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  • 11
    Electronic Resource
    Electronic Resource
    Age-related morphologic changes of the central canal of the human spinal cord (1999)
    Springer
    Acta neuropathologica 97 (1999), S. 253-259 
    Details
    ISSN: 1432-0533
    Keywords: Key words Aging ; Spinal canal ; Histology ; Spinal cord ; Syringomyelia
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract To elucidate the role of the human central canal on the physiology and pathogenesis of acquired syringomyelia, we analyzed the age-related morphologic changes in the normal human central canal of the spinal cord. The subjects included 158 autopsy cases ranging in age from 1 week postnatally to 116 years of age. Each segment of the whole spinal cords was investigated from the C3 to S3 levels. The microscopic pictures of the central canal were classified as patent or occluded at each level for each age decade. The patency rate under 1 year of age was 100% in almost all the segments, which markedly decreased in the second decade, and the canals were occluded in all the segments with advancing age. According to the longitudinal pattern of the central canal occlusion, 19 of 20 cases where the canals were patent in all segment levels were less than 10 years of age. Cases in which the canals were occluded in all segment levels appeared in the second decade, and their number increased gradually with advancing age. The occlusion of the central canal started at the T6 and L5 to S2 levels. We suggest that the central canal does not function after infancy because of its occlusion, and that it is not involved in the development of syringomyelia in adult patients.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s004010050982
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  • 12
    Electronic Resource
    Electronic Resource
    The lateral corticospinal tract and spinal ventral horn in X-linked recessive spinal and bulbar muscular atrophy: a quantitative study (1996)
    Springer
    Acta neuropathologica 93 (1996), S. 1-6 
    Details
    ISSN: 1432-0533
    Keywords: Key words X-linked recessive spinal and bulbar ; muscular atrophy ; Corticospinal tract ; Spinal ventral horn cells ; Alpha motor neuron ; Interneuron
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract A quantitative study was performed on spinal cord lesions in seven patients with X-linked recessive spinal and bulbar muscular atrophy. The myelinated fiber density of the lateral corticospinal tracts at the T7 cord level was well preserved for both large and small myelinated fibers. On the other hand, neurons in the L4 ventral horn were markedly depleted; marked loss was noted of the large alpha and medium-sized gamma motor neurons located in the lateral and medial nuclei as well as the small neurons in the intermediate zones of the ventral horn. These results suggest that myelinated fiber density and fiber-size distribution in the corticospinal tract are well preserved and that neuronal loss in the ventral horns is not restricted to alpha and gamma motoneurons but also involves small interneurons.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s004010050575
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  • 13
    Electronic Resource
    Electronic Resource
    Age-related changes in human spinal ventral horn cells with special reference to the loss of small neurons in the intermediate zone: a quantitative analysis (1996)
    Springer
    Acta neuropathologica 92 (1996), S. 109-114 
    Details
    ISSN: 1432-0533
    Keywords: Key words Spinal ventral horn ; Aging ; Interneuron ; Alpha motor neuron ; Morphometry
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract A cytoarchitectonic study of spinal ventral horn cells was performed to identify age-related changes. The diameter distribution of ventral horn neurons of the fourth lumbar segment of the spinal cord and their size and topographical distributions were investigated in 14 autopsy cases. These cases represented patients of 18–100 years of age who had died of non-neurological diseases. The results indicate that small neurons widely distributed in the intermediate zone of the ventral horn significantly diminished with aging (P 〈 0.0005, r = –0.898), whereas medium-sized and large neurons located in the medial and lateral nuclei showed only a slight decrease with advancing age. The total number of neurons in the whole ventral horn was also noted to decrease significantly with aging (P 〈 0.0005, r = –0.899). While small neurons in the intermediate zone of the ventral horn are thought to be mostly interneurons, their physiological function still remains obscure in many respects. The findings of this study provide insight into age-related cell loss in terms of size and location.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s004010050497
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  • 14
    Electronic Resource
    Electronic Resource
    Tissue distribution of pathological lesions and Hu antigen expression in paraneoplastic sensory neuronopathy (1998)
    Springer
    Acta neuropathologica 95 (1998), S. 641-648 
    Details
    ISSN: 1432-0533
    Keywords: Key words Paraneoplastic sensory neuronopathy ; Hu antigen ; Multifocal lesion ; Tissue distribution
