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M. Hayashi - One of the best experts on this subject based on the ideXlab platform.

  • Neuropathological analysis in spinal muscular atrophy type II
    Acta Neuropathologica, 2003
    Co-Authors: S. Araki, M. Hayashi, K. Tamagawa, M. Saito, S. Kato, T. Komori, Y. Sakakihara, T. Mizutani
    Abstract:

    We performed a neuropathological analysis, including in situ nick end labeling (ISEL) and immunohistochemistry, of two cases of clinicogenetically confirmed infantile spinal muscular atrophy (SMA) type II. Both cases showed severe reduction of the motor neurons and gliosis in the spinal cord and brain stem, although the occurrences of Central Chromatolysis and ballooned neurons were not frequent. Clark's and lateral thalamic nuclei, which are usually altered in SMA type I, were spared, whereas Betz cells in the preCentral gyrus and large myelinated fibers in the lateral funiculus were reduced in number. Regarding apoptosis, only the younger case demonstrated a few ISEL-positive nuclei in the dorsal horn, with reduced Bcl-x expression level in the Purkinje cells. Unlike SMA type I, the expression of neurofilaments was not disturbed and the reduction in synaptophysin expression level in the anterior horn was mild. An oxidative stress-related product was deposited in atrophic motor neurons in the spinal cord, and neurons with nuclei immunoreactive for 8-hydroxy-2'-deoxyguanosine were found in the lateral thalamus. In contrast, the expression of glial glutamate transporters was not altered. These data suggest that oxidative stress and, to a lesser extent, apoptotic cell death, but not disturbed neurofilament metabolism or excitotoxicity, may be involved in neurodegeneration in SMA type II.

  • A study of cell death in Werdnig Hoffmann disease brain.
    Neuroscience Letters, 1998
    Co-Authors: M. Hayashi, N Arai, T Murakami, M Yoshio, M Oda, H Matsuyama
    Abstract:

    We examined the occurrence of apoptotic cell death in the autopsied brains of four patients with Werdnig Hoffmann disease (WH), using TdT-mediated DIG-dUTP nick end labeling (TUNEL) and immunohistochemistry for apoptosis-related proteins. Three of the four patients, aged over 6 months, exhibited TUNEL-positive cells in the lateral nuclei of the thalamus, and one of the three patients also had TUNEL-positive cells in the cerebral cortex. The labeled nuclei did not show characteristic features such as nuclear fragmentation or apoptotic bodies, and synaptophysin-positive granules were observed around some of the TUNEL-positive cells, although none of the antibodies against glial markers could visualize TUNEL-positive cells. TUNEL-positive cells were not observed in other regions examined, including the spinal cord, medulla and cerebellum or in the brains of three age-matched controls. There were neither immunopositive structures for bcl-2 or p53 nor alteration of in situ expression of bcl-xs/l or bax in any subject, and the TUNEL-positive cells lacked immunopositivity against apoptosis-related proteins. The presence of these TUNEL-positive cells might suggest latent neurodegeneration in the thalamus before Central Chromatolysis of neurons or neuronal loss appears, although it is not clear whether apoptotic cell death is involved in this degenerative process.

Tadashi Miyatake - One of the best experts on this subject based on the ideXlab platform.

  • Motor neuron disease with dementia and ophthalmoplegia
    Journal of Neurology, 1994
    Co-Authors: Hiroshi Komachi, Riki Okeda, Naoko Ishii, Katsuhiko Yanagisawa, Masahito Yamada, Tadashi Miyatake
    Abstract:

    Ophthalmoplegia and dementia are not usually observed in patients with amyotrophic lateral sclerosis. We report the case of a 60-year-old female with ophthalmoplegia and frontal-type dementia which appeared at an early stage of her illness that presented with dysarthria and weakness in the upper extremities. Notable autopsy findings in the Central nervous system were, in addition to the degeneration of upper and lower motor neurons, moderate neuronal loss and spongy degeneration in layer II of the frontal cortex with prominent astrocytosis, and moderate neuronal loss with astrocytosis in both the substantia nigra and the red nucleus. Central Chromatolysis of a few neurons in the oculomotor nucleus was seen. This case is considered to be a new subtype of motor neuron disease.

