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Diffusion-weighted MR Imaging of Early-Stage Creutzfeldt-Jakob Disease: Typical and Atypical Manifestations¹

¹ From the Department of Radiology, Showa University Northern Yokohama Hospital, 35-1 Chigasaki-chuou, Tsuzuki-ku, Yokohama 224-8503, Japan. Presented as an education exhibit at the 2005 RSNA Annual Meeting. Received February 6, 2006; revision requested March 14 and received May 1; accepted June 2. The authors have no financial relationships to disclose.

View larger version (140K): [in this window] [in a new window] [Download PPT slide]   Figure 1a.  Sporadic Creutzfeldt-Jakob disease in a 72-year-old woman with left hemiparesis. (a–c) MR images obtained 7 weeks after the onset of dementia and 2 weeks before the appearance of periodic synchronous discharges on the EEG and a negative result at testing for the 14-3-3 protein level in cerebrospinal fluid. Axial T1-weighted (a) and axial T2-weighted (b) images show no abnormalities. The diffusion-weighted image (c) reveals symmetric bilateral areas of abnormal high signal intensity in the frontotemporal region of the cerebral cortex, caudate nuclei, and putamen. (d–f) MR images obtained after disease progression to akinetic mutism, 27 weeks after the onset of symptoms. The T1-weighted image (d) shows marked cerebral atrophy. The T2-weighted image (e) shows periventricular white-matter signal hyperintensity and caudate nucleus atrophy. Asymmetric bilateral areas of high signal intensity are visible in the basal ganglia in e. The diffusion-weighted image (f) shows that the bilateral areas of high signal intensity in the basal ganglia have disappeared. High signal intensity is noticeable only in the insula and the thalamus in f. (g) Photomicrograph (original magnification, x20; hematoxylin and eosin stain) shows severe spongiform vacuolar degeneration and neuronal loss of the gray matter neuropil (ie, status spongiosus) in a specimen from the cerebral cortex.

 

View larger version (129K): [in this window] [in a new window] [Download PPT slide]   Figure 1b.  Sporadic Creutzfeldt-Jakob disease in a 72-year-old woman with left hemiparesis. (a–c) MR images obtained 7 weeks after the onset of dementia and 2 weeks before the appearance of periodic synchronous discharges on the EEG and a negative result at testing for the 14-3-3 protein level in cerebrospinal fluid. Axial T1-weighted (a) and axial T2-weighted (b) images show no abnormalities. The diffusion-weighted image (c) reveals symmetric bilateral areas of abnormal high signal intensity in the frontotemporal region of the cerebral cortex, caudate nuclei, and putamen. (d–f) MR images obtained after disease progression to akinetic mutism, 27 weeks after the onset of symptoms. The T1-weighted image (d) shows marked cerebral atrophy. The T2-weighted image (e) shows periventricular white-matter signal hyperintensity and caudate nucleus atrophy. Asymmetric bilateral areas of high signal intensity are visible in the basal ganglia in e. The diffusion-weighted image (f) shows that the bilateral areas of high signal intensity in the basal ganglia have disappeared. High signal intensity is noticeable only in the insula and the thalamus in f. (g) Photomicrograph (original magnification, x20; hematoxylin and eosin stain) shows severe spongiform vacuolar degeneration and neuronal loss of the gray matter neuropil (ie, status spongiosus) in a specimen from the cerebral cortex.

 

View larger version (138K): [in this window] [in a new window] [Download PPT slide]   Figure 1c.  Sporadic Creutzfeldt-Jakob disease in a 72-year-old woman with left hemiparesis. (a–c) MR images obtained 7 weeks after the onset of dementia and 2 weeks before the appearance of periodic synchronous discharges on the EEG and a negative result at testing for the 14-3-3 protein level in cerebrospinal fluid. Axial T1-weighted (a) and axial T2-weighted (b) images show no abnormalities. The diffusion-weighted image (c) reveals symmetric bilateral areas of abnormal high signal intensity in the frontotemporal region of the cerebral cortex, caudate nuclei, and putamen. (d–f) MR images obtained after disease progression to akinetic mutism, 27 weeks after the onset of symptoms. The T1-weighted image (d) shows marked cerebral atrophy. The T2-weighted image (e) shows periventricular white-matter signal hyperintensity and caudate nucleus atrophy. Asymmetric bilateral areas of high signal intensity are visible in the basal ganglia in e. The diffusion-weighted image (f) shows that the bilateral areas of high signal intensity in the basal ganglia have disappeared. High signal intensity is noticeable only in the insula and the thalamus in f. (g) Photomicrograph (original magnification, x20; hematoxylin and eosin stain) shows severe spongiform vacuolar degeneration and neuronal loss of the gray matter neuropil (ie, status spongiosus) in a specimen from the cerebral cortex.

