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Kurt A. Jellinger - One of the best experts on this subject based on the ideXlab platform.

  • Absence of α-synuclein pathology in Postencephalitic Parkinsonism
    Acta Neuropathologica, 2009
    Co-Authors: Kurt A. Jellinger
    Abstract:

    Postencephalitic Parkinsonism (PEP), a chronic complication of encephalitis lethargica, is a tauopathy characterized by multisystem neuronal loss and gliosis with widespread neurofibrillary lesions composed of both 3- and 4-repeat (3R and 4R) tau isoforms. Previous immunohistochemical studies in a small number of PEP cases demonstrated absence of Lewy bodies as well as the lack of other α-synuclein pathology, classifying PEP as a “pure” tauopathy. Neuropathologic examination of 10 brains with clinico-pathologically verified PEP confirmed widespread neurodegeneration in subcortical and brainstem areas associated with multifocal neurofibrillary pathology comprising both 3R and 4R tau. Very rare β-amyloid deposits were observed in two elderly patients, while Lewy bodies and neurites or any other α-synuclein deposits were completely absent. The causes and molecular background of total absence of α-synuclein pathology in PEP, in contrast to most other tauopathies, remain as unknown as the pathogenesis of PEP.

  • Absence of α-synuclein pathology in Postencephalitic Parkinsonism
    Acta neuropathologica, 2009
    Co-Authors: Kurt A. Jellinger
    Abstract:

    Postencephalitic Parkinsonism (PEP), a chronic complication of encephalitis lethargica, is a tauopathy characterized by multisystem neuronal loss and gliosis with widespread neurofibrillary lesions composed of both 3- and 4-repeat (3R and 4R) tau isoforms. Previous immunohistochemical studies in a small number of PEP cases demonstrated absence of Lewy bodies as well as the lack of other alpha-synuclein pathology, classifying PEP as a "pure" tauopathy. Neuropathologic examination of 10 brains with clinico-pathologically verified PEP confirmed widespread neurodegeneration in subcortical and brainstem areas associated with multifocal neurofibrillary pathology comprising both 3R and 4R tau. Very rare beta-amyloid deposits were observed in two elderly patients, while Lewy bodies and neurites or any other alpha-synuclein deposits were completely absent. The causes and molecular background of total absence of alpha-synuclein pathology in PEP, in contrast to most other tauopathies, remain as unknown as the pathogenesis of PEP.

  • Accuracy of the clinical diagnosis of Postencephalitic Parkinsonism: a clinicopathologic study.
    European journal of neurology, 1998
    Co-Authors: Irene Litvan, Ann C Mckee, Kurt A. Jellinger, Joseph Jankovic, Christopher G. Goetz, Gregory K. Wenning, Narahary Sastry, Eugene C. Lai, Jean Philippe Brandel, Marc Verny
    Abstract:

    The accuracy of the clinical diagnosis of Postencephalitic Parkinsonism (PEP) is unknown. We determined the validity of the clinical diagnosis of PEP by presenting 105 records with neuropathologic diagnoses of PEP (n = 7), progressive supranuclear palsy (n = 24), Parkinson's disease (n = 15), dementia with Lewy bodies (n = 14), multiple system atrophy (n = 16), corticobasal degeneration (n = 10), Creutzfeldt-Jakob disease (n = 4), and other dementia disorders (n = 15), as clinical vignettes to six neurologists unaware of the autopsy findings. The neurologists' own clinical diagnoses were compared with neuropathologic diagnoses for measures of diagnostic accuracy, including reliability (kappa statistics), sensitivity and positive predictive values for the first and last visits. The group reliability for the diagnosis of PEP was almost perfect (kappa = 0.91, 0.9). The mean sensitivity at the first visit was 86% (range, 71-100%) with minimal change at the last visit (83%; range, 71-100%). Positive predictive values remained unchanged (100%). The high reliability, sensitivity and positive predictive values of the clinical diagnosis of PEP indicate that neurologists identify this disorder even when they report that they have never evaluated a case. In our data set, the best predictors for the diagnosis of PEP included onset below middle age; symptom duration lasting more than 10 years, and the presence of oculogyric crisis. History of encephalitis lethargica, present in most PEP cases, was an important individual diagnostic predictor. Copyright 1998 Lippincott Williams & Wilkins

Irene Litvan - One of the best experts on this subject based on the ideXlab platform.

