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  • 1
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 82 (1991), S. 295-301 
    ISSN: 1432-0533
    Keywords: Hyaline inclusions ; Motor neuron disease ; Amyotrophic lateral sclerosis ; Immunocytochemistry ; Ultrastructure
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary We investigated hyaline inclusion bodies (HI) immunocytochemically and ultrastructurally in six cases of sporadic motor neuron disease (MND). All HI contained large amounts of ubiquitin and some HI were stained at the core or the center with anti-neurofilament antibody, with the surrounding halo unstained. No HI were stained with antibodies raised against cytoskeletal proteins such as high-molecular weight microtubule-associated proteins and phosphorylated tau. Ultrastructurally, HI were chiefly composed of filaments measuring about 20 nm in diameter thicker than neurofilaments, and contained fine granules and frequently one or more of four characteristic profiles, i.e., small electron-dense materials resembling Bunina bodies, bundles of tubular filaments measuring approximately 20 nm in diameter, large electron-dense cores, and focal accumulations of randomly arranged neurofilaments. Hyaline inclusions can be regarded as one of the characteristic markers for sporadic MND as well as familial amyotrophic lateral sclerosis. Hyaline inclusions have a markedly heterogeneous ultrastructure and, therefore, differences in immunoreactivity with antineurofilament antibodies are not unexpected.
    Type of Medium: Electronic Resource
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  • 2
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 88 (1994), S. 222-227 
    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
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  • 3
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 91 (1996), S. 416-421 
    ISSN: 1432-0533
    Keywords: Key words Motor neuron disease ; Anterior horn ; neuron ; Synapse ; Active zone ; Ultrastructure
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract This report concerns an ultrastructural investigation of the synapses of anterior horn neurons in the lumbar spinal cord of four patients with lower motor neuron disease (LMND) who had no upper motor neuron and corticospinal tract involvement. Anterior horn neurons of five normal individuals served as controls. The cell body area and the number of synapses of the normal-appearing neurons of the LMND patients were significantly reduced (P 〈 0.0001). These findings suggest that synaptic changes of anterior horn neurons could be ascribed to the degeneration of lower motor neurons rather than to the influence of upper motor neuron system degeneration. On the other hand, the lengths of individual synapses (P 〈 0.0001) and of their active zones (P 〈 0.05) were significantly increased in the patients. These increases would indicate that synapses on anterior horn neurons of individuals with LMND appear to have the capacity to react to progressive degeneration and loss of other synapses by means of a compensatory response or plasticity that enhances their efficiency.
    Type of Medium: Electronic Resource
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  • 4
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 94 (1997), S. 338-344 
    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
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  • 5
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 95 (1998), S. 367-372 
    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
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  • 6
    Electronic Resource
    Electronic Resource
    Springer
    Experimental brain research 56 (1984), S. 308-322 
    ISSN: 1432-1106
    Keywords: Descending pathways ; Forelimb afferents ; C3-C4 inhibition ; C3-C4 propriospinal neurones
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Extra- and intracellular recording was made from cells in the C3-C4 segments with the aim of finding interneurones of previously described inhibitory pathways to the C3-C4 propriospinal neurones, which may mediate descending feed-forward inhibition and feed-back inhibition from the forelimb, respectively. The lateral interneurones were found in the lateral part of lamina VII interspersed among the C3-C4 PNs and like them they receive convergent monosynaptic EPSPs and disynaptic IPSPs from the cortico-, rubro-, tecto- and reticulospinal tracts. Disynaptic IPSPs, but only rarely monosynaptic EPSPs, are evoked in them from forelimb nerves. The lateral interneurones do not project to the lateral reticular nucleus (LRN). The medial interneurones were found medially in laminae V and VI in a region where volleys in forelimb nerves evoke extracellular monosynaptic focal potentials (Rosén 1969). There is somatotopic organization of the projection from the forelimb to this region. Many neurones are strongly monosynaptically excited from group I muscle or/and cutaneous forelimb afferents. In addition, late discharges are evoked in many cells from cutaneous afferents and high threshold muscle afferents. Corticospinal volleys evoked monosynapic excitation in the great majority of these cells and usually also late EPSPs or IPSPs. Typically, rubrospinal and tectospinal volleys evoked neither monosynaptic excitation nor late effects as those elicited from corticospinal fibres. In some of the interneurones, IPSPs were evoked from forelimb nerves. About 20% of the medial “interneurones” have an ascending projection to the caudal brain stem. Threshold mapping for antidromic stimulation revealed termination in the main cuneate nucleus, the external cuneate nucleus and/or the LRN and also a branch projecting to more rostral levels in the brain. A few of the neurones in the medial region are PNs projecting to the forelimb segments. It is postulated that interneurones both of the lateral and medial type are inhibitory and project to the C3-C4 PNs. It is further postulated that the former are intercalated in the descending feed-forward inhibitory pathway to the C3-C4 PNs and the latter in the feed-back inhibitory pathway from the forelimb to these PNs. The role of feed-forward and feed-back inhibition of transmission from the brain to forelimb motoneurones via the C3-C4 PNs is discussed.
