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  • 1
    Electronic Resource
    Electronic Resource
    Excitatory amino acid transporter 1 and 2 immunoreactivity in the spinal cord in amyotrophic lateral sclerosis (2000)
    Springer
    Acta neuropathologica 100 (2000), S. 138-144 
    Details
    ISSN: 1432-0533
    Keywords: Key words Amyotrophic lateral sclerosis ; Motor neuron disease ; Excitatory amino acid ; Astroglial glutamate transporter ; Spinal cord
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract The spinal cord of 20 patients with amyotrophic lateral sclerosis (ALS) and 5 patients with lower motor neuron disease (LMND) were investigated immunohistochemically using anti-human excitatory amino acid transporter 1 (EAAT1) and EAAT2 antibodies which are the astrocytic transporters. The purpose of the study was to examine relationships between EAAT1 and EAAT2 immunoreactivity and degeneration of anterior horn neurons. Specimens from 20 patients without any neurological disease served as controls. In controls, spinal cord gray matter was densely immunostained by antibodies, whereas the white matter was generally not immunostained. In motor neuron disease (MND) patients, EAAT1 immunoreactivity was relatively well preserved in the gray matter despite neuronal loss of anterior horn cells. On the other hand, EAAT2 immunoreactivity in anterior horns correlated with the degree of neuronal loss of anterior horn cells: in the patients with mild neuronal depletion, anterior horns were densely immunostained by the antibody, whereas in the patients with severe neuronal loss, EAAT2 expression was markedly reduced. Degenerated anterior horn cells frequently showed a much denser EAAT1 and EAAT2 immunoreactivity around the surface of the neurons and their neuronal processes than that observed in normal-appearing neurons. There was no difference in the expression of EAAT1 and EAAT2 immunoreactivity between LMND and ALS patients. These findings suggest that in the early stage of degeneration of anterior horn cells, EAAT1 and EAAT2 immunoreactivity is preserved in the astrocytic foot directly attached to normal-appearing neurons, whereas levels of EAAT1 and EAAT2 protein rather increase in the astrocytic foot directly attached to degenerated anterior horn neurons; the latter effect most probably reduces the elevated glutamate level, compensates for the reduced function of astroglial glutamate transporters, or represents a condensation of EAAT1 and EAAT2 immunoreactivity secondary to loss of neurites and greater condensation of astrocytic processes. Thus, we demonstrate a difference in EAAT1 and EAAT2 immunoreactivity in different stages of progression in ALS, as a feature of the pathomechanism of this disease.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s004019900159
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  • 2
    Electronic Resource
    Electronic Resource
    Nonoxidative protein glycation is implicated in familial amyotrophic lateral sclerosis with superoxide dismutase-1 mutation (2000)
    Springer
    Acta neuropathologica 100 (2000), S. 275-284 
    Details
    ISSN: 1432-0533
    Keywords: Key words Amyotrophic lateral sclerosis ; Imidazolone ; Nɛ-carboxymethyl-lysine ; Pyrraline ; Superoxide dismutase
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract To assess a role for oxidative stress in the pathogenesis of amyotrophic lateral sclerosis (ALS), we analyzed the immunohistochemical localization of 8-hydroxy-2′-deoxyguanosine (OHdG) as a nucleic acid oxidation product, acrolein-protein adduct and 4-hydroxy-2-nonenal (HNE)-protein adduct as lipid peroxidation products, N ɛ-carboxymethyl-lysine (CML) as a lipid peroxidation or protein glycoxidation product, pentosidine as a protein glycoxidation product, and imidazolone and pyrraline as nonoxidative protein glycation products in the spinal cord of three familial ALS patients with superoxide dismutase-1 (SOD1) A4V mutation, six sporadic ALS patients, and six age-matched control individuals. The spinal cord sections of the control cases did not show any distinct immunoreactivities for these examined products. In the familial ALS cases, intense immunoreactivities for pyrraline and CML were confined to the characteristic Lewy body-like hyaline inclusions, and imidazolone immunoreactivity was located in the cytoplasm of the residual motor neurons. No significant immunoreactivities for other examined products were detected in the familial ALS spinal cords. In the sporadic ALS cases, intense immunoreactivities for pentosidine, CML and HNE-protein adduct were seen in the cytoplasm of the degenerated motor neurons, and OHdG immunoreactivity was located in the cell nuclei of the residual neurons and glial cells. The present results indicate that oxidative reactions are involved in the disease processes of sporadic ALS, while there is no evidence for increased oxidative damage except for CML deposition in the familial ALS spinal cords. Furthermore, it is likely that the accumulation of pyrraline and imidazolone supports a nonoxidative mechanism in SOD1-related motor neuron degeneration.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s004019900173
