Cerebellar abnormality in children and young adults with tuberous sclerosis complex: MR and diffusion weighted imaging findings

Objectives - The goal of our study was to determine the incidence of cerebellar lesions in a cohort of children and young adults with TSC and analyze the magnetic resonance imaging (MRI) findings of cerebellar TSC lesions including their contrast behavior and diffusion characteristics Material and Methods - MRI studies of 27 TSC patients (mean age 10 6 years) were evaluated for cortical/subcortical tubers white matter lesions subependymal nodules and giant cell astrocytomas Patients with cerebellar involvement were further analyzed for the imaging and diffusion characteristics ADC measurements of the cerebellar tubers were performed and corn pared with the contralateral normal appearing cerebellum The clinical charts were revisited for symptoms suggesting cerebellar involvement Results - Cerebellar tubers were seen in 8/27 patients cerebellar atrophy in 1/27 patients Cerebellar tubers showed a pyramidal/wedge appearance with a broad base reaching the cortex The majority of the cerebellar tubers (11/12 92%) showed a zebra like contrast enhancement All cerebellar tubers had increased ADC values (mean ADC 1472 x 10(-6) mm(2)/s) None of the patients had typical cerebellar symptoms Conclusion - Thirty three percent of TSC patients had cerebellar lesions most of them being cerebellar tubers Cerebellar tubers differ from supratentonal tubers both concerning shape and contrast behavior The exact etiology of contrast enhancement remains unclear Future studies have to determine the impact of cerebellar lesions on neurocognitive development (C) 2010 Elsevier Masson SAS All rights reserved