Neuroimaging correlation with neurological severity in tuberous sclerosis complex.
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.
Chou
I-Jun
IJ
Division of Pediatric Neurology, Department of Pediatrics, Chang Gung Children's Hospital, 5 Fu-Shin Street, Kwei-Shan 333, Taoyuan, Taiwan.
Lin
Kuang-Lin
KL
Wong
Alex M
AM
Wang
Huei-Shyong
HS
Chou
Min-Liang
ML
Hung
Po-Cheng
PC
Hsieh
Meng-Ying
MY
Chang
Ming-Yu
MY
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*