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  • 1
    Electronic Resource
    Electronic Resource
    Ubiquitin-immunoreactive skein-like inclusions in the neostriatum are not restricted to amyotrophic lateral sclerosis, but are rather aging-related structures (2000)
    Springer
    Acta neuropathologica 100 (2000), S. 43-49 
    Details
    ISSN: 1432-0533
    Keywords: Key words Aging ; Immunohistochemistry ; Inclusion body ; Neostriatum ; Ubiquitin
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract We examined the presence of ubiquitin-immunoreactive skein-like inclusions (SLI) in the neostriatum and spinal cord in normal individuals and patients with different neurodegenerative diseases. Ubiquitin-immunoreactive SLI in the neostriatum were observed both in the normal individuals and in the patients with a variety of neurodegenerative diseases. In particular, SLI were frequently seen in normal aged subjects and certain neurodegenerative diseases, such as progressive supranuclear palsy and myotonic dystrophy. In contrast, the occurrence rate of SLI in cases with Pick’s disease and multiple system atrophy tended to decrease. On the other hand, SLI in the spinal anterior horn were detected in cases of amyotrophic lateral sclerosis, but not in any cases with other neurodegenerative diseases. SLI in the neostriatum were also identifiable using phosphotungstic acid-hematoxylin and Gomori trichrome staining. Ubiquitin immunoelectron microscopy demonstrated that the SLI in the neostriatum corresponded to bundles of filaments. These features of SLI in the neostriatum were quite similar to those of intracytoplasmic rod-like inclusions (RLI) in the large neurons of caudate nucleus, which were first described by Kojima and Ogawa in 1974. Our findings indicate that SLI in the neostriatum are ubiquitin-related structures whose occurrence increases by aging, and less frequently accompany several neurodegenerative diseases, and are identical to at least some RLI.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s004010051191
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  • 2
    Electronic Resource
    Electronic Resource
    Skein-like inclusions in the neostriatum from a case of amyotrophic lateral sclerosis with dementia (1998)
    Springer
    Acta neuropathologica 96 (1998), S. 541-545 
    Details
    ISSN: 1432-0533
    Keywords: Key words Amyotrophic lateral sclerosis ; Ubiquitin ; Inclusion body ; Neostriatum ; Immunohistochemistry
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Skeins or skein-like inclusions (SLIs) in motor neurons detected by ubiquitin immunohistochemistry are a characteristic finding of amyotrophic lateral sclerosis (ALS). Here we report ubiquitinated SLIs in the putamen and caudate nucleus from a case of ALS with dementia. A 48-year-old Japanese man developed apathy and amimia. Mental and neurological examinations revealed severe character change, muscle atrophy and fasciculation of the distal upper extremities and the tongue, and an exaggeration of the deep tendon reflex. He subsequently showed dysphagia and dysarthria. He died at the age of 51 years, after a total clinical course of about 2.5 years. By immunohistochemistry, ubiquitin-immunoreactive intraneuronal inclusions were observed in the spinal anterior horn cells, the frontal, temporal and entorhinal cortices, dentate fascia of the hippocampus and the amygdala. In addition, ubiquitinated inclusions were also seen in the putamen and caudate nucleus, which appeared as aggregates of thread-like structures similar to SLIs in the spinal anterior horn neurons. They were not seen on hematoxylin-eosin staining, and they also did not show any argentophilia nor did they react with other antibodies, including antibody against tau protein. To our knowledge, this is the first report of the presence of SLIs in non-motor neurons. Our results thus support the notion that ALS is a multisystem disease, and not simply a disease of the motor neurons.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s004010050932
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  • 3
    Electronic Resource
    Electronic Resource
    Preferential neurodegeneration in the cervical spinal cord of progressive supranuclear palsy (1999)
    Springer
    Acta neuropathologica 97 (1999), S. 577-584 
    Details
    ISSN: 1432-0533
    Keywords: Key words Progressive supranuclear palsy ; Microtubule-associated protein 2 ; Semiquantitative analysis ; Spinal cord ; Dystonia