    Type of Medium: Electronic Resource
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  • 7
    ISSN: 1432-1106
    Keywords: Horizontal semicircular canal ; Vestibularnuclei ; Vestibulo-collic ; Neck motoneuron ; HRP ; Cat
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary 1.The somatic location and axonal projections of inhibitory vestibular nucleus neurons activated by the horizontal semicircular canal nerve (HCN) were studied in anesthetized cats. Cats were anesthetized with ketamine hydrochloride and pentobarbital sodium. 2.Intracellular recordings were obtained from 11 neck extensor motoneurons which were identified by antidromic activation from the dosai rami (DR) in the C1 segment. Stimulation of the ipsilateral (i-) HCN and the ipsilateral abducens (AB) nucleus evoked IPSPs in the motoneurons. These IPSPs were fully or partially occluded when they were evoked simultaneously. 3. Intracellular recordings were obtained from 8 AB motoneurons. Stimulation of the i-HCN and the i-C1DR motoneuron pool evoked IPSPs in the AB motoneurons. These IPSPs were also partially occluded when they were evoked simultaneously, which implied that some HCN-activated neurons inhibit both i-AB motoneurons and ipsilateral neck motoneurons. 4. Unit activity was extracellularly recorded from 30 vestibular neurons that were activated monosynaptically by i-HCN stimulation. Their axonal projections were determined by stimulating the i-AB nucleus and the i-C1DR motoneuron pool. Eight neurons were activated by both stimuli, and were termed vestibulooculo-collic (VOC) neurons. Their axonal branching was examined by means of local stimulation in and around the i-AB nucleus and the i-C1DR motoneuron pool. Eighteen neurons were antidromically activated from the i-C1DR motoneuron pool but not from the i-AB nucleus. These were termed vestibulo-collic (VC) neurons. Four neurons were activated from the i-AB nucleus but not from the ventral funiculus in the C1 segment, and were termed vestibulo-ocular (VO) neurons. The HCN-activated inhibitory neurons were mostly localized in the rostroventral part of the medial vestibular nucleus. 5. Horseradish peroxidase (HRP) was injected iontophoretically into descending axons of 2 HCN-activated inhibitory VOC neurons which were identified by stimulation of the i-HCN and the i-AB nucleus. Axon collaterals were ramified from a stem axon in the ventral funiculus, and entered the gray matter and spread in the laminae VIII and IX. Terminal boutons were distributed over the medial and the ventromedial parts of the vental horn in the C1 segment.
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  • 8
    Electronic Resource
    Electronic Resource
    Springer
    Experimental brain research 92 (1992), S. 183-193 
    ISSN: 1432-1106
    Keywords: Trigeminal afferents ; Trigeminospinal neurones ; Excitation ; Neck motoneurones ; Cat
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Excitation of dorsal neck motoneurones evoked by electrical stimulation of primary trigeminal afferents in the Gasserian ganglion has been investigated with intracellular recording from α-motoneurones in the cat. Single stimulation in the Gasserian ganglion ipsi-and contralateral to the recording side evoked excitatory postsynaptic potentials (EPSPs) in motoneurones innervating the lateral head flexor muscle splenius (SPL) and the head elevator muscles biventer cervicis and complexus (BCC). The gasserian EPSPs were composed of early and late components which gave the EPSPs a hump-like shape. A short train of stimuli, consisting of two to three volleys, evoked temporal facilitation of both the early and late EPSP components. The latencies of the gasserian EPSPs ranged from 1.6 to 3.6 ms in SPL motoneurones and from 1.6 to 5.8 ms among BCC motoneurones. A rather similar latency distribution between 1.6 and 2.4 ms was found for ipsi- and contralateral EPSPs in SPL and BCC motoneurones, which is compatible with a minimal disynaptic linkage between primary trigeminal afferents and neck motoneurones. Systematic transections of the ipsi- and contralateral trigeminal tracts were performed in the brain stem between 3 and 12 mm rostral to the level of obex. The results demonstrate that both the ipsi- and contralateral disynaptic and late gasserian EPSPs can be mediated via trigeminospinal neurones which take their origin in the nucleus trigeminalis spinalis oralis. Transection of the midline showed that the contralateral trigeminospinal neurones cross in the brain stem. Systematic tracking in and around the ipsilateral trigeminal nuclei demonstrated that the axons of ipsilateral trigeminospinal neurones descend just medial to and/or in the medial part of the nucleus. Spinal cord lesions revealed a location of the axons of the ipsilateral trigeminospinal neurones in the lateral and ventral funiculi. Interaction between the ipsi- and contralateral gasserian EPSPs showed complete summation of the disynaptic EPSP component, while the late components were occluded by about 45%. These results show that the disynaptic EPSPs are mediated by separate trigeminospinal neurones from the ipsi- and contralateral side, while about half of the late EPSPs are mediated by common neurones which receive strong bilateral excitation from commissural neurones in the trigeminal nuclei. Spatial facilitation was found in the late gasserian EPSP but not in the disynaptic gasserian EPSP by conditioning stimulation of cortico- and tectofugal fibres. Disynaptic pyramidal and tectal EPSPs, which are mediated by reticulospinal neurones, were facilitated by a single stimulation in the gasserian ganglion at an optimal interval of 2 ms. It is suggested that primary trigeminal afferents can excite the reticulospinal neurones via a disynaptic trigeminoreticular pathway.
