Combined diffusion imaging and MR spectroscopy in the diagnosis of human prion diseases

Damien Galanaud1, S Haik, M G Linguraru

  • 1Department of Neuroradiology, Pitié-Salpêtrière Hospital, Paris, France. galanaud@dat.org

Abstract

Insights

Magnetic Resonance Imaging (MRI) combined with Magnetic Resonance Spectroscopy (MRS) aids in diagnosing Transmissible Spongiform Encephalopathies (TSEs). This multimodal approach offers insights into the physiopathology of signal intensity and apparent diffusion coefficient (ADC) changes in prion diseases.

Area of Science:

  • Neuroimaging
  • Prion Diseases
  • Biochemistry

Background:

  • The physiopathologic mechanisms behind signal intensity changes and reduced diffusion in Transmissible Spongiform Encephalopathies (TSEs) remain unclear.
  • Magnetic Resonance Imaging (MRI) techniques like Diffusion-Weighted Imaging (DWI) and Magnetic Resonance Spectroscopy (MRS) show promise in TSE diagnosis.

Purpose of the Study:

  • To evaluate the utility of MRS combined with DWI as a diagnostic tool for TSEs.
  • To elucidate the underlying mechanisms of signal intensity and apparent diffusion coefficient (ADC) changes in TSEs.

Main Methods:

  • A prospective study involving 45 patients with suspected TSEs and 11 healthy volunteers.
  • Multimodal MRI protocol including T1, FLAIR, DWI sequences, and MRS of specific brain regions (cerebellum, pulvinar, lenticular nucleus, frontal cortex).
  • Visual assessment of MR images and calculation of ADC values.

Main Results:

  • 31 patients were diagnosed with TSEs (including sporadic, iatrogenic, variant, and genetic forms), and 14 with alternative diseases.
  • Abnormal FLAIR and DWI signals were observed in most TSE patients, with decreased ADC in the basal ganglia correlating with high DWI signals.
  • Metabolic alterations (increased myo-inositol, decreased N-acetylaspartate) were detected in all TSE patients, but did not correlate with ADC values, suggesting factors beyond neuronal stress, loss, or astrogliosis contribute to ADC reduction.

Conclusions:

  • MRS combined with other MRI techniques is valuable for diagnosing TSEs.
  • This multimodal approach provides significant insights into the physiopathologic processes of prion diseases.

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