  • Accuracy of the clinical diagnosis of Postencephalitic Parkinsonism: a clinicopathologic study.
    European journal of neurology, 1998
    Co-Authors: Irene Litvan, Ann C Mckee, Kurt A. Jellinger, Joseph Jankovic, Christopher G. Goetz, Gregory K. Wenning, Narahary Sastry, Eugene C. Lai, Jean Philippe Brandel, Marc Verny
    Abstract:

    The accuracy of the clinical diagnosis of Postencephalitic Parkinsonism (PEP) is unknown. We determined the validity of the clinical diagnosis of PEP by presenting 105 records with neuropathologic diagnoses of PEP (n = 7), progressive supranuclear palsy (n = 24), Parkinson's disease (n = 15), dementia with Lewy bodies (n = 14), multiple system atrophy (n = 16), corticobasal degeneration (n = 10), Creutzfeldt-Jakob disease (n = 4), and other dementia disorders (n = 15), as clinical vignettes to six neurologists unaware of the autopsy findings. The neurologists' own clinical diagnoses were compared with neuropathologic diagnoses for measures of diagnostic accuracy, including reliability (kappa statistics), sensitivity and positive predictive values for the first and last visits. The group reliability for the diagnosis of PEP was almost perfect (kappa = 0.91, 0.9). The mean sensitivity at the first visit was 86% (range, 71-100%) with minimal change at the last visit (83%; range, 71-100%). Positive predictive values remained unchanged (100%). The high reliability, sensitivity and positive predictive values of the clinical diagnosis of PEP indicate that neurologists identify this disorder even when they report that they have never evaluated a case. In our data set, the best predictors for the diagnosis of PEP included onset below middle age; symptom duration lasting more than 10 years, and the presence of oculogyric crisis. History of encephalitis lethargica, present in most PEP cases, was an important individual diagnostic predictor. Copyright 1998 Lippincott Williams & Wilkins

  • Supranuclear gaze palsy and eyelid apraxia in Postencephalitic Parkinsonism
    Journal of Neural Transmission, 1997
    Co-Authors: G. K. Wenning, K. Jellinger, Irene Litvan
    Abstract:

    We describe six patients with clinicopathologically confirmed Postencephalitic Parkinsonism (PEP) in whom oculomotor abnormalities developed several years after suffering the initial episode of encephalitis lethargica. Four of the cases had vertical supranuclear gaze palsy and two eyelid apraxia, features typically associated with progressive supranuclear palsy (PSP). Our findings indicate that the presence of gaze palsy alone may not be a reliable clinical discriminator between PEP and PSP. Involvement of the dorsal central gray nucleus, nucleus centralis pontis oralis, nucleus dorsal raphe interpositus, rostral interstitial nucleus of the medial longitudinal fasciculus (riMLF), nucleus interstitialis of Cajal, nucleus of the posterior commissure, pedunculopontine nuclei and frontal cortex was observed in several of our PEP cases and may contribute to the oculomotor abnormalities in this disorder. Whether the dorsal tegmental nucleus, caudal to the supratrochlear nucleus, severely affected in all our PEP cases, has a role in vertical gaze needs to be further studied.

  • Validity and reliability of the preliminary NINDS neuropathologic criteria for progressive supranuclear palsy and related disorders.
    Journal of Neuropathology and Experimental Neurology, 1996
    Co-Authors: Irene Litvan, John J. Bartko, Christian Bancher, Susan E. Daniel, Peter L. Lantos, Dikran S. Horoupian, Jean-jacques Hauw, Dennis W Dickson, Ann C Mckee, Massimo Tabaton
    Abstract:

    We investigated the validity and reliability of diagnoses made by eight neuropathologists who used the preliminary NINDS neuropathologic diagnostic criteria for progressive supranuclear palsy (PSP) and related disorders. The specific disorders were typical, atypical, and combined PSP, Postencephalitic Parkinsonism, corticobasal ganglionic degeneration, and Pick's disease. These disorders were chosen because of the difficulties in their neuropathologic differentiation. We assessed validity by measuring sensitivity and positive predictive value. Reliability was evaluated by measuring pairwise and group agreement. From a total of 62 histologic cases, each neuropathologist independently classified 16 to 19 cases for the pairwise analysis and 5 to 6 cases for the group analysis. The neuropathologists were unaware of the study design, unfamiliar with the assigned cases, and initially had no clinical information about the cases. Our results showed that with routine sampling and staining methods, neuropathologic examination alone was not fully adequate for differentiating the disorders. The main difficulties were discriminating the subtypes of PSP and separating Postencephalitic Parkinsonism from PSP. Corticobasal ganglionic degeneration and Pick's disease were less difficult to distinguish from PSP. The addition of minimal clinical information contributed to the accuracy of the diagnosis. On the basis of results obtained, we propose clinicopathologic diagnostic criteria to improve on the NINDS criteria.