 

View larger version (151K): [in this window] [in a new window] [Download PPT slide]   Figure 1d.  Sporadic Creutzfeldt-Jakob disease in a 72-year-old woman with left hemiparesis. (a–c) MR images obtained 7 weeks after the onset of dementia and 2 weeks before the appearance of periodic synchronous discharges on the EEG and a negative result at testing for the 14-3-3 protein level in cerebrospinal fluid. Axial T1-weighted (a) and axial T2-weighted (b) images show no abnormalities. The diffusion-weighted image (c) reveals symmetric bilateral areas of abnormal high signal intensity in the frontotemporal region of the cerebral cortex, caudate nuclei, and putamen. (d–f) MR images obtained after disease progression to akinetic mutism, 27 weeks after the onset of symptoms. The T1-weighted image (d) shows marked cerebral atrophy. The T2-weighted image (e) shows periventricular white-matter signal hyperintensity and caudate nucleus atrophy. Asymmetric bilateral areas of high signal intensity are visible in the basal ganglia in e. The diffusion-weighted image (f) shows that the bilateral areas of high signal intensity in the basal ganglia have disappeared. High signal intensity is noticeable only in the insula and the thalamus in f. (g) Photomicrograph (original magnification, x20; hematoxylin and eosin stain) shows severe spongiform vacuolar degeneration and neuronal loss of the gray matter neuropil (ie, status spongiosus) in a specimen from the cerebral cortex.

 

View larger version (147K): [in this window] [in a new window] [Download PPT slide]   Figure 1e.  Sporadic Creutzfeldt-Jakob disease in a 72-year-old woman with left hemiparesis. (a–c) MR images obtained 7 weeks after the onset of dementia and 2 weeks before the appearance of periodic synchronous discharges on the EEG and a negative result at testing for the 14-3-3 protein level in cerebrospinal fluid. Axial T1-weighted (a) and axial T2-weighted (b) images show no abnormalities. The diffusion-weighted image (c) reveals symmetric bilateral areas of abnormal high signal intensity in the frontotemporal region of the cerebral cortex, caudate nuclei, and putamen. (d–f) MR images obtained after disease progression to akinetic mutism, 27 weeks after the onset of symptoms. The T1-weighted image (d) shows marked cerebral atrophy. The T2-weighted image (e) shows periventricular white-matter signal hyperintensity and caudate nucleus atrophy. Asymmetric bilateral areas of high signal intensity are visible in the basal ganglia in e. The diffusion-weighted image (f) shows that the bilateral areas of high signal intensity in the basal ganglia have disappeared. High signal intensity is noticeable only in the insula and the thalamus in f. (g) Photomicrograph (original magnification, x20; hematoxylin and eosin stain) shows severe spongiform vacuolar degeneration and neuronal loss of the gray matter neuropil (ie, status spongiosus) in a specimen from the cerebral cortex.

 

View larger version (141K): [in this window] [in a new window] [Download PPT slide]   Figure 1f.  Sporadic Creutzfeldt-Jakob disease in a 72-year-old woman with left hemiparesis. (a–c) MR images obtained 7 weeks after the onset of dementia and 2 weeks before the appearance of periodic synchronous discharges on the EEG and a negative result at testing for the 14-3-3 protein level in cerebrospinal fluid. Axial T1-weighted (a) and axial T2-weighted (b) images show no abnormalities. The diffusion-weighted image (c) reveals symmetric bilateral areas of abnormal high signal intensity in the frontotemporal region of the cerebral cortex, caudate nuclei, and putamen. (d–f) MR images obtained after disease progression to akinetic mutism, 27 weeks after the onset of symptoms. The T1-weighted image (d) shows marked cerebral atrophy. The T2-weighted image (e) shows periventricular white-matter signal hyperintensity and caudate nucleus atrophy. Asymmetric bilateral areas of high signal intensity are visible in the basal ganglia in e. The diffusion-weighted image (f) shows that the bilateral areas of high signal intensity in the basal ganglia have disappeared. High signal intensity is noticeable only in the insula and the thalamus in f. (g) Photomicrograph (original magnification, x20; hematoxylin and eosin stain) shows severe spongiform vacuolar degeneration and neuronal loss of the gray matter neuropil (ie, status spongiosus) in a specimen from the cerebral cortex.