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  • 9
    ISSN: 1432-1106
    Keywords: C3-C4 propriospinal neurones ; Excitatory projection ; Forelimb motoneurones ; Lateral reticular nucleus
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary In the preceding report (Alstermark and Sasaki 1986) it was shown that a stimulus of 500 μA applied in the lateral reticular nucleus (LRN) evokes a maximal or near monosynaptic EPSP (LRN EPSP) in forelimb motoneurones. This EPSP which is assumed to be selectively mediated by C3-C4 propriospinal neurones (PNs), was used to estimate the strength of the excitatory projection from C3-C4 PNs. A systematic comparison was made of the size and time course of the maximal LRN EPSP in various species of forelimb α-motoneurones innervating shoulder, elbow, wrist and digit muscles. The LRN EPSP was evoked in all investigated species of forelimb motoneurones. When either the peak amplitude or the underlying area of the LRN EPSP was compared, a three-fold range was found with some tendency for the size to vary in the order of wrist 〉 shoulder ≈ elbow 〉 digit 〉 intrinsic paw motor nuclei. Generally, a positive correlation was found in each motor nucleus between the peak amplitude of the LRN EPSP versus the monosynaptic homonymous group Ia EPSP, input resistance and afterhyperpolarization duration respectively (cf. Alstermark and Sasaki 1986). It is therefore postulated, that the LRN EPSP peak amplitude is correlated with motor unit type. Comparison of the time course of the LRN EPSPs was made by measuring the time-to-peak (T-t-p) and half-width (H-w). The finding in the preceding report that the T-t-p and H-w is longer in slow than in fast motoneurones was confirmed and extended to all the investigated motor nuclei. The hypothesis that both fast slow motoneurones receive projection from a group of fast C3-C4 PNs, while slow motoneurones receive an additional projection from a group with lower conduction velocity, can therefore be applied to all forelimb motor nuclei. In addition, it is proposed that some slow shoulder, wrist and digit motoneurones receive projection from a special subpopulation of C3-C4 PNs with very slow conduction velocity.
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  • 10
    ISSN: 1432-1106
    Keywords: C3-C4 propriospinal neurones ; Differential projection ; Fast and slow forelimb motoneurones ; Lateral reticular nucleusn
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary The projection of C3-C4 propriospinal neurones (PNs) to α-motoneurones of forelimb muscles has been analysed with the aid of antidromic stimulation of the ascending branch of the PNs to the lateral reticular nucleus (LRN). A single stimulus of 500 μA applied in the caudo-dorsal part of the LRN evoked a maximal or 〉 90% maximal monosynaptic EPSP in the motoneurones. Systematic mapping of EPSPs evoked by stimulation of 500 μA in and around the LRN revealed that at this strength there was hardly any co-activation of a medial system (Peterson et al. 1979) which evoked small monosynaptic EPSPs with shorter latency and faster time course. The LRN EPSP amplitude was positively correlated with the homonymous group Ia EPSP amplitude, the input resistance and the afterhyperpolarization (AHP) duration. It is therefore postulated that the LRN EPSP amplitude is correlated with motor unit type (Burke 1967, 1968; Burke et al. 1973) with the largest EPSPs in slow (S), the smallest in fast, fatiguable (FF) and possibly intermediate sized in fast, fatigue resistant (FR) units. There was only a small difference in latency of the LRN EPSP in fast and slow motoneurones, while the time course was considerably slower in the latter. It is suggested that slow motoneurones receive projection both from fast and slowly conducting PNs but fast motoneurones mainly from fast PNs. Comparison of the disynaptic pyramidal EPSPs and the LRN EPSPs revealed a positive correlation, but the amplitude ratio pyramidal EPSP: LRN EPSP was smaller in slow than in fast motoneurones. A negative correlation was found between this amplitude ratio and the latency of the disynaptic pyramidal EPSP. It is suggested that this correlation reflects the excitability level in the PNs and that low excitability is due to inhibition of the PNs.
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