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Spectroscopy and serial diffusion MR findings in hGH-Creutzfeldt-Jakob disease

¹ Department of Neuroimaging, Sainte-Anne Hospital, 75014 Paris, France
² Neurological Department, Sainte-Anne Hospital
³ Neuroradiological Department, Pitié-Salpêtrière Hospital, 75013 Paris, France
⁴ Internal Medicine Department, Cochin Port-Royal Hospital, 75013 Paris, France
⁵ Intensive Care Unit, Pitié-Salpêtrière Hospital

Correspondence to:
Correspondence to:
Dr C Oppenheim
Département d’Imagerie Morphologique et Fonctionnelle, Centre Hospitalier Sainte-Anne, Université Paris V, 1 rue Cabanis, 75014 Paris, France; oppenheim{at}chsa.broca.inserm.fr

ABSTRACT
This report describes the magnetic resonance imaging (MRI) findings in two patients fulfilling the diagnostic criteria for human growth hormone Creutzfeldt-Jakob disease, who initially had pronounced bilateral DWI/FLAIR (diffusion weighted imaging/fluid attenuated inversion recovery) hyperintensities in the basal ganglia, with decreased apparent diffusion coefficient (ADC) values (range, 58–82% of normal). MRI spectroscopy, obtained in one case, showed decreased N-acetyl aspartate/creatine (NAA/Cr) ratio in the atrophic vermis (0.79; normal: mean, 1.20; SD, 0.13), despite the lack of DWI/FLAIR signal changes, whereas NAA/Cr was normal in the putamina (1.6; normal: mean, 1.56; SD, 0.17), despite striking DWI signal changes and decreased ADC values (60% of normal). Serial DWI, obtained in the other case, showed a progressive disappearance of DWI hypersignal of the basal ganglia replaced by pronounced atrophy. Data from these two patients suggest that restricted diffusion associated with a normal NAA value might indicate spongiform changes of still viable cells, and that any subsequent regression of the DWI signal changes, atrophy, or decreased NAA values could be related to progressive neuronal death.

Abbreviations: ADC, apparent diffusion coefficient; CJD, Creutzfeldt-Jakob disease; Cr, creatine; CSF, cerebrospinal fluid; DWI, diffusion weighted imaging; EEG, electroencephalogram; FLAIR, fluid attenuated inversion recovery; hGH, human growth hormone; MRI, magnetic resonance imaging; NAA, N-acetyl aspartate; PRNP, human prion protein gene

Keywords: Creutzfeldt-Jakob disease; magnetic resonance imaging; brain; diffusion; spectroscopy

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