title: Neuroimaging correlation with neurological severity in tuberous sclerosis complex.
abstract: OBJECTIVE
  To delineate the relationship between neurological severity and neuroimage of lesion load including specific topography of supratentorial cortical tubers and white matter lesions in tuberous sclerosis complex (TSC).
  
  
  METHODS
  Twenty-five TSC patients more than 2 years of age who underwent conventional and fluid-attenuated inversion recovery sequence (FLAIR) magnetic resonance imaging (MRI) were retrospectively studied. Neurological severity score was designated for three items: seizure, developmental delay and/or mental retardation, and autism. A neuroimaging scoring system was designed to evaluate the load of the cerebrum lesions with respect to location and size of cortical tubers and white matter lesions based on FLAIR MRI.
  
  
  RESULTS
  A linear trend was observed between MRI lesion score and neurological severity score (r=0.511; p=0.009). The lesion score in the left temporal lobe has positive correlation to neurological severity score (r=0.609; p=0.001).
  
  
  CONCLUSIONS
  The brain lesion load was positively correlated with neurological prognosis in TSC patients. Patients with larger lesion load in the left temporal lobe may be correlated with increased neurological severity in right-handed patients with TSC.
authors.affiliation: Division of Pediatric Neurology, Department of Pediatrics, Chang Gung Children's Hospital, 5 Fu-Shin Street, Kwei-Shan 333, Taoyuan, Taiwan.
authors.forename: I-Jun
authors.initials: IJ
authors.lastname: Chou
authors.forename: Kuang-Lin
authors.initials: KL
authors.lastname: Lin
authors.forename: Alex M
authors.initials: AM
authors.lastname: Wong
authors.forename: Huei-Shyong
authors.initials: HS
authors.lastname: Wang
authors.forename: Min-Liang
authors.initials: ML
authors.lastname: Chou
authors.forename: Po-Cheng
authors.initials: PC
authors.lastname: Hung
authors.forename: Meng-Ying
authors.initials: MY
authors.lastname: Hsieh
authors.forename: Ming-Yu
authors.initials: MY
authors.lastname: Chang
mesh_terms: D000293:Adolescent; D000328:Adult; D001921:Brain / Q000473:pathology*; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007091:Image Processing, Computer-Assisted; D008279:Magnetic Resonance Imaging; D008297:Male; D009420:Nervous System / Q000473:pathology*; D009483:Neuropsychological Tests; D011379:Prognosis; D012189:Retrospective Studies; D015173:Supratentorial Neoplasms / Q000473:pathology*; D014402:Tuberous Sclerosis / Q000473:pathology*