Correlation between magnetic resonance imaging and histopathologic grades in Rasmussen syndrome.
The aim of this study was to investigate the correlation between magnetic resonance imaging (MRI) and histopathologic findings in Rasmussen syndrome. Serial MRIs were obtained for five patients who had histologically proven Rasmussen syndrome. The histopathologic grades of the lesions were subdivided into phases: active 1-3, resolving 1-3, and chronic inflammatory. The images were then correlated with histopathologic findings. Neuropathologic findings in the central areas on MRI demonstrated only the chronic and resolving grades, but active inflammatory abnormalities were present not only at the margins of the lesions, but also in areas of subtle signal abnormality on MRI. Atrophic areas on MRI exhibited all grades of histopathologic abnormalities, but chronic and resolving grades were predominant. Seizure duration of less than 6 months was associated with very active grades, duration of 1-2 years with variable grades, and duration greater than 6 years with chronic and resolving grades only. The MRI images correlated highly with histopathologic analysis. These findings suggest that the lesions initially arise from one site in the brain, and so support the centrifugal spreading theory of this disease. Findings also suggest that the margin rather than the center of the MRI abnormality may be the most ideal site for biopsy.
Department of Pediatrics, Chonbuk National University Medical School, Jeonju, Korea.
Sun Jun
SJ
Kim
Yong D
YD
Park
Richard
R
Hessler
Mark R
MR
Lee
Joseph R
JR
Smith
D000293:Adolescent; D000328:Adult; D001284:Atrophy / Q000473:pathology; D001479:Basal Ganglia / Q000473:pathology; D001706:Biopsy; D001921:Brain / Q000473:pathology* / Q000503:physiopathology; D002552:Cerebral Ventricles / Q000473:pathology; D002648:Child; D002675:Child, Preschool; D018450:Disease Progression; D004660:Encephalitis / Q000473:pathology* / Q000503:physiopathology; D005260:Female; D006801:Humans; D007249:Inflammation / Q000473:pathology; D008279:Magnetic Resonance Imaging; D008297:Male; D012640:Seizures / Q000175:diagnosis; D012720:Severity of Illness Index; D055815:Young Adult