 

View larger version (166K): [in this window] [in a new window] [Download PPT slide]   Figure 1g.  Sporadic Creutzfeldt-Jakob disease in a 72-year-old woman with left hemiparesis. (a–c) MR images obtained 7 weeks after the onset of dementia and 2 weeks before the appearance of periodic synchronous discharges on the EEG and a negative result at testing for the 14-3-3 protein level in cerebrospinal fluid. Axial T1-weighted (a) and axial T2-weighted (b) images show no abnormalities. The diffusion-weighted image (c) reveals symmetric bilateral areas of abnormal high signal intensity in the frontotemporal region of the cerebral cortex, caudate nuclei, and putamen. (d–f) MR images obtained after disease progression to akinetic mutism, 27 weeks after the onset of symptoms. The T1-weighted image (d) shows marked cerebral atrophy. The T2-weighted image (e) shows periventricular white-matter signal hyperintensity and caudate nucleus atrophy. Asymmetric bilateral areas of high signal intensity are visible in the basal ganglia in e. The diffusion-weighted image (f) shows that the bilateral areas of high signal intensity in the basal ganglia have disappeared. High signal intensity is noticeable only in the insula and the thalamus in f. (g) Photomicrograph (original magnification, x20; hematoxylin and eosin stain) shows severe spongiform vacuolar degeneration and neuronal loss of the gray matter neuropil (ie, status spongiosus) in a specimen from the cerebral cortex.

 

View larger version (157K): [in this window] [in a new window] [Download PPT slide]   Figure 2a.  Sporadic Creutz-feldt-Jakob disease in a 65-year-old man 10 weeks after the onset of dementia, insomnia, and optical hallucinations and 4 weeks before the appearance of periodic synchronous discharges on the EEG. (a) Axial T2-weighted image shows no abnormalities. (b) Diffusion-weighted image shows bilateral areas of abnormal high signal intensity in the cerebral cortex, caudate nuclei, putamen, and thalamus.

 

View larger version (154K): [in this window] [in a new window] [Download PPT slide]   Figure 2b.  Sporadic Creutz-feldt-Jakob disease in a 65-year-old man 10 weeks after the onset of dementia, insomnia, and optical hallucinations and 4 weeks before the appearance of periodic synchronous discharges on the EEG. (a) Axial T2-weighted image shows no abnormalities. (b) Diffusion-weighted image shows bilateral areas of abnormal high signal intensity in the cerebral cortex, caudate nuclei, putamen, and thalamus.

 

View larger version (129K): [in this window] [in a new window] [Download PPT slide]   Figure 3a.  Iatrogenic Creutzfeldt-Jakob disease after a cadaveric dura mater transplantation in a 57-year-old woman. Images were obtained 7 weeks after the onset of progressive dementia and right hemiplegia and 4 weeks before the appearance of periodic synchronous discharges on the EEG. (a) Axial T2-weighted image shows no areas of abnormal signal intensity and only postoperative changes in the left parieto-occipital region. (b) Diffusion-weighted image shows areas of abnormal high signal intensity in the left cerebral cortex and the bilateral caudate nuclei. (c) Photomicrograph (original magnification, x20; hematoxylin and eosin stain) shows spongiform (vacuolar) degeneration of the gray matter neuropil in a specimen from the cerebral cortex.

 

View larger version (144K): [in this window] [in a new window] [Download PPT slide]   Figure 3b.  Iatrogenic Creutzfeldt-Jakob disease after a cadaveric dura mater transplantation in a 57-year-old woman. Images were obtained 7 weeks after the onset of progressive dementia and right hemiplegia and 4 weeks before the appearance of periodic synchronous discharges on the EEG. (a) Axial T2-weighted image shows no areas of abnormal signal intensity and only postoperative changes in the left parieto-occipital region. (b) Diffusion-weighted image shows areas of abnormal high signal intensity in the left cerebral cortex and the bilateral caudate nuclei. (c) Photomicrograph (original magnification, x20; hematoxylin and eosin stain) shows spongiform (vacuolar) degeneration of the gray matter neuropil in a specimen from the cerebral cortex.

