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DOI: 10.1055/s-2007-985368
© Georg Thieme Verlag KG Stuttgart · New York
The Correlation between 1H-MR Spectroscopy and Clinical Manifestation with Tuberous Sclerosis Complex
Publication History
received 11.11.2006
accepted 01.06.2007
Publication Date:
05 November 2007 (online)
Abstract
Tuberous sclerosis complex (TSC) is an autosomal dominant neurocutaneous disease. Cortical tubers are one of the standard intracranial hallmarks of TSC, they comprise subependymal hamartomas protruding into the ventricles, cortical and white matter hamartomas, and giant cell tumors. The clinical course of TSC varies from asymptomatic to severe, with epileptic seizures and psychomotor retardation. We discuss here the correlation between clinical manifestation and features on 1H-MR spectroscopy (1H-MRS) of the white matter involving cortical tubers in patients with TSC. Statistical analysis of the N-acetylaspartate (NAA), choline (Cho) and myoinositol (mI)/creatinine (Cr) ratios between tubers and normal controls showed decreased NAA/Cr and increased mI/Cr ratios (P<0.05) in tubers, but no significance difference in Cho/Cr. The significance of the clinical appearance is associated with a decreased ratio of NAA/Cr in tubers with TSC. An elevated ratio of mI/Cr in tuber does not parallel the severity of the clinical features of TSC. These findings suggest that 1H-MRS may be useful for the evaluation of the clinical severity and prognostic diagnosis of TSC.
Key words
Tuberous sclerosis complex (TSC) - MR imaging - MR spectroscopy - N-acetylaspartate - myoinositol - choline
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Correspondence
A. ImamuraMD
Department of Pediatrics
Gifu Prefectural General Medical Center
4-6-1 Noishiki
500-8717 Gifu
Japan
Phone: +81/58/246 11 11
Fax: +81/58/248 38 05
Email: aimamura30@hotmail.com