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  • Human  (3)
  • Sural nerve biopsy  (3)
  • 1
    ISSN: 1432-0533
    Keywords: Key words Adrenoleukodystrophy ; Adrenomyeloneuropathy ; Mitochondrial abnormalities ; Sural nerve biopsy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Clinical and sural nerve biopsy findings in two brothers and their mother affected by adrenomyeloneuropathy/adrenoleukodystrophy (AMN/ALD) illustrate the variability of histopathological changes in this disorder. The number of diagnostic inclusions, i.e., trilaminar leaflets, varied considerably from case to case and showed no correlation to the extent of neurological symptoms. In addition, mitochondrial abnormalities (granular and filamentous inclusions and abnormal cristae), which have not previously been described in AMN/ALD, were detected. These alterations could be secondary to the peroxisomal defect and the increased amount of very long chain fatty acids or could be caused by a more generalized defect.
    Type of Medium: Electronic Resource
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  • 2
    ISSN: 1432-0533
    Keywords: Key words X-linked Charcot-Marie-Tooth disease ; Charcot-Marie-Tooth disease type 2 ; Hereditary ; motor and sensory neuropathy ; Connexin32 mutations ; Sural nerve biopsy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract X-linked Charcot-Marie-Tooth neuropathy (CMTX) is caused by mutations in the connexin32 gene on Xq13. Because of overlapping morphological and clinical data, CMTX patients often meet the criteria of autosomal-dominant CMT2, the neuronal type of CMT. Hence, it might be useful to analyse the connexin32 gene in suspected CMT2 patients when there is no male-to-male transmission. We selected a cohort of 30 patients who were considered having CMT2 on the basis of previous clinical and histopathological evaluation. DNA was extracted from paraffin-embedded sural nerve biopsy samples and screened for connexin32 mutations to verify the possible diagnosis of CMTX. In 2 patients mutations were found corresponding to amino acid substitutions of arginine for tryptophan in codon 15 and arginine for glutamine in codon 22 of connexin32. This study illustrates that archival material allows genetic classification of suspected CMT cases. Furthermore, there is additional proof that connexin32 mutations represent the underlying genetic defect in some cases of predominantly neuronal CMT.
    Type of Medium: Electronic Resource
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  • 3
    ISSN: 1432-0533
    Keywords: Key words B-50(GAP-43) ; Spinal cord ; Human
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract B-50(GAP-43) is a phosphoprotein mainly found in the nervous system which plays a major role in neurite growth during development and regeneration as well as in synaptic remodelling. In the mature intact central nervous system, intense B-50 immunoreactivity (B-50-IR) can still be detected in regions which maintain residual capacity for structural re-organization. B-50 expression has been studied extensively in laboratory animals; however, its distribution and regulation in the human spinal cord is largely unknown. As a first step to analyze lesion-induced structural alterations, we investigated the distribution of B-50 protein and mRNA in the normal adult human spinal cord and dorsal root ganglia. Intense B-50-IR was localized to the superficial laminae of the dorsal horn at all segmental levels, the intermediolateral nucleus at thoracic levels and Onuf’s nucleus at sacral levels. Scattered neurons, particularly in the ventral horn of lumbar and sacral segmental levels (and occasionally also in Clarke’s nucleus) displayed intense B-50-IR in close apposition to the perikaryal and proximal dendritic surfaces. Nonradioactive in situ hybridization indicated that B-50 mRNA could also be detected in neurons of the ventral horn and also in the intermediolateral nucleus. The distribution of B-50 mRNA and protein in the normal human spinal cord shows a marked similarity to that reported in experimental animals, including the selective labelling of Onuf’s nucleus. However, the strong B-50-IR on the surface of some large anterior horn motor neurons has not been observed in other mammals. This finding might reflect a particular state of readiness for synaptic plasticity.