 

View larger version (187K): [in this window] [in a new window] [Download PPT slide]   Figure 3c.  Iatrogenic Creutzfeldt-Jakob disease after a cadaveric dura mater transplantation in a 57-year-old woman. Images were obtained 7 weeks after the onset of progressive dementia and right hemiplegia and 4 weeks before the appearance of periodic synchronous discharges on the EEG. (a) Axial T2-weighted image shows no areas of abnormal signal intensity and only postoperative changes in the left parieto-occipital region. (b) Diffusion-weighted image shows areas of abnormal high signal intensity in the left cerebral cortex and the bilateral caudate nuclei. (c) Photomicrograph (original magnification, x20; hematoxylin and eosin stain) shows spongiform (vacuolar) degeneration of the gray matter neuropil in a specimen from the cerebral cortex.

 

View larger version (134K): [in this window] [in a new window] [Download PPT slide]   Figure 4a.  Sporadic Creutzfeldt-Jakob disease in a 62-year-old woman 3 weeks after the onset of progressive dementia and 6 weeks before the appearance of periodic synchronous discharges on the EEG. (a, b) Axial diffusion-weighted images at two different levels show areas of abnormal high signal intensity in the cerebral cortex on both sides. Subtle high-signal-intensity areas in the caudate nucleus and the anterior portion of the putamen also are visible. (c) Axial ADC map from diffusion-weighted imaging shows the cortical lesions as areas of decreased signal intensity, with the most striking decreases visible in the caudate nucleus and putamen.

 

View larger version (120K): [in this window] [in a new window] [Download PPT slide]   Figure 4b.  Sporadic Creutzfeldt-Jakob disease in a 62-year-old woman 3 weeks after the onset of progressive dementia and 6 weeks before the appearance of periodic synchronous discharges on the EEG. (a, b) Axial diffusion-weighted images at two different levels show areas of abnormal high signal intensity in the cerebral cortex on both sides. Subtle high-signal-intensity areas in the caudate nucleus and the anterior portion of the putamen also are visible. (c) Axial ADC map from diffusion-weighted imaging shows the cortical lesions as areas of decreased signal intensity, with the most striking decreases visible in the caudate nucleus and putamen.

 

View larger version (104K): [in this window] [in a new window] [Download PPT slide]   Figure 4c.  Sporadic Creutzfeldt-Jakob disease in a 62-year-old woman 3 weeks after the onset of progressive dementia and 6 weeks before the appearance of periodic synchronous discharges on the EEG. (a, b) Axial diffusion-weighted images at two different levels show areas of abnormal high signal intensity in the cerebral cortex on both sides. Subtle high-signal-intensity areas in the caudate nucleus and the anterior portion of the putamen also are visible. (c) Axial ADC map from diffusion-weighted imaging shows the cortical lesions as areas of decreased signal intensity, with the most striking decreases visible in the caudate nucleus and putamen.

 

View larger version (164K): [in this window] [in a new window] [Download PPT slide]   Figure 5a.  Sporadic Creutzfeldt-Jakob disease in a 67-year-old woman 9 weeks after the onset of progressive dementia and visual hallucinations and 9 weeks before the appearance of periodic synchronous discharges on the EEG and a negative result for 14-3-3 protein testing in the cerebrospinal fluid. (a) Axial T2-weighted MR image shows an area of subtle abnormal signal hyperintensity in the right temporo-occipital cortex. (b–d) Axial diffusion-weighted images show bilateral areas of abnormal high signal intensity at three different levels in the cerebral cortex. (e) Axial ADC map from diffusion-weighted imaging shows the lesions as areas of decreased signal intensity. (f) Coronal FLAIR image shows areas of abnormal high signal intensity in the cerebral cortex that correspond to but are less obvious than those observed on the diffusion-weighted images. (g) MR spectroscopic image reveals a decrease of N-acetylaspartate in the head of the right caudate nucleus. (h) Statistical parametric maps of brain perfusion, obtained with 99mTc-ECD SPECT 2 days before MR imaging, reveal areas of perfusion deficit that correspond to the areas of abnormal high signal intensity on diffusion-weighted images. However, these findings are nonspecific for Creutzfeldt-Jakob disease.

