Serial diffusion-weighted MRI and SPECT findings in a Creutzfeldt–Jakob disease patient with V180I mutation
We report serial changes of diffusion-weighted imaging (DWI) and single photon emission computed tomography (SPECT) in a patient with Creutzfeldt–Jakob disease with V180I mutation (CJD180). DWI abnormalities in our patient were more predominantly observed in the left cerebral cortex than left basal...
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| Vydané v: | Journal of the neurological sciences Ročník 301; číslo 1; s. 100 - 103 |
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| Hlavní autori: | , , , , , , , |
| Médium: | Journal Article |
| Jazyk: | English |
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Elsevier B.V
15.02.2011
Elsevier |
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| Abstract | We report serial changes of diffusion-weighted imaging (DWI) and single photon emission computed tomography (SPECT) in a patient with Creutzfeldt–Jakob disease with V180I mutation (CJD180). DWI abnormalities in our patient were more predominantly observed in the left cerebral cortex than left basal ganglia. Hemilateral abnormalities progressed over 5
months to involve the contralateral side with increasing DWI signals. At 6
months, SPECT showed hypoperfusion in the left parietal and frontal lobes and the hypoperfusion region spread to the bilateral basal ganglia, right parietal and frontal lobes. SPECT imaging revealed marked cerebral blood flow reduction, predominantly in the cerebral cortex corresponding to brain areas with high-intensity DWI signals. During the follow-up period of CJD180, DWI was more sensitive than conventional FLAIR and T2-weighted images (T2WI) to detect and monitor the progression of abnormal hyperintense lesions. We suggest that serial DWI and SPECT findings are useful for not only early diagnosis of CJD but also for monitoring disease progression. |
|---|---|
| AbstractList | We report serial changes of diffusion-weighted imaging (DWI) and single photon emission computed tomography (SPECT) in a patient with Creutzfeldt-Jakob disease with V180I mutation (CJD180). DWI abnormalities in our patient were more predominantly observed in the left cerebral cortex than left basal ganglia. Hemilateral abnormalities progressed over 5 months to involve the contralateral side with increasing DWI signals. At 6 months, SPECT showed hypoperfusion in the left parietal and frontal lobes and the hypoperfusion region spread to the bilateral basal ganglia, right parietal and frontal lobes. SPECT imaging revealed marked cerebral blood flow reduction, predominantly in the cerebral cortex corresponding to brain areas with high-intensity DWI signals. During the follow-up period of CJD180, DWI was more sensitive than conventional FLAIR and T2-weighted images (T2WI) to detect and monitor the progression of abnormal hyperintense lesions. We suggest that serial DWI and SPECT findings are useful for not only early diagnosis of CJD but also for monitoring disease progression. Abstract We report serial changes of diffusion-weighted imaging (DWI) and single photon emission computed tomography (SPECT) in a patient with Creutzfeldt–Jakob disease with V180I mutation (CJD180). DWI abnormalities in our patient were more predominantly observed in the left cerebral cortex than left basal ganglia. Hemilateral abnormalities progressed over 5 months to involve the contralateral side with increasing DWI signals. At 6 months, SPECT showed hypoperfusion in the left parietal and frontal lobes and the hypoperfusion region spread to the bilateral basal ganglia, right parietal and frontal lobes. SPECT imaging revealed marked cerebral blood flow reduction, predominantly in the cerebral cortex corresponding to brain areas with high-intensity DWI signals. During the follow-up period of CJD180, DWI was more sensitive than conventional FLAIR and T2-weighted images (T2WI) to detect and monitor the progression of abnormal hyperintense lesions. We suggest that serial DWI and SPECT findings are useful for not only early diagnosis of CJD but also for monitoring disease progression. We report serial changes of diffusion-weighted imaging (DWI) and single photon emission computed tomography (SPECT) in a patient with Creutzfeldt–Jakob disease with V180I mutation (CJD180). DWI abnormalities in our patient were more predominantly observed in the left cerebral cortex than left basal ganglia. Hemilateral abnormalities progressed over 5 months to involve the contralateral side with increasing DWI signals. At 6 months, SPECT