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract We investigated the distribution of lesions and Hu antigen expression in two autopsied cases of anti-Hu antibody-positive paraneoplastic sensory neuronopathy (carcinomatous subacute sensory neuropathy). Pathological changes in both patients were limited to the primary sensory neurons, some of the sympathetic ganglia and hippocampal regions. The lesions showed a multifocal distribution that differed among the spinal segmental levels and in the individual dorsal root ganglia as well as in the nerve fascicles. Western blot analysis of the patients’ serum revealed that Hu antigens were extensively and widely expressed throughout the central nervous system, sensory and sympathetic ganglia and cancer cells, but not in the non-neural visceral tissues. Reverse transcriptase-polymerase chain reaction also showed that the Hu D, Hu C, Hel-N1 and Hel-N2 mRNAs were extensively and widely expressed through the neural tissues and cancer cells, but not in the visceral tissues. Thus, the distribution of antigen expression was very different from that of the lesions. Taken together with the distribution of lesions and Hu antigen expression, it is suggested that factors other than anti-Hu antibodies are also involved in the pathogenesis of this neuronopathy.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s004010050851
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  • 15
    Electronic Resource
    Electronic Resource
    The role of lymphotoxin in pathogenesis of polymyositis (2000)
    Springer
    Acta neuropathologica 100 (2000), S. 521-527 
    Details
    ISSN: 1432-0533
    Keywords: Key words Polymyositis ; Lymphotoxin ; Perforin ; Fas ; ligand
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Polymyositis (PM) is a cell-mediated autoimmune disease. Perforin (PF), Fas ligand (FasL) and TNF-α are considered to be important factors in cytotoxic T lymphocyte-mediated cell injury, and several studies have established a role of lymphotoxin (LT) in T helper type 1 (Th1)-induced cell-mediated autoimmune diseases. In the present study, to determine how LT, PF and FasL are involved in the pathogenesis of PM, we used immunohistochemical staining (IHC), reverse transcription polymerase chain reaction (RT-PCR), and in situ hybridization (ISH) on muscle specimens from patients with PM, amyotrophic lateral sclerosis (ALS), myotonic dystrophy (MyD) and controls (NC). There were many mononuclear cells (MNCs) immunoreactive for LT and some for PF and FasL within the fasciculus in PM muscles. On the other hand, only few or no LT-, PF- and FasL-positive cells were detected in MyD, ALS and NC muscles. The results of mRNA expression of these three molecules with RT-PCR were consistent with those using IHC methods. The number of MNCs positive for LT with ISH was far higher in PM compared to MyD, ALS and NC (P 〈 0.05 or 0.01). The MNCs located in the connective tissue or in the vicinity of necrotizing or non-necrotizing muscles were mainly LT mRNA and CD4 positive, while MNCs invading the non-necrotic fibers were mainly LT mRNA and CD8 positive. Our results indicated that the expression of LT was up-regulated in PM, and LT plays an important role in muscle injury and orchestrating the inflammatory reaction in PM.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s004010000207
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  • 16
    Electronic Resource
    Electronic Resource
    Spinal and cranial motor nerve roots in amyotrophic lateral sclerosis and X-linked recessive bulbospinal muscular atrophy: Morphometric and teased-fiber study (1981)
    Springer
    Acta neuropathologica 55 (1981), S. 227-235 
    Details
    ISSN: 1432-0533
    Keywords: Nerve roots ; Amyotrophic lateral sclerosis ; X-linked recessive bulbospinal muscular atrophy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Amyotrophic lateral sclerosis (ALS) and adult onset X-linked recessive bulbospinal muscular atrophy (SPMA), constituting the category of adult onset form of motor neuron disease, were analyzed on motor nerve roots. The results of morphometric analysis on ventral spinal roots (VSR) of all spinal segments from ALS and SPMA revealed the following three findings: (1) the large-myelinated α-motoneuron fibers were markedly decreased in number throughout all segments; (2) thin-myelinated autonomic preganglionic fibers were almost completely preserved; (3) small-intermediate-myelinated fibers which are considered to correspond to γ-motoneuron fibers were generally well preserved in ALS, but decreased by one-half to one-third in SPMA. However, all the components of the nerve roots of the oculomotor, trochlear, and abducent nerves were completely preserved in both ALS and SPMA. Moreover, the teasedfiber study showed that the regenerating-sprouting process rarely occurred in the VSR of ALS and SPMA. The present study suggested that the site of the primary lesion seems to be in the α-motoneuron fibers in motor neuron diseases, such as ALS or SPMA. However, the marked discrepancy in the pathologic change in the α-motoneuron fibers in the VSR and the nerve roots innervating the external ocular muscles was noteworthy.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00691322
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  • 17
    Electronic Resource
    Electronic Resource
    Locations of crossover breakpoints within the CMT1A-REP repeat in Japanese patients with CMT1A and HNPP (1997)
    Springer
    Human genetics 〈Berlin〉 99 (1997), S. 151-154 
    Details
    ISSN: 1432-1203
    Source: Springer Online Journal Archives 1860-2000
    Topics: Biology , Medicine
