  6 in total

1.  Long-term outcome in children with intractable epilepsy showing bilateral diffuse cortical glucose hypometabolism pattern on positron emission tomography.

Authors:  Varun Shandal; Amy L Veenstra; Michael Behen; Senthil Sundaram; Harry Chugani
Journal:  J Child Neurol       Date:  2011-09-22       Impact factor: 1.987

Review 2.  Inborn errors of the purine nucleotide cycle: adenylosuccinase deficiency.

Authors:  G Van den Berghe; M F Vincent; J Jaeken
Journal:  J Inherit Metab Dis       Date:  1997-06       Impact factor: 4.982

3.  Cerebral metabolic change after treatment in biotinidase deficiency.

Authors:  I T Lott; S Lottenberg; W L Nyhan; M J Buchsbaum
Journal:  J Inherit Metab Dis       Date:  1993       Impact factor: 4.982

4.  Neuronal ceroid-lipofuscinosis: preferential metabolic alterations in thalamus and posterior association cortex demonstrated by PET.

Authors:  A G De Volder; S Cirelli; T de Barsy; J M Brucher; A Bol; C Michel; A M Goffinet
Journal:  J Neurol Neurosurg Psychiatry       Date:  1990-12       Impact factor: 10.154

5.  Adenylosuccinase deficiency: a patient with impaired erythrocyte activity and anomalous response to intravenous fructose.

Authors:  C Salerno; C Crifò; O Giardini
Journal:  J Inherit Metab Dis       Date:  1995       Impact factor: 4.982

6.  Exploring metabolic pathway disruption in the subchronic phencyclidine model of schizophrenia with the Generalized Singular Value Decomposition.

Authors:  Xiaolin Xiao; Neil Dawson; Lynsey Macintyre; Brian J Morris; Judith A Pratt; David G Watson; Desmond J Higham
Journal:  BMC Syst Biol       Date:  2011-05-16
  6 in total

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