 

View larger version (94K): [in this window] [in a new window] [Download PPT slide]   Figure 5b.  Sporadic Creutzfeldt-Jakob disease in a 67-year-old woman 9 weeks after the onset of progressive dementia and visual hallucinations and 9 weeks before the appearance of periodic synchronous discharges on the EEG and a negative result for 14-3-3 protein testing in the cerebrospinal fluid. (a) Axial T2-weighted MR image shows an area of subtle abnormal signal hyperintensity in the right temporo-occipital cortex. (b–d) Axial diffusion-weighted images show bilateral areas of abnormal high signal intensity at three different levels in the cerebral cortex. (e) Axial ADC map from diffusion-weighted imaging shows the lesions as areas of decreased signal intensity. (f) Coronal FLAIR image shows areas of abnormal high signal intensity in the cerebral cortex that correspond to but are less obvious than those observed on the diffusion-weighted images. (g) MR spectroscopic image reveals a decrease of N-acetylaspartate in the head of the right caudate nucleus. (h) Statistical parametric maps of brain perfusion, obtained with 99mTc-ECD SPECT 2 days before MR imaging, reveal areas of perfusion deficit that correspond to the areas of abnormal high signal intensity on diffusion-weighted images. However, these findings are nonspecific for Creutzfeldt-Jakob disease.

 

View larger version (117K): [in this window] [in a new window] [Download PPT slide]   Figure 5c.  Sporadic Creutzfeldt-Jakob disease in a 67-year-old woman 9 weeks after the onset of progressive dementia and visual hallucinations and 9 weeks before the appearance of periodic synchronous discharges on the EEG and a negative result for 14-3-3 protein testing in the cerebrospinal fluid. (a) Axial T2-weighted MR image shows an area of subtle abnormal signal hyperintensity in the right temporo-occipital cortex. (b–d) Axial diffusion-weighted images show bilateral areas of abnormal high signal intensity at three different levels in the cerebral cortex. (e) Axial ADC map from diffusion-weighted imaging shows the lesions as areas of decreased signal intensity. (f) Coronal FLAIR image shows areas of abnormal high signal intensity in the cerebral cortex that correspond to but are less obvious than those observed on the diffusion-weighted images. (g) MR spectroscopic image reveals a decrease of N-acetylaspartate in the head of the right caudate nucleus. (h) Statistical parametric maps of brain perfusion, obtained with 99mTc-ECD SPECT 2 days before MR imaging, reveal areas of perfusion deficit that correspond to the areas of abnormal high signal intensity on diffusion-weighted images. However, these findings are nonspecific for Creutzfeldt-Jakob disease.

 

View larger version (130K): [in this window] [in a new window] [Download PPT slide]   Figure 5d.  Sporadic Creutzfeldt-Jakob disease in a 67-year-old woman 9 weeks after the onset of progressive dementia and visual hallucinations and 9 weeks before the appearance of periodic synchronous discharges on the EEG and a negative result for 14-3-3 protein testing in the cerebrospinal fluid. (a) Axial T2-weighted MR image shows an area of subtle abnormal signal hyperintensity in the right temporo-occipital cortex. (b–d) Axial diffusion-weighted images show bilateral areas of abnormal high signal intensity at three different levels in the cerebral cortex. (e) Axial ADC map from diffusion-weighted imaging shows the lesions as areas of decreased signal intensity. (f) Coronal FLAIR image shows areas of abnormal high signal intensity in the cerebral cortex that correspond to but are less obvious than those observed on the diffusion-weighted images. (g) MR spectroscopic image reveals a decrease of N-acetylaspartate in the head of the right caudate nucleus. (h) Statistical parametric maps of brain perfusion, obtained with 99mTc-ECD SPECT 2 days before MR imaging, reveal areas of perfusion deficit that correspond to the areas of abnormal high signal intensity on diffusion-weighted images. However, these findings are nonspecific for Creutzfeldt-Jakob disease.

