Familial Creutzfeldt-Jakob Disease with a Codon 200 Mutation Presenting as Thalamic Syndrome: Diagnosis by Single Photon Emission Computed Tomography using 99mTc-ethyl Cysteinate Dimer
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概要
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The clinical features of familial Creutzfeldt-Jakob disease with a codon 200 point mutation [fCJD (E200K)] are similar to those of sporadic CJD (sCJD). MRI diffusion-weighted imaging (MRI-DWI) has been reported to be useful for the early diagnosis of CJD. We describe a Japanese fCJD (E200K) case in which thalamic symptoms were the initial manifestations. On admission, electroencephalography (ECG) showed no periodic synchronous discharge (PSD), and MRI showed no abnormalities. However, single photon emission computed tomography (SPECT) using 99mTc-ethyl cysteinate dimer (99mTc-ECD) revealed hypoperfusion in the right thalamus. We conclude that the thalamic form of CJD tends to show no high-intensity area (HIA) by MRI-DWI, and that SPECT may be more useful for visualizing the affected area responsible for the thalamic symptoms at an early stage.
著者
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Kurihara Teruyuki
Division Of Neurology Department Of Internal Medicine Toho University Ohashi Hospital
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Sugimoto Hideki
Division Of Chemistry Graduate School Of Science Hokkaido University
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Nakazora Hiroshi
Division Of Neurology Department Of Internal Medicine Toho University Ohashi Medical Center
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Fujioka Toshiki
Division of Neurology, Department of Internal Medicine Toho University Ohashi Medical Center
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Yoshii Yasuhiro
Division of Neurology, Department of Internal Medicine Toho University Ohashi Medical Center
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Konno Shingo
Division of Neurology, Department of Internal Medicine Toho University Ohashi Medical Center
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Murata Mayumi
Division of Neurology, Department of Internal Medicine Toho University Ohashi Medical Center
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Toda Takahiro
Division of Neurology, Department of Internal Medicine Toho University Ohashi Medical Center
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Nomoto Nobuatsu
Division of Neurology, Department of Internal Medicine Toho University Ohashi Medical Center
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Nemoto Hiroshi
Division of Neurology, Department of Internal Medicine Toho University Ohashi Medical Center
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Wakata Nobuo
Division of Neurology, Department of Internal Medicine Toho University Ohashi Medical Center
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- Familial Creutzfeldt-Jakob Disease with a Codon 200 Mutation Presenting as Thalamic Syndrome: Diagnosis by Single Photon Emission Computed Tomography using 99mTc-ethyl Cysteinate Dimer
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