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  • 1
    Electronic Resource
    Electronic Resource
    Golgi apparatus and intraneuronal inclusions of anterior horn cells in amyotrophic lateral sclerosis: an immunohistochemical study (1996)
    Springer
    Acta neuropathologica 91 (1996), S. 603-607 
    Details
    ISSN: 1432-0533
    Keywords: Key words Amyotrophic lateral sclerosis ; Golgi ; apparatus ; Intraneuronal inclusions ; Bunina body ; Immunohistochemistry
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract We applied immunohistochemical techniques to study alterations of the Golgi apparatus, and to determine possible relationships between this complex and the intraneuronal inclusions of lower motor neurons of patients with the sporadic form of amyotrophic lateral sclerosis (ALS). Spinal cords from normal individuals served as controls. Monoclonal antibodies to the Golgi zone and to Golgi β-COP protein were used. Immunoreactivity with these antibodies was seen in frozen sections of paraformaldehyde-fixed spinal cord tissue, but not in formalin-fixed, paraffin-embedded specimens. The immunoreaction products were seen as granular structures, diffusely distributed in neurons and glial cells. Although immunostaining of some ALS neurons was reduced, fragmentation of the Golgi apparatus was not evident with either antibody. Bunina bodies and skein-like inclusions, characteristically found in spinal anterior horn cells of ALS patients, were not stained by these antibodies to epitopes of the Golgi apparatus, nor were Lewy body-like hyaline inclusions, present in some cases of sporadic ALS and reported to be characteristic of familial ALS with posterior column degeneration. These results suggest that components of the Golgi apparatus are not directly incorporated into intraneuronal inclusions. However, the possibility that abnormal proteinaceous material of the Golgi apparatus may be involved in their genesis cannot be ruled out.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s004010050473
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    Paper (German National Licenses)
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  • 2
    Electronic Resource
    Electronic Resource
    Topographic involvement of the striatal efferents in basal ganglia of patients with adult-onset motor neuron disease with basophilic inclusions (1995)
    Springer
    Acta neuropathologica 89 (1995), S. 513-518 
    Details
    ISSN: 1432-0533
    Keywords: Key words Amyotrophic lateral sclerosis ; Basophilic ; inclusion ; Basal ganglia ; Met-enkephalin ; Substance P
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract This report concerns the topographic immunohistochemical analysis of the putamen, globus pallidus (GP) and substantia nigra (SN) of two patients with adult-onset motor neuron disease with basophilic inclusions (MND/BIs), seven patients with sporadic classic amyotrophic lateral sclerosis (sporadic ALS) and five neurologically normal individuals. The striatal efferent terminals of the GP and SN were visualized immunohistochemically using antibodies to met-enkephalin (MEnk) and substance P (SP). In specimens from patients with sporadic ALS and normal subjects there was intense immunostaining for MEnk and SP throughout the external and internal segments of the GP, respectively. By contrast, a marked reduction of MEnk- and SP-positive striatal efferents was seen in the ventrocaudal portions of both GP segments from the MND/BIs patients. Moreover, while MEnk-positive striosomes were readily detected in the putamen of normals and sporadic ALS patients, there was significant reduction in MEnk immunoreactivity, and no evidence of striosomal organization in the putamen of MND/BIs patients. In addition, whereas the SN of patients with sporadic ALS expressed SP, the ventrolateral SN portion of the MND/BIs patient tested had reduced immunoreactivity. The present findings on patients with MND/BIs may represent a reflection of the topographic striatum degeneration in this disease and appear to provide additional evidence for the heterogeneity of MND.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00571505
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    Paper (German National Licenses)
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  • 3
    Electronic Resource
    Electronic Resource
    Topographic involvement of the striatal efferents in basal ganglia of patients with adult-onset motor neuron disease with basophilic inclusions (1995)
    Springer
    Acta neuropathologica 89 (1995), S. 513-518 
    Details
    ISSN: 1432-0533
    Keywords: Amyotrophic lateral sclerosis ; Basophilic inclusion ; Basal ganglia ; Met-enkephalin ; Substance P
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract This report concerns the topographic immunohistochemical analysis of the putamen globus pallidus (GP) and substantia nigra (SN) of two patients with adultonset motor neuron disease with basophilic inclusions (MND/BIs), seven patients with sporadic classic amyotrophic lateral sclerosis (sporadic ALS) and five neurologically normal individuals. The striatal efferent terminals of the GP and SN were visualized immunohisto-chemically using antibodies to met-enkephalin (MEnk) and substance P (SP). In specimens from patients with sporadic ALS and normal subjects there was intense immunostaining for MEnk and SP throughout the external and internal segments of the GP, respectively. By contrast, a marked reduction of MEnk- and SP-positive striatal efferents was seen in the ventrocaudal portions of both GP segments from the MND/BIs patients. Moreover, while MEnk-positive striosomes were readily detected in the putamen of normals and sporadic ALS patients, there was significant reduction in MEnk immunoreactivity, and no evidence of striosomal organization in the putamen of MND/BIs patients. In addition, whereas the SN of patients with sporadic ALS expressed SP, the ventrolateral SN portion of the MND/BIs patient tested had reduced immunoreactivity. The present findings on patients with MND/BIs may represent a reflection of the topographic striatum degeneration in this disease and appear to provide additional evidence for the heterogeneity of MND.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00571505
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    Paper (German National Licenses)
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  • 4
    Electronic Resource
    Electronic Resource
    Intracisternal inclusions in Schwann cells of the sural nerve (1992)
    Springer
    Acta neuropathologica 83 (1992), S. 664-666 
    Details
    ISSN: 1432-0533
    Keywords: Dutcher's bodies ; Endoplasmic reticulum ; Microfilaments ; Perinuclear cistern ; Schwann cell
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Biopsy of the sural nerve in a 74-year-old man with chronic neuropathy demonstrated Schwann cells that possessed multiple cisterns of rough endoplasmic reticulum filled with 7- to 8-nm filaments and perinuclear cisterns that were markedly distended with fine granular substances and electron-dense globules. To our knowledge, this is the third case exhibiting filamentous inclusions in Schwann cells. Unlike the previous cases, however, this case showed inclusions in the distended perinuclear cisterns and axonal degeneration. The significance of these alterations remains to be elucidated.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00299418
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  • 5
    Electronic Resource
    Electronic Resource
    Ataxic variant of adrenoleukodystrophy: MRI and CT findings (1992)
    Springer
    Journal of neurology 239 (1992), S. 307-310 
    Details
    ISSN: 1432-1459
    Keywords: Adrenoleukodystrophy ; Cerebellar ataxia ; Spinocerebellar degeneration ; Computed tomography ; Magnetic resonance imaging
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary A 28-year-old man developed slowly progressive dysarthria and gait disturbance over 7 years. Neurological examination revealed marked ataxia of articulation and gait, mild ataxia and spasticity of all four limbs without intellectual, visual, auditory, sensory or sphincter dysfunction. No physical signs of adrenal hypofunction were found. However, analysis of fatty acid of plasma sphingomyelin showed an increase in very long chain fatty acids, compatible with the diagnosis of adrenoleukodystrophy. Computed tomography disclosed marked atrophy of the cerebellum and pons, and bilateral low-density lesions in the deep while matter of the cerebellum. Magnetic resonance imaging showed these lesions more clearly, as well as other lesions in the middle and superior cerebellar peduncles, despite the absence of cerebral white matter involvement at the time of presentation. This is a rare case of adrenoleukodystrophy presenting as spinocerebellar degeneration and showing marked atrophy and several parenchymatous lesions of the cerebellum and brain stem.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00867585
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    Paper (German National Licenses)
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