 

View larger version (118K): [in this window] [in a new window] [Download PPT slide]   Figure 5e.  Sporadic Creutzfeldt-Jakob disease in a 67-year-old woman 9 weeks after the onset of progressive dementia and visual hallucinations and 9 weeks before the appearance of periodic synchronous discharges on the EEG and a negative result for 14-3-3 protein testing in the cerebrospinal fluid. (a) Axial T2-weighted MR image shows an area of subtle abnormal signal hyperintensity in the right temporo-occipital cortex. (b–d) Axial diffusion-weighted images show bilateral areas of abnormal high signal intensity at three different levels in the cerebral cortex. (e) Axial ADC map from diffusion-weighted imaging shows the lesions as areas of decreased signal intensity. (f) Coronal FLAIR image shows areas of abnormal high signal intensity in the cerebral cortex that correspond to but are less obvious than those observed on the diffusion-weighted images. (g) MR spectroscopic image reveals a decrease of N-acetylaspartate in the head of the right caudate nucleus. (h) Statistical parametric maps of brain perfusion, obtained with 99mTc-ECD SPECT 2 days before MR imaging, reveal areas of perfusion deficit that correspond to the areas of abnormal high signal intensity on diffusion-weighted images. However, these findings are nonspecific for Creutzfeldt-Jakob disease.

 

View larger version (157K): [in this window] [in a new window] [Download PPT slide]   Figure 5f.  Sporadic Creutzfeldt-Jakob disease in a 67-year-old woman 9 weeks after the onset of progressive dementia and visual hallucinations and 9 weeks before the appearance of periodic synchronous discharges on the EEG and a negative result for 14-3-3 protein testing in the cerebrospinal fluid. (a) Axial T2-weighted MR image shows an area of subtle abnormal signal hyperintensity in the right temporo-occipital cortex. (b–d) Axial diffusion-weighted images show bilateral areas of abnormal high signal intensity at three different levels in the cerebral cortex. (e) Axial ADC map from diffusion-weighted imaging shows the lesions as areas of decreased signal intensity. (f) Coronal FLAIR image shows areas of abnormal high signal intensity in the cerebral cortex that correspond to but are less obvious than those observed on the diffusion-weighted images. (g) MR spectroscopic image reveals a decrease of N-acetylaspartate in the head of the right caudate nucleus. (h) Statistical parametric maps of brain perfusion, obtained with 99mTc-ECD SPECT 2 days before MR imaging, reveal areas of perfusion deficit that correspond to the areas of abnormal high signal intensity on diffusion-weighted images. However, these findings are nonspecific for Creutzfeldt-Jakob disease.

 

View larger version (56K): [in this window] [in a new window] [Download PPT slide]   Figure 5g.  Sporadic Creutzfeldt-Jakob disease in a 67-year-old woman 9 weeks after the onset of progressive dementia and visual hallucinations and 9 weeks before the appearance of periodic synchronous discharges on the EEG and a negative result for 14-3-3 protein testing in the cerebrospinal fluid. (a) Axial T2-weighted MR image shows an area of subtle abnormal signal hyperintensity in the right temporo-occipital cortex. (b–d) Axial diffusion-weighted images show bilateral areas of abnormal high signal intensity at three different levels in the cerebral cortex. (e) Axial ADC map from diffusion-weighted imaging shows the lesions as areas of decreased signal intensity. (f) Coronal FLAIR image shows areas of abnormal high signal intensity in the cerebral cortex that correspond to but are less obvious than those observed on the diffusion-weighted images. (g) MR spectroscopic image reveals a decrease of N-acetylaspartate in the head of the right caudate nucleus. (h) Statistical parametric maps of brain perfusion, obtained with 99mTc-ECD SPECT 2 days before MR imaging, reveal areas of perfusion deficit that correspond to the areas of abnormal high signal intensity on diffusion-weighted images. However, these findings are nonspecific for Creutzfeldt-Jakob disease.

 

View larger version (103K): [in this window] [in a new window] [Download PPT slide]   Figure 5h.  Sporadic Creutzfeldt-Jakob disease in a 67-year-old woman 9 weeks after the onset of progressive dementia and visual hallucinations and 9 weeks before the appearance of periodic synchronous discharges on the EEG and a negative result for 14-3-3 protein testing in the cerebrospinal fluid. (a) Axial T2-weighted MR image shows an area of subtle abnormal signal hyperintensity in the right temporo-occipital cortex. (b–d) Axial diffusion-weighted images show bilateral areas of abnormal high signal intensity at three different levels in the cerebral cortex. (e) Axial ADC map from diffusion-weighted imaging shows the lesions as areas of decreased signal intensity. (f) Coronal FLAIR image shows areas of abnormal high signal intensity in the cerebral cortex that correspond to but are less obvious than those observed on the diffusion-weighted images. (g) MR spectroscopic image reveals a decrease of N-acetylaspartate in the head of the right caudate nucleus. (h) Statistical parametric maps of brain perfusion, obtained with 99mTc-ECD SPECT 2 days before MR imaging, reveal areas of perfusion deficit that correspond to the areas of abnormal high signal intensity on diffusion-weighted images. However, these findings are nonspecific for Creutzfeldt-Jakob disease.