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  • 3
    Electronic Resource
    Electronic Resource
    Dendritic synapses of anterior horn neurons in amyotrophic lateral sclerosis: an ultrastructural study (1996)
    Springer
    Acta neuropathologica 91 (1996), S. 278-283 
    Details
    ISSN: 1432-0533
    Keywords: Key words Amyotrophic lateral sclerosis ; Primary dendrite ; Synapse ; Synaptic length ; Active synaptic zone
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract This ultrastructural study deals with the synapses of primary dendrites of the anterior horn neurons in the lower lumbar spinal cords of seven patients with amyotrophic lateral sclerosis (ALS) who had mild neuronal depletion. Specimens from seven age-matched, neurologically normal individuals served as controls. In each instance, the autopsy was performed within 6 h after death. Our results indicate a statistically significant increase in degenerative changes in the dendrite presynapses of normal-appearing neurons of the ALS patients. The alterations included aggregation of electron-dense synaptic vesicles and dark mitochondria with dense cristae, and bundles of neurofilaments. However, we found no significant difference between controls and patients with respect to cross-sectional area and length of the dendrites, number of synapses per dendrite, and lengths of individual synapses and their active zones in the normal-appearing neurons, even though the patients' neurons had a smaller cross-sectional area. In chromatolytic neurons, the number of synapses and the length of the active zone of the primary dendrites were significantly diminished. These findings suggest that despite degenerative changes of the presynapses, the synapses in the primary dendrites of the anterior horn neurons are preserved at the early stage of ALS. The preservation of these synapses may be due to their relative resistance to degenerative processes, or may represent a compensatory mechanism of the synapses for diminished synaptic function in distal dendrites.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s004010050426
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  • 4
    Electronic Resource
    Electronic Resource
    Immunocytochemical and ultrastructural study of pericapillary rosettes in amyotrophic lateral sclerosis (1997)
    Springer
    Acta neuropathologica 94 (1997), S. 338-344 
    Details
    ISSN: 1432-0533
    Keywords: Key words Amyotrophic lateral sclerosis ; Pericapillary rosette ; Immunocytochemistry ; Ultrastructure
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract This report concerns a comparative immunocytochemical and ultrastructural investigation on pericapillary rosettes (PR) in the lumbar spinal cords of 21 patients with amyotrophic lateral sclerosis (ALS) and 18 age-matched neurologically normal individuals. The purpose of the study was to determine the alteration of PR in relation to the neuronal loss in ALS. The PR were almost always positively immunostained for phosphorylated neurofilament, and some PR immunoreacted with antibodies to synaptophysin and β-amyloid precursor protein. This finding suggests that axonal transport, whether fast or slow, is impaired in the terminal portion of the axon that reaches the capillaries. Some PR were also positively immunostained by the antibody against ubiquitin, anti-calbindin-D 28 K antibody, anti-parvalbumin antibody and the antibody to superoxide dismutase 1. Morphometrically, the number of PR in the anterior horns and lateral column was markedly diminished in ALS compared with controls. At the ultrastructural level, the PR consisted mostly of unmyelinated degenerated axons, and were frequently found outside the basal laminae of the endothelial cell and of the astrocytic foot processes on the opposite side of the capillary, and less often in the space between the two basal laminae. The data indicate that the fate of PR is intimately associated with the neuronal loss of the anterior horn cells and with degenerative change of nerve fibers extending from their mother neurons to the capillaries.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s004010050716
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  • 5
    Electronic Resource
    Electronic Resource
    Synaptic loss in the proximal axon of anterior horn neurons in motor neuron disease (1995)
    Springer
    Acta neuropathologica 90 (1995), S. 170-175 
    Details
