Neuropsychological, Neuroimaging, and Neuropathological Correlations in Neurodegenerative Diseases : a Comparison of Left Posterior Temporal Variant Alzheimerʼs Disease and Semantic Dementia with TDP type C and Alzheimerʼs Disease Neuropathologic Changes.

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  • Kawakatsu Shinobu
    Department of Neuropsychiatry, Aizu Medical Center, Fukushima Medical University.
  • Kobayashi Ryota
    Department of Psychiatry, Yamagata University Faculty of Medicine
  • Morioka Daichi
    Department of Psychiatry, Yamagata University Faculty of Medicine
  • Sakamoto Kazutaka
    Department of Psychiatry, Yamagata University Faculty of Medicine
  • Hayashi Hiroshi
    Department of Occupational Therapy, School of Health Sciences Fukushima Medical University
  • Suzuki Akihito
    Department of Psychiatry, Yamagata University Faculty of Medicine

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  • 神経変性疾患における高次脳機能障害と画像・病理─左後部側頭葉型アルツハイマー病とアルツハイマー病理を伴う TDP タイプ C の意味性認知症の比較─

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Abstract

<p>  Case 1 involved a patient with the left posterior temporal variant of Alzheimerʼs disease. The patient initially developed amnestic aphasia and progressed to transcortical sensory aphasia, which ultimately resulted in jargon aphasia. Magnetic resonance imaging (MRI) and single-photon emission computed tomography (SPECT) showed left-predominant atrophy and hypoperfusion in the posterior temporal and parietal cortices. Neuronal loss with spongiform changes, abundant neurofibrillary tangles (NFTs) , neuropil threads, and neuritic plaques were observed in the temporal cortex with a left-sided predominant distribution. Case 2 involved a patient with semantic dementia, typical gogi-aphasia, and behavioral disturbances. The patient had no apparent left anterior temporal atrophy on MRI during the early stage of the illness. Neuronal loss with spongiform changes was observed in the left temporal cortex. The presence of phosphorylated TAR-binding DNA protein of 43 kDa (TDP-43) positive long dystrophic neurites (DNs) , which was consistent with TDP type C pathology, was observed in the frontal and parietal cortices, instead of the temporal cortex. The regions with TDP type C pathology were spared. Conversely, lesions with severe degeneration had few long DNs. Moderate amyloid pathology and mild NFTs (A2B1C2) were also observed ; however, these lesions were considered unrelated to the patientʼs symptoms. Since neuronal loss and spongiform changes corresponded to the focal neuropsychological symptoms in neurodegenerative diseases, these pathological findings serve as neuroimaging marker for establishing the diagnosis and treatment this disease.</p>

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