Proton MR spectroscopy of Sjögren-Larsson's syndrome.
نویسندگان
چکیده
We performed single-voxel proton MR spectroscopy (1H-MRS) in two children with Sjögren-Larsson's syndrome (SLS). Both patients showed two abnormal spectral peaks at 1.3 ppm and 0.9 ppm that were obtained with short echo times. These two abnormal spectral peaks were seen in high-intensity areas on T2-weighted images and also in basal ganglia of normal intensities. 1H-MRS may be useful for establishing the diagnosis and investigating the natural history of SLS, and for evaluating the efficacy of therapeutic approaches to SLS.
منابع مشابه
MR imaging and proton MR spectroscopic studies in Sjögren-Larsson syndrome: characterization of the leukoencephalopathy.
BACKGROUND AND PURPOSE Sjögren-Larsson syndrome (SLS) is a neurocutaneous syndrome caused by a genetic enzyme deficiency in lipid metabolism. Our purpose was to characterize the nature of the cerebral involvement in SLS. METHODS MR imaging was performed in 18 patients (aged 5 months to 45 years) and repeated in 14. Single-voxel proton MR spectra were acquired from cerebral white matter and gr...
متن کاملMRI and 1H-MRS findings of three patients with Sjögren-Larsson syndrome.
Sjögren-Larsson syndrome (SLS) is a rare autosomal recessive neurocutaneous disorder caused by deficiency of the microsomal enzyme fatty aldehyde dehydrogenase. Patients present the classical triad of congenital ichthyosis, mental retardation and spastic di- or tetraplegia. Magnetic resonance imaging (MRI) of the brain usually shows hypomyelination involving the periventricular white matter. Ce...
متن کاملMR sialography in patients with Sjögren syndrome.
PURPOSE The purpose of this study was to evaluate the effectiveness of MR sialography of the parotid gland ducts in the diagnosis and staging of Sjögren syndrome. METHODS MR imaging was performed on a 1.5-T unit with a neck phased-array coil. MR sialographic source images were obtained using a heavily T2-weighted fast spin-echo sequence with spectral fat suppression. All images were analyzed ...
متن کاملMR imaging and proton MR spectroscopy in A-to-G substitution at nucleotide position 3243 of leucine transfer RNA.
MR imaging of the brain in a 38-year-old woman with maternally inherited diabetes and deafness (MIDD) showed extensive subcortical and basal ganglia high signal intensity on T2-weighted studies though she was neurologically asymptomatic. High-signal-intensity changes were also seen in the basal ganglia on T1-weighted studies. Proton MR spectroscopy showed increased lactate, an increased choline...
متن کاملPremature fat deposition in the salivary glands associated with Sjögren syndrome: MR and CT evidence.
PURPOSE To investigate abnormal fat deposition in the major salivary glands associated with Sjögren syndrome. METHODS We analyzed the fat deposition in the parotid and submandibular glands of 33 patients with Sjögren syndrome by using short-inversion-time inversion recovery (STIR) and fat-saturation MR sequences and CT values. RESULTS All three in vivo techniques substantially confirmed pre...
متن کاملذخیره در منابع من
با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید
عنوان ژورنال:
- AJNR. American journal of neuroradiology
دوره 20 9 شماره
صفحات -
تاریخ انتشار 1999