    ISSN: 1432-0533
    Keywords: Key words Amyotrophic lateral sclerosis ; Motor ; neuron disease ; Axon hillock ; Initial segment ; Synapse
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract This report deals with an ultrastructural investigation of the synapses of the proximal axons of normal-appearing anterior horn neurons of 7 patients with amyotrophic lateral sclerosis (ALS) and 4 patients with motor neuron disease who had no upper motor neuron and corticospinal tract involvement (lower motor neuron disease, LMND). Specimens from 12 age-matched individuals who died of non-neurological diseases served as controls. Proximal axons directly emanating from the normal-appearing neurons were examined: 42 axons were from ALS patients, 43 from LMND patients and 87 from controls. Our results show that the number of synapses on axon hillocks, as well as the lengths of the synaptic contact and of the active zone were reduced in both groups of patients (P 〈 0.0001), but no significant differences were seen between patients and controls with respect to the synaptic parameters of initial axon segments. There was no overall difference between ALS and LMND patients. These findings suggest that the electrophysiological functions pertaining to integration of electrical inputs into the axon and information transduction on the axon may be greatly impaired in the early stages of motor neuron diseases, and that the observed synaptic alterations may be pathological events, likely to be due to anterior horn neuron degeneration.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00294317
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  • 6
    Electronic Resource
    Electronic Resource
    Synapse loss in anterior horn neurons in amyotrophic lateral sclerosis (1994)
    Springer
    Acta neuropathologica 88 (1994), S. 222-227 
    Details
    ISSN: 1432-0533
    Keywords: Key words Amyotrophic lateral sclerosis ; Anterior horn neuron ; Synapse ; Active zone ; Ultrastructure
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract This report deals with an ultrastructural investigation of the synapses of anterior horn neurons in the lumbar spinal cords of five patients with amyotrophic lateral sclerosis (ALS) who had mild neuronal depletion. Specimens from five age-matched, neurologically normal individuals served as controls. In each instance, the autopsy was performed within 3 h after death. A statistically significant decrease in cell body area, number of synapses and total synaptic length was found in the normal-appearing neurons of the ALS patients. The alterations were more pronounced in neurons with central chromatolysis. However, despite an approximately 20  % reduction in the number of synapses, the length of the active synaptic zone of the normal-appearing neurons in the ALS patients was not diminished. This observation may be accounted for by a plasticity to the loss of synapses which maintained the active zone of the remaining synapses to increase synaptic efficiency. It is suggested that when the plasticity of the active zone reaches its limit, the continuing loss of synapses may lead to functional impairment. The capacity of the active synaptic zone to respond to progressive denervation of the anterior horn neurons may preserve motor function or slow the development of motor deficits in the early stage of degeneration of the lower motor neurons.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00293397
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  • 7
    Electronic Resource
    Electronic Resource
    Immunoreactivity of β-amyloid precursor protein in amyotrophic lateral sclerosis (1999)
    Springer
    Acta neuropathologica 97 (1999), S. 463-468 
    Details
    ISSN: 1432-0533
    Keywords: Key words Amyotrophic lateral sclerosis ; β-Amyloid precursor protein ; Immunohistochemistry ; Fast axonal transport ; Anterior horn neuron
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract We investigated the localization and extent of β-amyloid precursor protein (β-APP695) immunoreactivity as a sensitive marker for impairment of fast axonal transport in the spinal cords of 21 patients with amyotrophic lateral sclerosis (ALS), paying special attention to anterior horn neurons. Specimens from 18 patients without neurological disease served as controls. Increased β-APP immunoreactivity was frequently recognized in the anterior horns of the ALS patients with short clinical courses or with mild depletion of anterior horn cells, while no β-APP immunoreactivity was demonstrated in those with severe depletion of anterior horn neurons or with long-standing clinical courses. Increased β-APP immunoreactivity in the anterior horn neurons was mainly confined to the perikarya and no immunoreactivity was recognized in the dendrites or proximal