Neziha Gouider-khouja - One of the best experts on this subject based on the ideXlab platform.

Kenji Kosaka - One of the best experts on this subject based on the ideXlab platform.

  • a neuropathologic study of long term economo type Postencephalitic Parkinsonism with a prolonged clinical course
    Psychiatry and Clinical Neurosciences, 1996
    Co-Authors: Katsuyoshi Mizukami, Kenji Ikeda, Megumi Sasaki, Hiroyasu Shiraishi, Kenji Kosaka
    Abstract:

    In this report, the neuropathologic features of five autopsied cases of Postencephalitic Parkinsonism of the Economo-type (PEPE) with a mean age of 66.6 years and a mean duration of the illness of 53.6 years are described. All five patients had presented with personality changes and severe Parkinsonism. In addition, four patients had also had ocular symptoms. A pronounced chronic progression of the symptoms characterized all five cases. Active degenerating lesions were found in the substantia nigra (patients 3, 4 and 5) and the oculomotor nucleus (patient 5) which might explain the clinical observation of chronic active disease in these patients. We found that the intraneuronal neurofibrillary tangles (NFT) were immunoreactive to paired helical filaments (PHF), tau and ubiquitin; but ghost tangles demonstrated immunoreactivity only to glial fibrillary acid protein (GFAP). The ghost tangles consisted of dispersed bundles of abnormal tubules, and electron-dense glial filaments would surround and occasionally invade the ghost tangles. The present study suggests that NFT in PEPE are similar in their immunohistochemistry and ultrastructure to those observed in the case of Alzheimer-type dementia.

  • A neuropathologic study of long‐term, Economo‐type Postencephalitic Parkinsonism with a prolonged clinical course
    Psychiatry and clinical neurosciences, 1996
    Co-Authors: Katsuyoshi Mizukami, Kenji Ikeda, Megumi Sasaki, Hiroyasu Shiraishi, Kenji Kosaka
    Abstract:

    In this report, the neuropathologic features of five autopsied cases of Postencephalitic Parkinsonism of the Economo-type (PEPE) with a mean age of 66.6 years and a mean duration of the illness of 53.6 years are described. All five patients had presented with personality changes and severe Parkinsonism. In addition, four patients had also had ocular symptoms. A pronounced chronic progression of the symptoms characterized all five cases. Active degenerating lesions were found in the substantia nigra (patients 3, 4 and 5) and the oculomotor nucleus (patient 5) which might explain the clinical observation of chronic active disease in these patients. We found that the intraneuronal neurofibrillary tangles (NFT) were immunoreactive to paired helical filaments (PHF), tau and ubiquitin; but ghost tangles demonstrated immunoreactivity only to glial fibrillary acid protein (GFAP). The ghost tangles consisted of dispersed bundles of abnormal tubules, and electron-dense glial filaments would surround and occasionally invade the ghost tangles. The present study suggests that NFT in PEPE are similar in their immunohistochemistry and ultrastructure to those observed in the case of Alzheimer-type dementia.

  • Peculiar axonal debris with subsequent astrocytic response (foamy spheroid body)
    Virchows Archiv A, 1992
    Co-Authors: Nobutaka Arai, Kenji Kosaka, Toshio Mizutani, Saburo Yagishita, Kazuaki Misugi, Masaya Oda, Yoshio Morimatsu
    Abstract:

    Foamy spheroid bodies (FSBs) are described, as newly identified pathological structures occurring in human brain. FSBs favoured the substantia nigra pars reticulata (SNPR) and/or globus pallidus (GP) in degenerative conditions especially Postencephalitic Parkinsonism, progressive supranuclear palsy, pallido-nigro-luysial atrophy and multiple system atrophy. No FSBs were observed anywhere in the presence of substantia nigra pars compacta (SNPC) degeneration, such as occurs in idiopathic Parkinson's disease, or luysio-pallidal system degeneration, such as found in dentato-rubro-pallidoluysial atrophy or Joseph's disease. FSBs were also occasionally identified in the substantia nigra (SN) and/or GP of aged persons. In addition to SN and GP lesions, FSBs were seen in diffuse axonal lesions of long fibre tracts (the corpus callosum, the superior cerebellar peduncle) after non-missile head injuries, and in peri-infarct lesions. Under the light microscope, FSBs appear as slightly eosinophilic, foamy and nearly round objects with vague outlines, measuring approximately 10–50 μm in diameter. Some FSBs contain coarse, eosinophilic clusters at their periphery. FSB stained black when stained by the Gallyas silver method. Some FSBs were immunohistochemically positive for synaptophysin and 68 kDa neurofilament. Glial fibrillary acidic proteinpositive fibres were observed alongside and/or inside some FSBs. Electron microscopically, FSBs were found to consist of collections of neuritic debris containing a variety of dense bodies and a small number of both mitochondria and neurofilaments. Some such collections were surrounded by astrocytic processes. These findings strongly suggest that FSBs are collections of small axonal debris destined for removal by astrocytes in due course. A variety of factors (degeneration of the SNPR and/or the GP, injury, infarction, ageing) seemed to be responsible for the histogenesis of FSBs.