    Notes: Abstract The crossover breakpoints for Charcot-Marie-Tooth disease type 1A (CMT1A) and hereditary neuropathy with liability to pressure palsies (HNPP) are located in the CMT1A-REP repeat flanking a 1.5-Mb region of chromosome 17p11.2–12. The precise locations of the breakpoints are heterogeneous, and we analyzed the relative frequency distribution of breakpoints in 33 unrelated Japanese CMT1A and 3 unrelated HNPP families. The CMT1A-REP repeat region was divided into five regions, A, B, C, D and E, based on restriction site differences between the proximal and distal CMT1A-REP repeats. The frequency distribution of breakpoints within the CMT1A-REP repeat in the Japanese patients was 3% in region A, 78% in B/C and 19% in D, which is similar to that in Caucasian patients. This result also indicates that an 8-kb region defined by region B/C is a recombinational hotspot within the CMT1A-REP repeat in Japanese patients.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s004390050330
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  • 18
    Electronic Resource
    Electronic Resource
    Clinico-pathophysiological features of acute autonomic and sensory neuropathy: A long-term follow-up study (1995)
    Springer
    Journal of neurology 242 (1995), S. 623-628 
    Details
    ISSN: 1432-1459
    Keywords: Acute autonomic and sensory neuropathy ; Sensory ataxia ; Ganglioneuronopathy ; Neuron-specific enolase ; S-100b protein
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract We evaluated the clinicopathophysiological features of three patients with acute autonomic and sensory neuropathy (AASN) who were followed for over 3 years. Signs of an autonomic disturbance including vomiting, anhidrosis, urinary disturbances, orthostatic hypotension and reduced coefficient of variation of the R-R interval on electrocardiography gradually improved about 1 year after onset. However, all three exhibited severe generalized sensory impairment for all modalities with the development of persistent sensory ataxia. No sensory nerve action potentials could be elicited and no somatosensory evoked potentials could be obtained. Sural nerve biopsy revealed severe axonopathy. In two patients, a high-intensity area was observed in the posterior column of the spinal cord on T2*-weighted axial magnetic resonance images. The level of neuron-specific enolase in cerebrospinal fluid was markedly elevated in two patients, indicating spinal nerve root or sensory neuron damage. Motor nerve function was well preserved in all patients. Our findings suggests that the major lesion in patients with AASN, particularly those with a sensory deficit, is present in the dorsal root ganglion neurons, that is there is a ganglioneuronopathy.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00866911
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  • 19
    Electronic Resource
    Electronic Resource
    Acquired idiopathic generalized anhidrosis: a distinctive clinical syndrome (1988)
    Springer
    Journal of neurology 235 (1988), S. 428-431 
    Details
    ISSN: 1432-1459
    Keywords: Anhidrosis ; Hypohidrosis ; Sweat gland ; Ventilated capsular method ; IgE
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary The present study concerns a 28-year-old Japanese man with acquired generalized anhidrosis. The patient's ability to perspire was investigated in an artificial climate room maintained at 40°C and 40% humidity. Although the body temperature rose to 38°C, the patient did not sweat. Neither did sweating occur when the patient was given an intradermal injection of pilocarpine or nicotine. The serum IgE level was elevated. Atrophy and degeneration of the sweat glands, as well as infiltration by lymphocytes and mast cells around the sweat glands, were observed in skin biopsies. Anhidrosis in this patient was suggested to be the result of reduced function of the sweat glands themselves with possible underlying immunemediated basis.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00314488
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  • 20
    Electronic Resource
    Electronic Resource
    Relationship between respiratory failure and plasma noradrenaline levels in amyotrophic lateral sclerosis (1997)
    Springer
    Clinical autonomic research 7 (1997), S. 173-177 
    Details
    ISSN: 1619-1560
    Keywords: amyotrophic lateral sclerosis ; plasma noradrenaline ; head-up tilt test ; sympathetic hyperactivity ; respiratory failure ; chemoreceptor
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract We evaluated plasma noradrenaline (NA) levels at rest and during head-up tilt test in 20 patients with sporadic amyotrophic lateral sclerosis (ALS). Their fasting plasma NA levels ranged from 195 to 4227 pg/ml. The average plasma NA level was 483 pg/ml in five ambulatory patients, 341 in two wheelchair-bound patients, 1264 in 11 bedridden patients, and 208 in two respirator-dependent patients whose disability grading was the worst among the four groups. Arterial carbon dioxide (PCO2) was evaluated as a measure of respiratory function. The coefficient of correlation between PCO2 and plasma NA wasr=0.654 (p〈0.01). Either respiratory failure or lower motor neuron dysfunction may relate to the elevation of plasma NA levels. In the two bedridden patients, plasma NA levels and heart rate at rest increased significantly as the disease progressed. Cardiovascular responses to head-up tilting were normal. These data suggest that the elevation of plasma NA levels may be related to progression of respiratory failure and lower motor neuron dysfunction. In conclusion, sympathetic hyperactivity in ALS is considered to be not primary, but secondary to somatic motor disabilities and respiratory failure.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF02267978
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