  • Motor neuron disease with dementia and ophthalmoplegia. A clinical and pathological study.
    Journal of Neurology, 1994
    Co-Authors: Hiroshi Komachi, Riki Okeda, Naoko Ishii, Katsuhiko Yanagisawa, Masahito Yamada, Tadashi Miyatake
    Abstract:

    Ophthalmoplegia and dementia are not usually observed in patients with amyotrophic lateral sclerosis. We report the case of a 60-year-old female with ophthalmoplegia and frontal-type dementia which appeared at an early stage of her illness that presented with dysarthria and weakness in the upper extremities. Notable autopsy findings in the Central nervous system were, in addition to the degeneration of upper and lower motor neurons, moderate neuronal loss and spongy degeneration in layer II of the frontal cortex with prominent astrocytosis, and moderate neuronal loss with astrocytosis in both the substantia nigra and the red nucleus. Central Chromatolysis of a few neurons in the oculomotor nucleus was seen. This case is considered to be a new subtype of motor neuron disease.

  • Association of IgG anti-GD1a antibody with severe Guillain-Barré syndrome.
    Muscle & Nerve, 1993
    Co-Authors: Nobuhiro Yuki, Mitsunori Yamada, Shuzo Sato, E. Ohama, Yasuhiro Kawase, Fusahiro Ikuta, Tadashi Miyatake
    Abstract:

    We earlier reported cases of 2 patients with severe acute Guillain–Barre syndrome (GBS) associated with high-IgG anti-GD1a antibody titer. We now have investigated the autoantibody against GD1a or GM1 in 37 GBS patients using the enzyme-linked immunosorbent assay and have found a statistically significant association between IgG anti-GD1a antibody and the severity of the disease (need of a respiratory for more than 1 month and a poor functional prognosis 3 months after neurologic onset). An autopsy which showed severe GBS associated with IgG anti-GD1a antibody produced the following findings: (1) severe axonal degeneration and segmental demyelination of peripheral nerves; (2) lymphocytic infiltration; and (3) marked Central Chromatolysis of the lower motoneurons. © 1993 John Wiley & Sons, Inc.

M. Osame - One of the best experts on this subject based on the ideXlab platform.

  • An autopsied case of the Crow-Fukase syndrome: a neuropathological study with emphasis on spinal roots
    Acta Neuropathologica, 1990
    Co-Authors: F. Umehara, S. Izumo, M. Zyounosono, M. Osame
    Abstract:

    An autopsied case of the Crow-Fukase syndrome is reported. Neuropathological findings were as follows: (1) in the sural nerve, there was marked decrease of large and small myelinated fibers. Myelinated fibers showing axonal degeneration and segmental demyelination and remyelination were moderately increased. (2) In the lumbar spinal roots, myelinated fibers showing segmental demyelination and remyelination were frequently observed. The density of myelinated fibers of the ventral root was less at the dural site than the spinal site, while that of the dorsal roots was less at the spinal site than the dural site. (3) In the dorsal root ganglion, there were Nageotte's residual nodules and satellitosis; (4) in the lumbar and thoracic spinal cord, there was pallor of the dorsal column; and (5) nerve cells showing Central Chromatolysis were frequently observed in the spinal anterior horn cells. Segmental demyelination and remyelination in the spinal roots and loss of myelinated fibers with axonal degeneration in the sural nerve are fibers with axonal degeneration in the sural nerve are main neuropathological features of this syndrome.

J. Q. Trojanowski - One of the best experts on this subject based on the ideXlab platform.

  • Phosphorylated high molecular weight neurofilament protein in lower motor neurons in amyotrophic lateral sclerosis and other neurodegenerative diseases involving ventral horn cells
    Acta Neuropathologica, 1990
    Co-Authors: G. Sobue, Y. Hashizume, T. Yasuda, E. Mukai, T. Kumagai, T. Mitsuma, J. Q. Trojanowski
    Abstract:

    Lower motor neurons of the spinal cord of patients with amyotrophic lateral sclerosis (ALS), Werdnig-Hoffmann's disease (WH), X-linked recessive bulbospinal neuronopathy (X-BSNP) and multiple system atrophy (MSA), all of which were known to involve the lower motor neurons, were immunohistochemically examined by using a monoclonal antibody (Ta-51) specific to phosphorylated epitopes of high molecular weight subunits of neurofilaments. The incidence of Ta-51-positive neurons was significantly increased in ALS, WH and MSA, but not in X-BSNP. Ta-51-positive neurons showed a wide variety of morphological appearances, including neurons with normal appearance, Central Chromatolysis, simple atrophy and neurons containing massive neurofilamentous accumulation. In aged-control cases, similar Ta-51-positive neurons were observed, although to a much lesser extent. In ALS, spheroids and globules, which were strongly positive for Ta-51, were also significantly increased. Ta-51-positive motor neurons, spheroids and globules appeared in proportional to the number of remaining large motor neurons in ALS.

Mohammad Hossein Nazemshirazi - One of the best experts on this subject based on the ideXlab platform.

  • RETRACTED ARTICLE: Neuropathological microscopic features of abortions induced by Bunyavirus / or Flavivirus infections
    Diagnostic Pathology, 2014
    Co-Authors: Javad Javanbakht, Seyed Hossein Mardjanmehr, Abbas Tavasoly, Mohammad Hossein Nazemshirazi
    Abstract:

    Background The present study describes the pathologic changes in the brain and the spinal cord of aborted, stillbirth and deformities of newborn lambs infected with viral agents. Methods From February 2012 to March 2013, a total of 650 aborted fetuses from 793 pregnant ewes were studied from 8 flocks at different areas in the Mazandaran province in the north of Iran. And randomly, systematic necropsy was performed to collect tissues, and all gross abnormalities were recorded at necropsy by the pathologist .Nevertheless, we conducted a limited number of necropsies for aborted fetuses. Results In the most cases, arthrogryposis was the most common musculoskeletal defects and at necropsy, malformations of the brain included hydranencephaly, porencephaly, hydrocephalus and cerebellar hypoplasia, mainly in the brain stem and gray and white matter of the brain and cerebellum were observed. Histopathologic lesions included chronic multifocal lymphoplasmacytic encephalitis(nonsuppurative) with extensive perivascular cuffing in some cases, formation of glial nodules mainly in the mesencephalon, thalamus, hippocampus, pons and medulla oblongata in the brain of aborted fetuses, and neuronal degeneration, necrosis and Central Chromatolysis mainly in the cortex and subcortical of the brain and brain stem regions of them. Furthermore, microscopic lesions are mostly linked to a neurodegenerative and necrotic cell death process in the gray matter of ventral horn of the spinal cord. Briefly, histopathologic findings in the brain and spinal cord included hyperemia, hemorrhage, non-suppurative encephalitis, mononuclear perivascular cuffing, multifocal gliosis, cavitation, Central Chromatolysis, neuronal degeneration and necrosis, perineuronal and perivascular edema in the all regions of the brain and acute neuronal necrosis in the gray matter of ventral horn of the spinal cord were also seen. Conclusion Our study suggested that the sheep fetuses are fully susceptible to viral infections and may even develop neurolopathological lesions upon natural infection with mentioned pathogens .Therefore ,according to,specific lesions caused by viral infections, we believe that the histopathological pattern were detected in this study could be associated with either viral infection and or mainly by a Bunyavirus / or Flavivirus strains that extensively shares common lesions with Rift Valley fever , Wesselsbron , Cache valley virus / or and Akabane viruses. Virtual Slides The virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/13000_2014_223

  • Retraction note: Neuropathological microscopic features of abortions induced by Bunyavirus/or Flavivirus infections.
    Diagnostic Pathology, 2014
    Co-Authors: Javad Javanbakht, Seyed Hossein Mardjanmehr, Abbas Tavasoly, Mohammad Hossein Nazemshirazi
    Abstract:

    The present study describes the pathologic changes in the brain and the spinal cord of aborted, stillbirth and deformities of newborn lambs infected with viral agents. From February 2012 to March 2013, a total of 650 aborted fetuses from 793 pregnant ewes were studied from 8 flocks at different areas in the Mazandaran province in the north of Iran. And randomly, systematic necropsy was performed to collect tissues, and all gross abnormalities were recorded at necropsy by the pathologist .Nevertheless, we conducted a limited number of necropsies for aborted fetuses. In the most cases, arthrogryposis was the most common musculoskeletal defects and at necropsy, malformations of the brain included hydranencephaly, porencephaly, hydrocephalus and cerebellar hypoplasia, mainly in the brain stem and gray and white matter of the brain and cerebellum were observed. Histopathologic lesions included chronic multifocal lymphoplasmacytic encephalitis(nonsuppurative) with extensive perivascular cuffing in some cases, formation of glial nodules mainly in the mesencephalon, thalamus, hippocampus, pons and medulla oblongata in the brain of aborted fetuses, and neuronal degeneration, necrosis and Central Chromatolysis mainly in the cortex and subcortical of the brain and brain stem regions of them. Furthermore, microscopic lesions are mostly linked to a neurodegenerative and necrotic cell death process in the gray matter of ventral horn of the spinal cord. Briefly, histopathologic findings in the brain and spinal cord included hyperemia, hemorrhage, non-suppurative encephalitis, mononuclear perivascular cuffing, multifocal gliosis, cavitation, Central Chromatolysis, neuronal degeneration and necrosis, perineuronal and perivascular edema in the all regions of the brain and acute neuronal necrosis in the gray matter of ventral horn of the spinal cord were also seen. Our study suggested that the sheep fetuses are fully susceptible to viral infections and may even develop neurolopathological lesions upon natural infection with mentioned pathogens .Therefore ,according to,specific lesions caused by viral infections, we believe that the histopathological pattern were detected in this study could be associated with either viral infection and or mainly by a Bunyavirus / or Flavivirus strains that extensively shares common lesions with Rift Valley fever ,Wesselsbron ,Cache valley virus / or and Akabaneviruses. The virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/13000_2014_223

  • Neuropathological microscopic features of abortions induced by Bunyavirus / or Flavivirus infections.
    Diagnostic Pathology, 2014
    Co-Authors: Javad Javanbakht, Seyed Hossein Mardjanmehr, Abbas Tavasoly, Mohammad Hossein Nazemshirazi
    Abstract:

    Background: The present study describes the pathologic changes in the brain and the spinal cord of aborted, stillbirth and deformities of newborn lambs infected with viral agents. Methods: From February 2012 to March 2013, a total of 650 aborted fetuses from 793 pregnant ewes were studied from 8 flocks at different areas in the Mazandaran province in the north of Iran. And randomly, systematic necropsy was performed to collect tissues, and all gross abnormalities were recorded at necropsy by the pathologist . Nevertheless, we conducted a limited number of necropsies for aborted fetuses. Results: In the most cases, arthrogryposis was the most common musculoskeletal defects and at necropsy, malformations of the brain included hydranencephaly, porencephaly, hydrocephalus and cerebellar hypoplasia, mainly in the brain stem and gray and white matter of the brain and cerebellum were observed. Histopathologic lesions included chronic multifocal lymphoplasmacytic encephalitis(nonsuppurative) with extensive perivascular cuffing in some cases, formation of glial nodules mainly in the mesencephalon, thalamus, hippocampus, pons and medulla oblongata in the brain of aborted fetuses, and neuronal degeneration, necrosis and Central Chromatolysis mainly in the cortex and subcortical of the brain and brain stem regions of them. Furthermore, microscopic lesions are mostly linked to a neurodegenerative and necrotic cell death process in the gray matter of ventral horn of the spinal cord. Briefly, histopathologic findings in the brain and spinal cord included hyperemia, hemorrhage, non-suppurative encephalitis, mononuclear perivascular cuffing, multifocal gliosis, cavitation, Central Chromatolysis, neuronal degeneration and necrosis, perineuronal and perivascular edema in the all regions of the brain and acute neuronal necrosis in the gray matter of ventral horn of the spinal cord were also seen. Conclusion: Our study suggested that the sheep fetuses are fully susceptible to viral infections and may even develop neurolopathological lesions upon natural infection with mentioned pathogens .Therefore ,according to, specific lesions caused by viral infections, we believe that the histopathological pattern were detected in this study could be associated with either viral infection and or mainly by a Bunyavirus /o rFlavivirus strains that extensively shares common lesions with Rift Valley fever ,Wesselsbron ,Cache valley virus /o r andAkabaneviruses. Virtual Slides: The virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx. eu/vs/13000_2014_223