    Type of Medium: Electronic Resource
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  • 4
    ISSN: 1432-0533
    Keywords: Cerebral autosomal dominant arteriopathy Immunoglobulin deposits ; Peripheral neuropathy ; Sural nerve biopsy ; Small vessel disease
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy' (CADASIL) has recently been identified as a hereditary disorder with characteristic fine structural changes of small intracerebral arteries and arterioles. Electron microscopically there are characteristic perivascular deposits of granular electron-dense material resembling immunoglobulin deposits. The present case from a family with four affected members in three successive generations shows that similar vascular changes as described in the central nervous system are present in blood vessels of the sural nerve, although less pronounced and, therefore, affording electron microscopy for their unequivocal detection. Nevertheless it has been shown for the first time that the diagnosis of CADASIL can be verified by a sural nerve biopsy. Occasional focal accumulation of pinocytotic vesicles opposite the granular deposits suggests exocytosis as one of the possible pathomechanisms for their production.
    Type of Medium: Electronic Resource
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  • 5
    Electronic Resource
    Electronic Resource
    Springer
    Cell & tissue research 290 (1997), S. 31-37 
    ISSN: 1432-0878
    Keywords: Key words: Sural nerve ; Blood vessels ; Angiopathic neuropathy ; Vasculitis ; Peripheral neuropathy ; Human
    Source: Springer Online Journal Archives 1860-2000
    Topics: Biology , Medicine
    Notes: Abstract. The number and dimensions of epineurial blood vessels in normal human sural nerves have, thus far, not been determined using systematic, reproducible morphometric methods, although this nerve is most frequently used for diagnostic biopsies. Quantitative changes in epineurial blood vessels appear to be major parameters for identifying angiopathy and angiopathic peripheral neuropathy. Therefore, we examined the epineurial blood-vessel number in relation to the age of the patients and to the number and size of the nerve fascicles in each of 51 human sural nerve biopsies. The data from a control group were compared with pathological cases. We found that the number of epineurial blood vessels (normal mean: 57.7) increased significantly (up to 196) in biopsies where there were signs of angiopathy (P≤0.01) or vasculitis (P≤0.05). The increase in the number of epineurial blood vessels usually resulted from a proliferation of capillaries. The fascicular cross-sectional area did not appear to be related to the number of epineurial blood vessels, although it increased significantly in cases with vasculitis (P≤0.05) or an axonal type of neuropathy (P≤0.05). Thus, this study shows that the number of epineurial blood vessels is a helpful parameter in verifying angiopathy and angiopathic peripheral neuropathy.
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  • 6
    Electronic Resource
    Electronic Resource
    Springer
    Cell & tissue research 273 (1993), S. 499-509 
    ISSN: 1432-0878
    Keywords: Ranvier's node ; Development ; Sural nerve ; Axon ; Myelin sheath ; Paranodal junctions ; Human
    Source: Springer Online Journal Archives 1860-2000
    Topics: Biology , Medicine
    Notes: Abstract Developmental alterations of paranodal fiber segments have not been investigated systematically in human nerve fibers at the light- and electron-microscopic level. We have therefore analyzed developmental changes in the fine structure of the paranode in 43 human sural nerves during the axonal growth period up to 5 years of age, and during the subsequent myelin development up to 20 years and thereafter. The nodal, internodal, and paranodal axon diameters reach their adult values at 4–5 years of age. The ratio between internodal and paranodal axon diameters remains constant at 1.8–2.0. Despite a considerable increase in myelin sheath thickness, the length of the paranodal myelin sheath attachment zone at the axon does not increase correspondingly, because of attenuation, separation from the axolemma, and piling up of myelin loops in the paranode. Separation of variable numbers of terminal myelin loops from the underlying axolemma results in the formation of bracelets of Nageotte, whereas the transverse bands of these loops disappear. The adaptation of the paranodal myelin sheath to axonal expansion during development probably occurs by uneven gliding of the paranodal myelin loops simultaneously with internodal slippage of myelin lamellae. Since mechanically stabilizing structures (tight junctions and desmosomes between adjacent paranodal myelin processes; transverse bands between myelin loops and paranodal axolemma) are unevenly arranged, especially during rapid axonal growth, paranodal axonal growth with simultaneous adaptation of the myelin sheath is probably discontinuous with time.
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