 

View larger version (162K): [in this window] [in a new window] [Download PPT slide]   Figure 6a.  Sporadic Creutzfeldt-Jakob disease in a 71-year-old woman 3 weeks after the onset of progressive insomnia and left hemiparesis and 8 weeks before the appearance of periodic synchronous discharges on the EEG. (a) Axial T2-weighted MR image shows no abnormalities. (b) Diffusion-weighted image shows areas of abnormal high signal intensity in the right cerebral cortex. (c) Axial ADC map from diffusion-weighted imaging shows the lesions as areas of decreased signal intensity.

 

View larger version (140K): [in this window] [in a new window] [Download PPT slide]   Figure 6b.  Sporadic Creutzfeldt-Jakob disease in a 71-year-old woman 3 weeks after the onset of progressive insomnia and left hemiparesis and 8 weeks before the appearance of periodic synchronous discharges on the EEG. (a) Axial T2-weighted MR image shows no abnormalities. (b) Diffusion-weighted image shows areas of abnormal high signal intensity in the right cerebral cortex. (c) Axial ADC map from diffusion-weighted imaging shows the lesions as areas of decreased signal intensity.

 

View larger version (119K): [in this window] [in a new window] [Download PPT slide]   Figure 6c.  Sporadic Creutzfeldt-Jakob disease in a 71-year-old woman 3 weeks after the onset of progressive insomnia and left hemiparesis and 8 weeks before the appearance of periodic synchronous discharges on the EEG. (a) Axial T2-weighted MR image shows no abnormalities. (b) Diffusion-weighted image shows areas of abnormal high signal intensity in the right cerebral cortex. (c) Axial ADC map from diffusion-weighted imaging shows the lesions as areas of decreased signal intensity.

 

View larger version (101K): [in this window] [in a new window] [Download PPT slide]   Figure 7a.  MELAS in a 41-year-old woman with subacute progressive personality changes, depression, and confusion. (a–c) Axial diffusion-weighted images at three different levels show regions of abnormal high signal intensity restricted to the cerebral cortex, similar to our findings in patients with Creutzfeldt-Jakob disease. (d, e) Axial T2-weighted image (d) and coronal FLAIR image (e) show lesions with severe gyriform swelling that does not extend to the underlying white matter. These features are never seen in early-stage Creutzfeldt-Jakob disease.

 

View larger version (109K): [in this window] [in a new window] [Download PPT slide]   Figure 7b.  MELAS in a 41-year-old woman with subacute progressive personality changes, depression, and confusion. (a–c) Axial diffusion-weighted images at three different levels show regions of abnormal high signal intensity restricted to the cerebral cortex, similar to our findings in patients with Creutzfeldt-Jakob disease. (d, e) Axial T2-weighted image (d) and coronal FLAIR image (e) show lesions with severe gyriform swelling that does not extend to the underlying white matter. These features are never seen in early-stage Creutzfeldt-Jakob disease.

 

View larger version (107K): [in this window] [in a new window] [Download PPT slide]   Figure 7c.  MELAS in a 41-year-old woman with subacute progressive personality changes, depression, and confusion. (a–c) Axial diffusion-weighted images at three different levels show regions of abnormal high signal intensity restricted to the cerebral cortex, similar to our findings in patients with Creutzfeldt-Jakob disease. (d, e) Axial T2-weighted image (d) and coronal FLAIR image (e) show lesions with severe gyriform swelling that does not extend to the underlying white matter. These features are never seen in early-stage Creutzfeldt-Jakob disease.

 

View larger version (157K): [in this window] [in a new window] [Download PPT slide]   Figure 7d.  MELAS in a 41-year-old woman with subacute progressive personality changes, depression, and confusion. (a–c) Axial diffusion-weighted images at three different levels show regions of abnormal high signal intensity restricted to the cerebral cortex, similar to our findings in patients with Creutzfeldt-Jakob disease. (d, e) Axial T2-weighted image (d) and coronal FLAIR image (e) show lesions with severe gyriform swelling that does not extend to the underlying white matter. These features are never seen in early-stage Creutzfeldt-Jakob disease.

