Proton magnetic resonance spectroscopy of a patient with Gerstmann-Straussler-Scheinker disease

K. Konaka, M. Kaido, Y. Okuda, F. Aoike, K. Abe, T. Kitamoto, T. Yanagihara

Research output: Contribution to journalArticlepeer-review

23 Citations (Scopus)


A 23-year-old woman with Gerstmann-Straussler-Scheinker disease (GSS) was investigated by 1H-magnetic resonance spectroscopy (1H-MRS). She developed gait ataxic at 22 years. The diagnosis was confirmed by DNA analysis showing a proline-to-leucine point mutation at codon 102 of the prion protein. On 1H-MRS, she showed a remarkable reduction of the N-acetylaspartate/creatine ratio in the frontal lobe, cerebellar hemisphere and vermis and putamen. MRI revealed mild atrophy of the cerebellar hemispheres and vermis and cerebral cortex, but single-photon emission computed tomography (SPECT) with 99mHMPAO showed normal perfusion in the cerebellum. The imaging studies suggest that MRS might be superior to MRI or SPECT for detection of early neuronal degeneration.

Original languageEnglish
Pages (from-to)662-665
Number of pages4
Issue number9
Publication statusPublished - 2000


  • Gerstmann-Straussler-Scheinker disease
  • Magnetic resonance spectroscopy
  • Single-photon emission computed tomography

ASJC Scopus subject areas

  • Radiology Nuclear Medicine and imaging
  • Clinical Neurology
  • Cardiology and Cardiovascular Medicine


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