The Experts below are selected from a list of 6 Experts worldwide ranked by ideXlab platform
Youngsoon Yang - One of the best experts on this subject based on the ideXlab platform.
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Posterior Cortical Atrophy with Acute Onset and Rapid Progressive Visual Symptoms: A Case Report
Dementia and Neurocognitive Disorders, 2015Co-Authors: In Ha Hwang, Kyum Il Kwon, Youngsoon YangAbstract:Posterior Cortical atrophy (PCA) is a rare degenerative disease with unknown cause. In 1988, Benson et al.1 reported some patients whose cerebral images showed significant atrophy of the parieto-occipital region with progressive dementia and Cortical visual dysfunction. They named this disease as PCA based on image findings. Since PCA is most often associated with the pathological findings of Alzheimer’s disease (AD), some people consider PCA as a visual variant of AD. Because PCA invades the posterior cerebral cortex, Gerstmann syndrome, Balint syndrome, and trans-Cortical Sensory Aphasia may occur along with visual agnosia that occurs relatively early.2,3 Here we report a patient who visited our hospital with complaints of only visual symptom that rapidly progressed. We diagnosed it as PCA through clinical findings, image examinations, and other evidences. CASE REPORT
In Ha Hwang - One of the best experts on this subject based on the ideXlab platform.
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Posterior Cortical Atrophy with Acute Onset and Rapid Progressive Visual Symptoms: A Case Report
Dementia and Neurocognitive Disorders, 2015Co-Authors: In Ha Hwang, Kyum Il Kwon, Youngsoon YangAbstract:Posterior Cortical atrophy (PCA) is a rare degenerative disease with unknown cause. In 1988, Benson et al.1 reported some patients whose cerebral images showed significant atrophy of the parieto-occipital region with progressive dementia and Cortical visual dysfunction. They named this disease as PCA based on image findings. Since PCA is most often associated with the pathological findings of Alzheimer’s disease (AD), some people consider PCA as a visual variant of AD. Because PCA invades the posterior cerebral cortex, Gerstmann syndrome, Balint syndrome, and trans-Cortical Sensory Aphasia may occur along with visual agnosia that occurs relatively early.2,3 Here we report a patient who visited our hospital with complaints of only visual symptom that rapidly progressed. We diagnosed it as PCA through clinical findings, image examinations, and other evidences. CASE REPORT
Kyum Il Kwon - One of the best experts on this subject based on the ideXlab platform.
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Posterior Cortical Atrophy with Acute Onset and Rapid Progressive Visual Symptoms: A Case Report
Dementia and Neurocognitive Disorders, 2015Co-Authors: In Ha Hwang, Kyum Il Kwon, Youngsoon YangAbstract:Posterior Cortical atrophy (PCA) is a rare degenerative disease with unknown cause. In 1988, Benson et al.1 reported some patients whose cerebral images showed significant atrophy of the parieto-occipital region with progressive dementia and Cortical visual dysfunction. They named this disease as PCA based on image findings. Since PCA is most often associated with the pathological findings of Alzheimer’s disease (AD), some people consider PCA as a visual variant of AD. Because PCA invades the posterior cerebral cortex, Gerstmann syndrome, Balint syndrome, and trans-Cortical Sensory Aphasia may occur along with visual agnosia that occurs relatively early.2,3 Here we report a patient who visited our hospital with complaints of only visual symptom that rapidly progressed. We diagnosed it as PCA through clinical findings, image examinations, and other evidences. CASE REPORT
Hiroshi Hayashi - One of the best experts on this subject based on the ideXlab platform.
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Typical and atypical appearance of early‐onset Alzheimer's disease: A clinical, neuroimaging and neuropathological study
Neuropathology, 2017Co-Authors: Shinobu Kawakatsu, Ryota Kobayashi, Hiroshi HayashiAbstract:The International Working Group (IWG) has classified Alzheimer's disease (AD) as two different types, the typical form and the atypical form, but clinicopathological studies of atypical AD are limited. Because atypical AD cases usually present with early-onset dementia, we investigated 12 patients with early-onset AD, including two patients with typical AD and 10 patients with atypical AD. Of these patients, six had the posterior variant, three had the frontal variant and one had the logopenic variant mixed with semantic dementia. We reported MRI, single-photon emission CT and neuropathological findings in six representative cases. We also described a “left temporal variant” of AD presenting with transCortical Cortical Sensory Aphasia, which has not been reported previously and is another subtype of the posterior variant of AD. We found a significant correlation between regional cerebral blood flow and counts of NFTs in the cerebral cortices. An atypical presentation with focal neuropsychological symptoms roughly correlated with the density of NFTs in the cerebral cortex and more directly related to spongiform changes in the superficial layers of these areas. In contrast, the distribution of amyloid depositions was diffuse and did not necessarily correlate with focal neuropsychological symptoms. Braak staging or ABC score is not necessarily appropriate to evaluate atypical AD, and instead, spongiform changes in addition to tau pathology in the association cortices better explain the diversity of atypical AD. Interestingly, another patient with a posterior variant of AD had a novel type of atypical plaque, which we referred to as “lucent plaque”. They were recognizable with HE staining in the circumference and dystrophic neurites were abundant with Gallyas-Braak staining. These plaques demonstrated intense immunoreactivity to both tau AT-8 and amyloid β (Aβ), suggesting a peculiar coexistence pattern of amyloid and tau in these plaques. Clinicopathological studies of atypical AD will provide a new understanding of the pathophysiology of AD.
Shinobu Kawakatsu - One of the best experts on this subject based on the ideXlab platform.
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Typical and atypical appearance of early‐onset Alzheimer's disease: A clinical, neuroimaging and neuropathological study
Neuropathology, 2017Co-Authors: Shinobu Kawakatsu, Ryota Kobayashi, Hiroshi HayashiAbstract:The International Working Group (IWG) has classified Alzheimer's disease (AD) as two different types, the typical form and the atypical form, but clinicopathological studies of atypical AD are limited. Because atypical AD cases usually present with early-onset dementia, we investigated 12 patients with early-onset AD, including two patients with typical AD and 10 patients with atypical AD. Of these patients, six had the posterior variant, three had the frontal variant and one had the logopenic variant mixed with semantic dementia. We reported MRI, single-photon emission CT and neuropathological findings in six representative cases. We also described a “left temporal variant” of AD presenting with transCortical Cortical Sensory Aphasia, which has not been reported previously and is another subtype of the posterior variant of AD. We found a significant correlation between regional cerebral blood flow and counts of NFTs in the cerebral cortices. An atypical presentation with focal neuropsychological symptoms roughly correlated with the density of NFTs in the cerebral cortex and more directly related to spongiform changes in the superficial layers of these areas. In contrast, the distribution of amyloid depositions was diffuse and did not necessarily correlate with focal neuropsychological symptoms. Braak staging or ABC score is not necessarily appropriate to evaluate atypical AD, and instead, spongiform changes in addition to tau pathology in the association cortices better explain the diversity of atypical AD. Interestingly, another patient with a posterior variant of AD had a novel type of atypical plaque, which we referred to as “lucent plaque”. They were recognizable with HE staining in the circumference and dystrophic neurites were abundant with Gallyas-Braak staining. These plaques demonstrated intense immunoreactivity to both tau AT-8 and amyloid β (Aβ), suggesting a peculiar coexistence pattern of amyloid and tau in these plaques. Clinicopathological studies of atypical AD will provide a new understanding of the pathophysiology of AD.
