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ARTICLE

Proton MR Spectroscopy of Sjögren-Larsson's Syndrome

Toshiyuki Manoa, Jiro Onoa, Tatsuro Kaminagaa, Katsumi Imaia, Kosuke Sakuraia, Koushi Haradaa, Toshisaburo Nagaia, William B. Rizzoa and Shintaro Okadaa

a From the Department of Pediatrics (T.M., J.O., K.I., S.O.), Osaka University Medical School, the Department of Radiology (T.K.), Teikyo University medical School, the Department of Radiology (K.S.), Osaka Teisin Hospital, the Division of Radiology (K.H.), Kaizuka Municipal Hospital, the Department of Pediatrics (T.N.), Toyonaka Municipal Hospital, and the Departments of Pediatrics and Human Genetics (W.B.R.), Medical College of Virginia.

Summary: 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.




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