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Spinal cord lesions have seldom been described in cases with progressive supranuclear palsy (PSP). We thus decided to analyze spinal cord lesions by microtubule-associated protein 2 (MAP2) immunohistochemistry in six cases of PSP, five cases of Parkinson’s disease (PD) and two cases of corticobasal degeneration (CBD), all of which cause parkinsonism, while six patients without any neurological disease served as controls. In the PSP cases, the MAP2 expression in the cervical spinal cords significantly decreased in the medial division of the anterior gray horn, intermediate gray and posterior gray horn, but showed no significant change in the substantia gelatinosa and lateral division of the anterior gray horn. The thoracic and lumbar spinal cords were well preserved for MAP2 immunoreactivity. In addition, the globose type neurofibrillary tangles and glial fibrillary tangles were more conspicuous in the cervical than in the thoracic and lumbar spinal cord in PSP cases. On the other hand, the PD and CBD cases showed no significant decrease of MAP2 immunoreactivity in the spinal cords. The small neurons, which are located rather selectively in the intermediate zone of the spinal cord, are considered to be mostly present in the interneurons, and are also thought to play a role in various types of focal dystonia, such as neck dystonia. We therefore consider the distinct decrease in the MAP2-positive neuronal processes in the cervical spinal cord may partly reflect the loss of interneurons and may, thereby, possibly cause nuchal dystonia.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s004010051033
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  • 4
    Electronic Resource
    Electronic Resource
    Immunohistochemical analysis of spinal cord lesions in amyotrophic lateral sclerosis using microtubule-associated protein 2 (MAP2) antibodies (1999)
    Springer
    Acta neuropathologica 97 (1999), S. 13-21 
    Details
    ISSN: 1432-0533
    Keywords: Key words Amyotrophic lateral sclerosis ; Microtubule-associated protein 2 ; Semiquantitative analysis ; Spheroid ; Inclusion body
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract We have studied microtubule-associated protein 2 (MAP2) expression in anterior horn neurons in the cervical and lumbar spinal cords of 19 cases of adult-onset sporadic amyotrophic lateral scerlosis (ALS) using immunohistochemistry. Specimens from 7 patients without neurological disease served as controls. MAP2 expression decreased in the anterior gray horn of all ALS cases and in the intermediate gray of several ALS cases. Such reduction correlated with the degree of degeneration or neuronal loss in anterior horn cells and with the clinical symptoms of limb weakness. Cytopathologically, the MAP2 immunoreactivity decreased corresponding to the occurrence of individual signs of neuronal degeneration, such as chromatolytic neurons, shrunken neurons and pigmented neurons. MAP2 expression was relatively well preserved in the specimens in which spheroids are conspicuous. The findings of this study demonstrate MAP2 to be an excellent marker for the detection and quantification of anterior horn degeneration in ALS.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s004010050950
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  • 5
    Electronic Resource
    Electronic Resource
    α-Synuclein is expressed in a variety of brain tumors showing neuronal differentiation (2000)
    Springer
    Acta neuropathologica 99 (2000), S. 154-160 
    Details
    ISSN: 1432-0533
    Keywords: Key wordsα-Synuclein ; Brain tumors ; Neuronal ¶differentiation ; Immunohistochemistry ; Neuronal marker
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract α-Synuclein is presynaptic nerve terminal protein and its immunoreactivity has been observed in such neurodegenerative structures as senile plaques of Alzheimer’s disease or Lewy bodies of Parkinson’s disease. The physiological role of α-synuclein is still unknown. It is speculated that α-synuclein may be expressed in brain tumors, especially in those showing neuronal differentiation. We examined the immunohistochemical localization of α-synuclein in 77 human brain tumors. α-Synuclein was widely distributed in the brain tumors showing neuronal differentiation. As a result, positive immunostaining for α-synuclein was observed in ganglioglioma, medulloblastoma, neuroblastoma, primitive neuroectodermal tumor, pineocytoma/pineoblastoma, and central neurocytoma. Compared with other neuronal markers, the positive ratio of α-synuclein was not as high as synaptophysin, microtubule-associacted protein 2, neuron-specific enolase and tau, but it was higher than neurofilament and chromogranin A. The expression of synaptophysin was diffusely observed in the cytoplasm, cellular processes and nucleus in tumors showing neuronal differentiation; however, the expression of α-synuclein was predominantly observed in the cytoplasm of the tumors as well as in the cellular processes. On the other hand, non-neuronal brain tumors such as astrocytic tumors or meningiomas were totally negative for α-synuclein. In conclusion, the appearance of an α-synuclein-positive structure was not limited to neurodegenerative diseases, but could also be detected in neoplastic cells showing neuronal differentiation.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/PL00007419
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