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Proton magnetic resonance spectroscopic imaging in the clinical evaluation of patients with Niemann-Pick type C disease
  1. G Tedeschia,
  2. S Bonavitaa,
  3. N W Bartonb,
  4. A Bertolinoc,
  5. J A Frankd,
  6. N J Patronase,
  7. J R Algerf,
  8. R Schiffmannb
  1. aNeuroimaging Branch, bDevelopmental and Metabolic Neurology Branch, NINDS, cClinical Brain Disorders Branch, NIMH, dLaboratory of Diagnostic Radiology Research, eDepartment of Diagnostic Radiology, National Institutes of Health, Bethesda, MD, USA, fDepartment of Radiological Sciences, University of California, Los Angeles, CA, USA
  1. Professor Gioacchino Tedeschi, Istituto di Scienze Neurologiche, Seconda Universita’ di Napoli, Via Pansini 5, 80131 Napoli, Italy. Telephone 0039 81 566 6786; fax 0039 81 566 6787.

Abstract

OBJECTIVES 10 patients with Niemann-Pick disease type C (NP-C) were studied by proton magnetic resonance spectroscopic imaging (1H-MRSI) to assess the biochemical pathology of the brain and to determine whether this method can be useful to clinically evaluate these patients.

METHODS 1H-MRSI permits 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 slices divided into 0.84 ml single volume elements. Spectroscopic voxels were 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 significantly increased in the frontal cortex and centrum semiovale. Significant correlations were found between clinical staging scale scores and 1H-MRSI abnormalities.

CONCLUSION 1H-MRSI showed diffuse brain involvement in patients with NP-C consistent with the pathological features of the disease. 1H-MRSI is an objective and sensitive tool to neurologically evaluate patients with NP-C.

  • proton magnetic resonance spectroscopy
  • Niemann-Pick disease
  • clinical stage

https://doi.org/10.1136/jnnp.65.1.72

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