PROTON MAGNETIC-RESONANCE SPECTROSCOPIC IMAGING IN THE CLINICAL-EVALUATION OF PATIENTS WITH NIEMANN-PICK TYPE-C DISEASE

Objectives-10 patients with Niemann-Pick disease type C (NP-C) were st udied by proton magnetic resonance spectroscopic imaging (H-1-MRSI) to assess the biochemical pathology of the brain and to determine whethe r this method can be useful to clinically evaluate these patients. Met hods-H-1-MRSI permits the simultaneous measurement of N-acetyl asparta te (NA), compounds containing choline (Cho), creatine plus phosphocrea tine (Cre), and lactate (Lac) signal intensities from four 15 mm slice s divided into 0.84 mi single volume elements. Spectroscopic voxels we re identified from seven regions of interest. Results-In patients with NP-C, NA/Cre was significantly decreased in the frontal and parietal cortices, centrum semiovale, and caudate nucleus; Cho/Cre was signific antly increased in the frontal cortex and centrum semiovale. Significa nt correlations were found between clinical staging scale scores and H -1-MRSI abnormalities. Conclusion-H-1-MRSI showed diffuse brain involv ement in patients with NP-C consistent with the pathological features of the disease. H-1-MRSI is an objective and sensitive tool to neurolo gically evaluate patients with NP-C.