Kenji Ikeda - One of the best experts on this subject based on the ideXlab platform.

  • a neuropathologic study of long term economo type Postencephalitic Parkinsonism with a prolonged clinical course
    Psychiatry and Clinical Neurosciences, 1996
    Co-Authors: Katsuyoshi Mizukami, Kenji Ikeda, Megumi Sasaki, Hiroyasu Shiraishi, Kenji Kosaka
    Abstract:

    In this report, the neuropathologic features of five autopsied cases of Postencephalitic Parkinsonism of the Economo-type (PEPE) with a mean age of 66.6 years and a mean duration of the illness of 53.6 years are described. All five patients had presented with personality changes and severe Parkinsonism. In addition, four patients had also had ocular symptoms. A pronounced chronic progression of the symptoms characterized all five cases. Active degenerating lesions were found in the substantia nigra (patients 3, 4 and 5) and the oculomotor nucleus (patient 5) which might explain the clinical observation of chronic active disease in these patients. We found that the intraneuronal neurofibrillary tangles (NFT) were immunoreactive to paired helical filaments (PHF), tau and ubiquitin; but ghost tangles demonstrated immunoreactivity only to glial fibrillary acid protein (GFAP). The ghost tangles consisted of dispersed bundles of abnormal tubules, and electron-dense glial filaments would surround and occasionally invade the ghost tangles. The present study suggests that NFT in PEPE are similar in their immunohistochemistry and ultrastructure to those observed in the case of Alzheimer-type dementia.

  • A neuropathologic study of long‐term, Economo‐type Postencephalitic Parkinsonism with a prolonged clinical course
    Psychiatry and clinical neurosciences, 1996
    Co-Authors: Katsuyoshi Mizukami, Kenji Ikeda, Megumi Sasaki, Hiroyasu Shiraishi, Kenji Kosaka
    Abstract:

    In this report, the neuropathologic features of five autopsied cases of Postencephalitic Parkinsonism of the Economo-type (PEPE) with a mean age of 66.6 years and a mean duration of the illness of 53.6 years are described. All five patients had presented with personality changes and severe Parkinsonism. In addition, four patients had also had ocular symptoms. A pronounced chronic progression of the symptoms characterized all five cases. Active degenerating lesions were found in the substantia nigra (patients 3, 4 and 5) and the oculomotor nucleus (patient 5) which might explain the clinical observation of chronic active disease in these patients. We found that the intraneuronal neurofibrillary tangles (NFT) were immunoreactive to paired helical filaments (PHF), tau and ubiquitin; but ghost tangles demonstrated immunoreactivity only to glial fibrillary acid protein (GFAP). The ghost tangles consisted of dispersed bundles of abnormal tubules, and electron-dense glial filaments would surround and occasionally invade the ghost tangles. The present study suggests that NFT in PEPE are similar in their immunohistochemistry and ultrastructure to those observed in the case of Alzheimer-type dementia.

  • Anti-tau-positive glial fibrillary tangles in the brain of Postencephalitic Parkinsonism of Economo type
    Neuroscience Letters, 1993
    Co-Authors: Kenji Ikeda, Haruhiko Akiyama, Hiromi Kondo, Kazuhiko Ikeda
    Abstract:

    Tau-immunoreactive astrocytes have been reported in the brain of progressive supranuclear palsy (PSP) and are referred to as glial fibrillary tangles (GFTs). We found a number of GFTs in the heavily degenerated brain region in four cases of Postencephalitic Parkinsonism of Economo (PPE) with a clinical history of over a half-century. GFTs had the appearance of tufts of spider-like radiating fibers or small thorn-like feature by Gallyas-Braak method and also by anti-tau immunostaining.