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Proton magnetic resonance spectroscopic imaging in the clinicalevaluation of patients with Niemann-Pick type C disease |
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| Authors: | G Tedeschi S Bonavita N Barton A Bertolino J Frank N Patronas J Alger R Schiffmann |
| Affiliation: | Neuroimaging Branch, National Institutes of Health, Bethesda, MD, USA. |
| Abstract: | OBJECTIVES—10 patientswith Niemann-Pick disease type C (NP-C) were studied by proton magneticresonance spectroscopic imaging (1H-MRSI) to assess thebiochemical pathology of the brain and to determine whether this methodcan be useful to clinically evaluate these patients. METHODS—1H-MRSIpermits the simultaneous measurement of N-acetyl aspartate (NA),compounds containing choline (Cho), creatine plus phosphocreatine(Cre), and lactate (Lac) signal intensities from four 15 mm slicesdivided into 0.84 ml single volume elements. Spectroscopic voxels wereidentified from seven regions of interest. RESULTS—In patientswith NP-C, NA/Cre was significantly decreased in the frontal andparietal cortices, centrum semiovale, and caudate nucleus; Cho/Cre wassignificantly increased in the frontal cortex and centrum semiovale.Significant correlations were found between clinical staging scalescores and 1H-MRSI abnormalities. CONCLUSION—1H-MRSIshowed diffuse brain involvement in patients with NP-C consistent withthe pathological features of the disease. 1H-MRSI is anobjective and sensitive tool to neurologically evaluate patients withNP-C. |
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