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

doi:10.1007/s002340000371

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

MED - United Centers for Advanced Research and Translational Medicine (ART)

Research output: Contribution to journal › Article › peer-review

23 Citations (Scopus)

Abstract

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 language	English
Pages (from-to)	662-665
Number of pages	4
Journal	Neuroradiology
Volume	42
Issue number	9
DOIs	https://doi.org/10.1007/s002340000371
Publication status	Published - 2000

Keywords

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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10.1007/s002340000371

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title = "Proton magnetic resonance spectroscopy of a patient with Gerstmann-Straussler-Scheinker disease",

abstract = "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.",

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T1 - Proton magnetic resonance spectroscopy of a patient with Gerstmann-Straussler-Scheinker disease

AU - Konaka, K.

AU - Kaido, M.

AU - Okuda, Y.

AU - Aoike, F.

AU - Abe, K.

AU - Kitamoto, T.

AU - Yanagihara, T.

PY - 2000

Y1 - 2000

N2 - 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.

AB - 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.

KW - Gerstmann-Straussler-Scheinker disease

KW - Magnetic resonance spectroscopy

KW - Single-photon emission computed tomography

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

Abstract

Keywords

ASJC Scopus subject areas

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