showed hypoperfusion in the left parietal and frontal lobes and the hypoperfusion region spread to the bilateral basal ganglia, right parietal and frontal lobes. SPECT imaging revealed marked cerebral blood flow reduction, predominantly in the cerebral cortex corresponding to brain areas with high-intensity DWI signals. During the follow-up period of CJD180, DWI was more sensitive than conventional FLAIR and T2-weighted images (T2WI) to detect and monitor the progression of abnormal hyperintense lesions. We suggest that serial DWI and SPECT findings are useful for not only early diagnosis of CJD but also for monitoring disease progression. We report serial changes of diffusion-weighted imaging (DWI) and single photon emission computed tomography (SPECT) in a patient with Creutzfeldt-Jakob disease with V180I mutation (CJD180). DWI abnormalities in our patient were more predominantly observed in the left cerebral cortex than left basal ganglia. Hemilateral abnormalities progressed over 5 months to involve the contralateral side with increasing DWI signals. At 6 months, SPECT showed hypoperfusion in the left parietal and frontal lobes and the hypoperfusion region spread to the bilateral basal ganglia, right parietal and frontal lobes. SPECT imaging revealed marked cerebral blood flow reduction, predominantly in the cerebral cortex corresponding to brain areas with high-intensity DWI signals. During the follow-up period of CJD180, DWI was more sensitive than conventional FLAIR and T2-weighted images (T2WI) to detect and monitor the progression of abnormal hyperintense lesions. We suggest that serial DWI and SPECT findings are useful for not only early diagnosis of CJD but also for monitoring disease progression.We report serial changes of diffusion-weighted imaging (DWI) and single photon emission computed tomography (SPECT) in a patient with Creutzfeldt-Jakob disease with V180I mutation (CJD180). DWI abnormalities in our patient were more predominantly observed in the left cerebral cortex than left basal ganglia. Hemilateral abnormalities progressed over 5 months to involve the contralateral side with increasing DWI signals. At 6 months, SPECT showed hypoperfusion in the left parietal and frontal lobes and the hypoperfusion region spread to the bilateral basal ganglia, right parietal and frontal lobes. SPECT imaging revealed marked cerebral blood flow reduction, predominantly in the cerebral cortex corresponding to brain areas with high-intensity DWI signals. During the follow-up period of CJD180, DWI was more sensitive than conventional FLAIR and T2-weighted images (T2WI) to detect and monitor the progression of abnormal hyperintense lesions. We suggest that serial DWI and SPECT findings are useful for not only early diagnosis of CJD but also for monitoring disease progression. |
| Author | Omori, Nobuhiko Manabe, Yasuhiro Sakai, Yasuko Fujii, Daiki Kono, Syoichiro Narai, Hisashi Kitamoto, Tetsuyuki Abe, Koji |
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| Cites_doi | 10.1212/01.WNL.0000106954.54011.80 10.1212/WNL.0b013e3181a96e5d 10.1007/s00439-005-0020-1 10.1212/WNL.0b013e3181a18846 10.1097/00004728-200103000-00022 10.1007/BF00593711 10.2214/ajr.184.2.01840560 10.1016/j.neulet.2006.08.008 10.1159/000254842 |
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| Keywords | Magnetic resonance imaging Prion protein Creutzfeldt–Jakob disease SPECT Human Prion disease Nervous system diseases Creutzfeldt-Jakob disease Single photon emission tomography Nuclear magnetic resonance imaging Photon Cerebral disorder Infection Central nervous system disease Degenerative disease Prion Mutation Diffusion |
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| References_xml | – volume: 37 start-page: 531 year: 1995 end-page: 534 ident: bb0015 article-title: Serial MRI in early Creutzfeldt–Jakob disease with a point mutation of prion protein at codon 180 publication-title: Neuroradiology – volume: 72 start-page: 1425 year: 2009 end-page: 1431 ident: bb0050 article-title: Pathologic correlates of diffusion MRI changes in Creutzfeldt–Jakob disease publication-title: Neurology – volume: 25 start-page: 274 year: 2001 end-page: 277 ident: bb0025 article-title: Creutzfeldt–Jakob disease: Serial changes on diffusion-weighted MRI publication-title: J Comp Assist Tomogr – volume: 72 start-page: 1994 year: 2009 end-page: 2001 ident: bb0005 article-title: MRI lesion profiles in sporadic Creutzfeldt–Jakob disease publication-title: Neurology – volume: 62 start-page: 502 year: 2004 end-page: 505 ident: bb0010 