 

View larger version (154K): [in this window] [in a new window] [Download PPT slide]   Figure 7e.  MELAS in a 41-year-old woman with subacute progressive personality changes, depression, and confusion. (a–c) Axial diffusion-weighted images at three different levels show regions of abnormal high signal intensity restricted to the cerebral cortex, similar to our findings in patients with Creutzfeldt-Jakob disease. (d, e) Axial T2-weighted image (d) and coronal FLAIR image (e) show lesions with severe gyriform swelling that does not extend to the underlying white matter. These features are never seen in early-stage Creutzfeldt-Jakob disease.

 

View larger version (133K): [in this window] [in a new window] [Download PPT slide]   Figure 8a.  Venous hypertensive encephalopathy due to a dural arteriovenous fistula in a 62-year-old woman with a subacute progressive visual disturbance and optical hallucination. (a) Diffusion-weighted image obtained at admission reveals areas of abnormal high signal intensity in the left medial cerebral cortex that resemble findings in Creutzfeldt-Jakob disease. A dural arteriovenous fistula was diagnosed at gadolinium-enhanced MR angiography, and transarterial embolization was performed. (b) Diffusion-weighted image obtained after treatment shows no signal intensity abnormalities. As commonly occurs in venous hypertensive encephalopathy, the symptoms resolved after treatment. Early diagnosis and prompt treatment are important for successful resolution.

 

View larger version (128K): [in this window] [in a new window] [Download PPT slide]   Figure 8b.  Venous hypertensive encephalopathy due to a dural arteriovenous fistula in a 62-year-old woman with a subacute progressive visual disturbance and optical hallucination. (a) Diffusion-weighted image obtained at admission reveals areas of abnormal high signal intensity in the left medial cerebral cortex that resemble findings in Creutzfeldt-Jakob disease. A dural arteriovenous fistula was diagnosed at gadolinium-enhanced MR angiography, and transarterial embolization was performed. (b) Diffusion-weighted image obtained after treatment shows no signal intensity abnormalities. As commonly occurs in venous hypertensive encephalopathy, the symptoms resolved after treatment. Early diagnosis and prompt treatment are important for successful resolution.

 

View larger version (128K): [in this window] [in a new window] [Download PPT slide]   Figure 9a.  Chronic herpes simplex encephalitis in a 74-year-old man with a 3-week history of progressive dementia, convulsions, and consciousness disturbance, symptoms that improved after treatment with acyclovir. (a) Diffusion-weighted image reveals a high-signal-intensity abnormality in the right medial temporal region. (b) T2-weighted image shows an area of abnormal high signal intensity corresponding to the abnormality in a, as well as parenchymal swelling. (c) Gadolinium-enhanced T1-weighted image shows the lesion with parenchymal swelling, a feature that is never observed in Creutzfeldt-Jakob disease. Note the absence of the patchy or gyriform enhancement that is seen in cases of acute herpes simplex encephalitis.

 

View larger version (145K): [in this window] [in a new window] [Download PPT slide]   Figure 9b.  Chronic herpes simplex encephalitis in a 74-year-old man with a 3-week history of progressive dementia, convulsions, and consciousness disturbance, symptoms that improved after treatment with acyclovir. (a) Diffusion-weighted image reveals a high-signal-intensity abnormality in the right medial temporal region. (b) T2-weighted image shows an area of abnormal high signal intensity corresponding to the abnormality in a, as well as parenchymal swelling. (c) Gadolinium-enhanced T1-weighted image shows the lesion with parenchymal swelling, a feature that is never observed in Creutzfeldt-Jakob disease. Note the absence of the patchy or gyriform enhancement that is seen in cases of acute herpes simplex encephalitis.

 

View larger version (132K): [in this window] [in a new window] [Download PPT slide]   Figure 9c.  Chronic herpes simplex encephalitis in a 74-year-old man with a 3-week history of progressive dementia, convulsions, and consciousness disturbance, symptoms that improved after treatment with acyclovir. (a) Diffusion-weighted image reveals a high-signal-intensity abnormality in the right medial temporal region. (b) T2-weighted image shows an area of abnormal high signal intensity corresponding to the abnormality in a, as well as parenchymal swelling. (c) Gadolinium-enhanced T1-weighted image shows the lesion with parenchymal swelling, a feature that is never observed in Creutzfeldt-Jakob disease. Note the absence of the patchy or gyriform enhancement that is seen in cases of acute herpes simplex encephalitis.

 

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