Diagnostics (May 2020)

Functional Magnetic Resonance Imaging in the Final Stage of Creutzfeldt-Jakob Disease

  • Stefan M. Golaszewski,
  • Bettina Wutzl,
  • Axel F. Unterrainer,
  • Cristina Florea,
  • Kerstin Schwenker,
  • Vanessa N. Frey,
  • Martin Kronbichler,
  • Frank Rattay,
  • Raffaele Nardone,
  • Larissa Hauer,
  • Johann Sellner,
  • Eugen Trinka

DOI
https://doi.org/10.3390/diagnostics10050309
Journal volume & issue
Vol. 10, no. 5
p. 309

Abstract

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Sporadic Creutzfeldt-Jakob disease (sCJD) is a rare fatal degenerative disease of the central nervous system. The clinical course is characterized by rapid progression of neurological and neuromuscular symptoms. The late stage with loss of consciousness is not well characterized. We report a 62-year-old male patient with sCJD with the clinical picture of a vegetative state/apallic syndrome, in whom we studied cortical responses using a vibration paradigm. The functional magnetic resonance imaging (fMRI) investigation demonstrated a clear response within the sensorimotor cortex, the cerebellum, the parietal cortex, the insular, and frontal inferior region. The finding of persistent cortical activity on fMRI in a patient with CJD in a state of unconsciousness has implications for the clinical management and for ethical considerations.

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