article-title: Clinical features of Creutzfeldt–Jakob disease with V180I mutation publication-title: Neurology – volume: 184 start-page: 560 year: 2005 end-page: 566 ident: bb0020 article-title: Serial diffusion-weighted MRI of Creutzfeldt–Jakob disease publication-title: AJR Am J Roentgenol – volume: 23 start-page: 1164 year: 2002 end-page: 1172 ident: bb0030 article-title: Conspicuity and evolution of lesions in Creutzfeldt–Jakob disease at diffusion-weighted imaging publication-title: AJNR Am J Neuroradiol – volume: 28 start-page: 550 year: 2009 end-page: 557 ident: bb0045 article-title: Familial Creutzfeldt–Jakob disease with a V180I mutation: Comparative analysis with pathological findings and diffusion-weighted images publication-title: Dement Geriatr Cogn Disord – volume: 408 start-page: 165 year: 2006 end-page: 169 ident: bb0040 article-title: V180I mutation of the prion protein gene associated with atypical PrP publication-title: Neurosci Lett – volume: 118 start-page: 166 year: 2005 end-page: 174 ident: bb0035 article-title: Genetic prion disease: The EUROCJD experience publication-title: Hum Genet – volume: 62 start-page: 502 year: 2004 ident: 10.1016/j.jns.2010.10.032_bb0010 article-title: Clinical features of Creutzfeldt–Jakob disease with V180I mutation publication-title: Neurology doi: 10.1212/01.WNL.0000106954.54011.80 – volume: 23 start-page: 1164 year: 2002 ident: 10.1016/j.jns.2010.10.032_bb0030 article-title: Conspicuity and evolution of lesions in Creutzfeldt–Jakob disease at diffusion-weighted imaging publication-title: AJNR Am J Neuroradiol – volume: 72 start-page: 1994 year: 2009 ident: 10.1016/j.jns.2010.10.032_bb0005 article-title: MRI lesion profiles in sporadic Creutzfeldt–Jakob disease publication-title: Neurology doi: 10.1212/WNL.0b013e3181a96e5d – volume: 118 start-page: 166 year: 2005 ident: 10.1016/j.jns.2010.10.032_bb0035 article-title: Genetic prion disease: The EUROCJD experience publication-title: Hum Genet doi: 10.1007/s00439-005-0020-1 – volume: 72 start-page: 1425 year: 2009 ident: 10.1016/j.jns.2010.10.032_bb0050 article-title: Pathologic correlates of diffusion MRI changes in Creutzfeldt–Jakob disease publication-title: Neurology doi: 10.1212/WNL.0b013e3181a18846 – volume: 25 start-page: 274 year: 2001 ident: 10.1016/j.jns.2010.10.032_bb0025 article-title: Creutzfeldt–Jakob disease: Serial changes on diffusion-weighted MRI publication-title: J Comp Assist Tomogr doi: 10.1097/00004728-200103000-00022 – volume: 37 start-page: 531 year: 1995 ident: 10.1016/j.jns.2010.10.032_bb0015 article-title: Serial MRI in early Creutzfeldt–Jakob disease with a point mutation of prion protein at codon 180 publication-title: Neuroradiology doi: 10.1007/BF00593711 – volume: 184 start-page: 560 year: 2005 ident: 10.1016/j.jns.2010.10.032_bb0020 article-title: Serial diffusion-weighted MRI of Creutzfeldt–Jakob disease publication-title: AJR Am J Roentgenol doi: 10.2214/ajr.184.2.01840560 – volume: 408 start-page: 165 year: 2006 ident: 10.1016/j.jns.2010.10.032_bb0040 article-title: V180I mutation of the prion protein gene associated with atypical PrPSc glycosylation publication-title: Neurosci Lett doi: 10.1016/j.neulet.2006.08.008 – volume: 28 start-page: 550 year: 2009 ident: 10.1016/j.jns.2010.10.032_bb0045 article-title: Familial Creutzfeldt–Jakob disease with a V180I mutation: Comparative analysis with pathological findings and diffusion-weighted images publication-title: Dement Geriatr Cogn Disord doi: 10.1159/000254842 |
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| SubjectTerms | Age of Onset Aged Akinetic Mutism - diagnostic imaging Akinetic Mutism - etiology Akinetic Mutism - pathology Biological and medical sciences Brain - diagnostic imaging Brain - pathology Creutzfeldt-Jakob Syndrome - complications Creutzfeldt-Jakob Syndrome - diagnostic imaging Creutzfeldt-Jakob Syndrome - epidemiology Creutzfeldt-Jakob Syndrome - genetics Creutzfeldt-Jakob Syndrome - pathology Creutzfeldt–Jakob disease Degenerative and inherited degenerative diseases of the nervous system. Leukodystrophies. Prion diseases Diffusion Magnetic Resonance Imaging Disease Progression Early Diagnosis Follow-Up Studies Humans Magnetic resonance imaging Male Medical sciences Mutant Proteins - genetics Mutation, Missense Neurology Point Mutation Prion protein PrPSc Proteins - genetics SPECT Tomography, Emission-Computed, Single-Photon |
| Title | Serial diffusion-weighted MRI and SPECT findings in a Creutzfeldt–Jakob disease patient with V180I mutation |
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