axons directly emanating from the somata, except some spheroids (proximal axonal swellings) which showed increased immunoreactivity of β-APP. Increased β-APP immunoreactivity was spotted or focally aggregated in the perikarya of normal-looking large anterior horn neurons, while it was frequently diffuse in that of degenerative neurons such as central chromatolytic cells and or those with simple atrophy. On the other hand, the controls showed no immunostaining with β-APP in the spinal cord. These findings suggest that increased immunoreactivity of β-APP in neuronal perikarya of the anterior horn cells and in some proximal axonal swellings is an early change of ALS, and may be a response of the increased synthesis of β-APP resulting from neuronal damage, or the impairment of fast axonal transport.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s004010051015
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  • 8
    Electronic Resource
    Electronic Resource
    Motor neuron disease with predominantly upper extremity involvement: a clinicopathological study (1999)
    Springer
    Acta neuropathologica 98 (1999), S. 645-650 
    Details
    ISSN: 1432-0533
    Keywords: Key words Amyotrophic lateral sclerosis ; Autopsy ; Electron microscopy ; Immunocytochemistry ; Motor ; neuron disease
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract We report two autopsy cases of motor neuron disease (MND) patients with an unusual type of muscular atrophy predominantly affecting the shoulder girdle and the upper extremities with proximal dominance. Both patients are considered to be clinically categorized into the El Escorial suspected form of amyotrophic lateral sclerosis (ALS). At autopsy, they showed marked loss of spinal anterior horn cells accompanied by astrogliosis positively immunostained with anti-glial fibrillary acidic protein antibody at the cervical level. At the lumbosacral level, anterior horn neurons were relatively well preserved and Bunina bodies, ubiquitin-positive skein-like inclusions and Lewy body-like inclusions were observed in the remaining neurons. In one patient, brain stem motor neurons (nerves V, VII, XII) and motor cortex, including Betz cells, were also affected and the corticospinal tracts were degenerated at the level of the thoracic and lumbar spinal cord. Pathological findings of this patient are consistent with those of ALS. In the other patient, the motor cortex, brain stem motor nuclei and the corticospinal tracts were well preserved, which is pathologically compatible with progressive spinal muscular atrophy. These patients with such a peculiar pattern of progressive muscular atrophy should be placed in a subgroup of ALS.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s004010051131
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  • 9
    Electronic Resource
    Electronic Resource
    Characterizations of heterotopic neurons in the spinal cord of amyotrophic lateral sclerosis patients (1998)
    Springer
    Acta neuropathologica 95 (1998), S. 367-372 
    Details
    ISSN: 1432-0533
    Keywords: Key words Amyotrophic lateral sclerosis ; Heterotopic neuron ; Alpha motor neuron ; Immunocytochemistry ; Ultrastructure
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract This report concerns a comparative immunocytochemical, ultrastructural and morphometric investigation on heterotopic neurons in the white matter of the spinal cords of 19 patients with amyotrophic lateral sclerosis (ALS) and 18 age-matched neurologically normal individuals. The study revealed that the heterotopic neurons were scattered in the white matter, often adjacent to gray matter, that they immunoreacted with the antibody to synaptophysin, and that there were synaptic apparatuses on the surface of their somata and their neuronal processes. Bunina bodies and ubiquitin-positive inclusions such as Lewy body-like inclusions and skein-like inclusions, characteristic of anterior horn neurons of ALS, were present in the cytoplasm of the patients’ heterotopic neurons in the anterior or lateral column of the white matter. These findings suggest that heterotopic neurons in the anterior or lateral column have the characteristics of alpha motor neurons. The average number of heterotopic neurons observed in ALS patients was generally less than in normal subjects. This reduction was correlated with the severity of neuronal loss. The heterotopic neurons in ALS were less susceptible to the degenerative process as compared with spinal cord anterior horn cells. We assume that in this disease the heterotopic neurons may be degenerated and their number diminished after or concomitantly with the depletion of anterior horn neurons.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s004010050812
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