MR proton spectroscopy in patients with CNS involvement in Bourneville's disease

Abstract

Background: Despite much previous research, the origin of intracranial lesions in tuberous sclerosis is still unknown. We present MR and proton magnetic resonance (1H-MRS) studies of two patients with tuberous sclerosis.

Material and methods: The study material consisted of MR and 1H-MRS examinations from 2 patients (4-5 years old) with tuberous sclerosis. MR images (FSE, FAST, FLAIR) were obtained with a 1.5 T unit. Localized 1H-MRS studies (PRESS 35, TE 35 ms, TR 1500 ms, NEX 192) on cortical (n=3) and subependymal (n=1) lesions were also performed.

Results: Decreased N-acatyloaspartate/creatine (NAA/Cr) and increased myoinositol/creatine (mI/Cr) ratios were observed in the spectra obtained from the subcortical and subependymal tubers.

Conclusions: Our observations confirm the hypothesis that disturbances of migration, adhesion and differentiation of germ neuronal cells may underlay the pathogenesis of tuberous sclerosis.