title: A perfusion-metabolic mismatch in Sturge-Weber syndrome: a multimodality imaging study.
abstract: OBJECTIVE
  We combined perfusion weighted imaging (PWI) with 2-deoxy-2[(18)F]fluoro-D-glucose (FDG) positron emission tomography (PET) to study the relationship between regional metabolic and perfusion abnormalities and their clinical correlates in children with Sturge-Weber syndrome (SWS).
  
  
  METHODS
  Fifteen children (age: 0.9-10 years) with unilateral SWS underwent high-resolution PWI and FDG PET prospectively. Regional (lobar) asymmetry indices (AIs) of subcortical white matter (WM) cerebral blood flow (CBF) were correlated with corresponding cortical FDG uptake asymmetries, extent of leptomeningeal vascular malformation and clinical seizure variables.
  
  
  RESULTS
  Abnormal cortical glucose metabolism and/or subcortical WM CBF were seen in all lobes affected by vascular malformation and extended to lobes not affected by abnormal pial vessels in 6 patients. Lower CBF was associated with lower cortical glucose metabolism in the temporal, parietal and occipital lobes (p≤0.02). While decreased perfusion was associated with hypometabolism in most cases, increased regional CBF (found in 6 patients) was commonly associated with relatively mild or no hypometabolism. Ten of 24 cerebral lobes with normal glucose metabolism in the affected hemisphere showed abnormal perfusion. High seizure frequency was associated with severe parieto-occipital hypoperfusion (p≤0.03), while long duration of epilepsy was related to frontal lobe hypometabolism (p=0.015).
  
  
  CONCLUSIONS
  Regional perfusion and cortical metabolic abnormalities can extend beyond lobes affected by leptomeningeal vascular malformations and are related to epilepsy in SWS. Despite a general correlation between perfusion and metabolism, increased WM perfusion with preserved cortical metabolism in overlying cortex is a common pattern of a perfusion/metabolic mismatch. This may represent a disease stage where cortical function is preserved while increased WM perfusion provides collateral drainage of cortex via the deep vein system.
authors.affiliation: Positron Emission Tomography Center, Children's Hospital of Michigan, Detroit, MI 48201, USA.
authors.forename: Bálint
authors.initials: B
authors.lastname: Alkonyi
authors.forename: Yanwei
authors.initials: Y
authors.lastname: Miao
authors.forename: Jianlin
authors.initials: J
authors.lastname: Wu
authors.forename: Zhaocheng
authors.initials: Z
authors.lastname: Cai
authors.forename: Jiani
authors.initials: J
authors.lastname: Hu
authors.forename: Harry T
authors.initials: HT
authors.lastname: Chugani
authors.forename: Csaba
authors.initials: C
authors.lastname: Juhász
mesh_terms: D001921:Brain / Q000098:blood supply / Q000473:pathology; D002648:Child; D002675:Child, Preschool; D005260:Female; D019788:Fluorodeoxyglucose F18; D005947:Glucose / Q000378:metabolism; D006801:Humans; D007091:Image Processing, Computer-Assisted; D007223:Infant; D018810:Magnetic Resonance Angiography / Q000379:methods*; D008297:Male; D055420:Perfusion Imaging / Q000379:methods*; D049268:Positron-Emission Tomography / Q000379:methods*; D011446:Prospective Studies; D012640:Seizures / Q000175:diagnosis / Q000000981:diagnostic imaging / Q000378:metabolism; D013341:Sturge-Weber Syndrome / Q000000981:diagnostic imaging* / Q000378:metabolism
chemical_list: D019788:Fluorodeoxyglucose F18; D005947:Glucose
references.citation: Pediatr Clin North Am. 1992 Aug;39(4):591-620
references.pmid: 1635798
references.citation: Neurosurgery. 1985 Nov;17(5):711-48
references.pmid: 4069326
references.citation: J Neurol Neurosurg Psychiatry. 1989 Dec;52(12):1402-9
references.pmid: 2614436
references.citation: Childs Nerv Syst. 1997 Apr;13(4):214-9
references.pmid: 9202857
references.citation: Acta Paediatr. 1995 Nov;84(11):1289-95
references.pmid: 8580629
references.citation: J Magn Reson Imaging. 2008 Jun;27(6):1229-34
references.pmid: 18504740
references.citation: Magn Reson Med. 1996 Nov;36(5):715-25
references.pmid: 8916022
references.citation: Eur J Paediatr Neurol. 2010 Sep;14(5):425-33
references.pmid: 20447845
references.citation: J Magn Reson Imaging. 2008 Oct;28(4):1019-25
references.pmid: 18821602
references.citation: J Neurol Neurosurg Psychiatry. 1997 Nov;63(5):616-21
references.pmid: 9408103
references.citation: Pediatr Neurol. 2007 May;36(5):330-3
references.pmid: 17509466
references.citation: Curr Opin Neurol. 2007 Apr;20(2):194-202
references.pmid: 17351491
references.citation: J Comput Assist Tomogr. 1982 Apr;6(2):365-72
references.pmid: 6978896
references.citation: Clin Nucl Med. 2011 Apr;36(4):313-4
references.pmid: 21368611
references.citation: J Magn Reson Imaging. 2003 Oct;18(4):404-13
references.pmid: 14508776
references.citation: J Magn Reson Imaging. 2007 Apr;25(4):696-702
references.pmid: 17279531
references.citation: Magn Reson Med. 1996 Nov;36(5):726-36
references.pmid: 8916023
references.citation: J Child Neurol. 2007 May;22(5):617-20
references.pmid: 17690070
references.citation: Eur J Paediatr Neurol. 2007 Mar;11(2):96-103
references.pmid: 17317246
references.citation: Eur J Paediatr Neurol. 2007 Sep;11(5):277-84
references.pmid: 17408998
references.citation: AJNR Am J Neuroradiol. 2007 May;28(5):900-6
references.pmid: 17494666
references.citation: Hum Brain Mapp. 2011 Nov;32(11):1973-85
references.pmid: 21259384
references.citation: Pediatrics. 2006 Jun;117(6):2119-25
references.pmid: 16740855
references.citation: Ann Neurol. 1987 Oct;22(4):487-97
references.pmid: 3501693
references.citation: J Child Neurol. 1997 Apr;12(3):218-22
references.pmid: 9130099
references.citation: Neuroradiology. 1997 Mar;39(3):219-24
references.pmid: 9106299
references.citation: AJNR Am J Neuroradiol. 2000 Jan;21(1):203-12
references.pmid: 10669252
references.citation: AJNR Am J Neuroradiol. 2011 Aug;32(7):1280-5
references.pmid: 21724573
references.citation: Neurology. 2001 Jul 24;57(2):189-95
references.pmid: 11468301
references.citation: J Pediatr. 1989 Feb;114(2):244-53
references.pmid: 2783735
references.citation: J Neurol Sci. 2009 Dec 15;287(1-2):271-4
references.pmid: 19733861
references.citation: Pediatr Neurol. 2004 May;30(5):303-10
references.pmid: 15165630
references.citation: J Child Neurol. 1998 Dec;13(12):606-18
references.pmid: 9881531
references.citation: J Magn Reson Imaging. 2006 Aug;24(2):274-81
references.pmid: 16786573
references.citation: Epilepsia. 2011 Jul;52(7):1265-72
references.pmid: 21480889