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Cerebellar Lesions in Tuberous Sclerosis Complex

Neurobehavioral and Neuroimaging Correlates

Carman and Ann Adams Department of Pediatrics, Wayne State University

Michael E. Behen, PhD

Carman and Ann Adams Department of Pediatrics, Wayne State University, Department of Psychology, Wayne State University

Harry T. Chugani, MD

Carman and Ann Adams Department of Pediatrics, Wayne State University, dchugani{at}pet.wayne.edu, Department of Neurology, Wayne State University, Department of Radiology, Wayne State University

James Janisse, PhD

Center for Health Effectiveness Research, Children's Hospital of Michigan, Wayne State University Detroit, MI

Bruno Bernardi, MD

Department of Radiology, Wayne State University

Pulak Chakraborty, PhD

Department of Radiology, Wayne State University

Csaba Juhasz, MD,PhD

Carman and Ann Adams Department of Pediatrics, Wayne State University, Department of Neurology, Wayne State University

Otto Muzik, PhD

Carman and Ann Adams Department of Pediatrics, Wayne State University, Department of Radiology, Wayne State University

Diane C. Chugani, PhD

Carman and Ann Adams Department of Pediatrics, Wayne State University, Department of Radiology, Wayne State University

We assessed the structural and functional imaging features of cerebellar lesions and their neurobehavioral correlates in a large cohort of patients with tuberous sclerosis complex. A consecutive series of 78 patients with tuberous sclerosis complex underwent magnetic resonance imaging (MRI) and positron emission tomography (PET) studies with [¹⁸F]fluorodeoxyglucose (FDG) and -[¹¹C]methyl-L-tryptophan (AMT) as part of their evaluation for epilepsy surgery. Neurobehavioral assessment included the Gilliam Autism Rating Scales (GARS) and the Vineland Adaptive Behavior Scales (VABS). Twenty-one patients (27%) had cerebellar lesions (10 boys; mean age 9 ± 8 years; 9 had right-sided, 10 had left-sided, and 2 had bilateral cerebellar lesions). The lesions showed decreased glucose metabolism (0.79 ± 0.10) and increased (1.04 ± 0.10) AMT uptake compared with the normal (nonlesional) cerebellar cortex. Comparisons between patients with (n = 20) and without (n = 57) a cerebellar lesion on neurobehavioral functioning, controlling for the number and location of cortical tubers, revealed that the cerebellar lesion group had higher overall autistic symptomatology. Within-group analyses of the cerebellar lesion group revealed that children with right-sided cerebellar lesions had higher social isolation and communicative and developmental disturbance compared with children with left-sided cerebellar lesions. The side of the cerebellar lesion was not related to adaptive behavior functioning. These findings provide additional empiric support for a role of the cerebellum in autistic symptomatology. Further investigation of the potential role of the right cerebellum in autism, particularly with regard to the dentatothalamofrontal circuit, is warranted. (J Child Neurol 2006;21:846—851; DOI 10.2310/7010.2006.00192).

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