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  • 1
    Electronic Resource
    Electronic Resource
    Peripheral nerve elongation by laser Doppler flowmetry controlled expansion: morphological aspects (1995)
    Springer
    Acta neuropathologica 89 (1995), S. 166-171 
    Details
    ISSN: 1432-0533
    Keywords: Key words Peripheral nerve ; Expansion ; Elongation ; Paranodal widening ; Remyelination
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Peripheral nerve elongation by a tissue expander may offer an alternative to nerve grafting in the management of segmental nerve loss. We investigated the morphological changes in peripheral nerve following slow nerve elongation by laser Doppler flowmetry controlled expansion in a rabbit sciatic nerve model. The animals were randomly assigned to one of four groups, with an expander volume of 0, 5, 10 or 15 cm3, respectively. An elongation of up to 40% was possible with preservation of clinical function. Nerve conduction velocity decreased in relation to elongation. Paranodal widening, followed by remyelination of the node, were early and constant morphological features. Demyelination and remyelination of whole internodes, and axonal degeneration occurred sporadically and did not correlate with elongation, rate of elongation or neurophysiological parameters. The model of laser Doppler flowmetry controlled nerve expansion provides a method for remodelling of myelin sheaths and lengthening of nerve fibers without axonal damage.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00296361
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    Paper (German National Licenses)
    Fulltext
  • 2
    Electronic Resource
    Electronic Resource
    Chronic inflammatory demyelinating polyneuropathy or hereditary motor and sensory neuropathy? (1993)
    Springer
    Acta neuropathologica 86 (1993), S. 630-635 
    Details
    ISSN: 1432-0533
    Keywords: Chronic inflammatory demyelinating polyneuropathy ; Hereditary motor and sensory neuropathy type I ; Demyelinating neuropathy ; Mononuclear cell infiltrates ; Myelinated fibre-size histogram
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract The pathological changes generally considered to distinguish chronic inflammatory demyelinating polyneuropathy (CIDP) from hereditary motor and sensory neuropathy (HMSN) are: mononuclear cell infiltrates, prominent endoneurial oedema, and marked fascicle-to-fascicle variability. We evaluated the diagnostic significance of these pathological features which are suggestive of CIDP. Nerve biopsies from 42 dominant HMSN type I cases with a normal disease course were investigated for the occurrence of inflammatory features. A small cluster of mononuclear cells was found in 12% of the cases and marked endoneurial oedema in 21%. Variability in pathology between the fascicles was not observed. The histogram configuration yielded additional information for differential diagnosis. Subsequently, we reviewed the clinical, electrophysiological and morphological features of 18 sporadic cases of chronic progressive demyelinating motor and sensory neuropathy with mainly classic onion bulbs in their nerve biopsies and a disease onset in the first decade. In all these patients DNA investigation for the 17p11.2 duplication was performed. According to the results of the DNA investigation, autosomal dominant HMSN type Ia was diagnosed in eight patients, although in six slight ‘CIDP-positive’ features were present. A diagnosis was definite or most probable CIDP in eight patients. In two patients no definite diagnosis could be made. Testing for the presence of the 17p11.2 duplication is, therefore, helpful in distinguishing between CIDP and HMSN type I. The diagnosis of CIDP requires careful evaluation of the clinical, electrophysiological and morphological data to avoid false-positive diagnoses of inflammatory disorders.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00294303
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    Paper (German National Licenses)
    Fulltext
  • 3
    Electronic Resource
    Electronic Resource
    Congenital muscular dystrophy and severe central nervous system atrophy in two siblings (1995)
    Springer
    Acta neuropathologica 90 (1995), S. 650-656 
    Details
    ISSN: 1432-0533
    Keywords: Key words Congenital muscular dystrophy ; Cerebral atrophy ; Myelin deficiency ; Multisystem degeneration ; Sural nerve
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Severe degenerative features of the nervous system of a hitherto unknown kind, associated with a neuromuscular disorder with histopathological features of congenital muscular dystrophy, are reported in two female siblings. The clinical profile was characterized by generalized hypotonia followed by spastic tetraplegia, contractures, polyneuropathy, lack of cognitive development and progressive microcephaly. There was no involvement of the eyes. Neuropathological examination of the brain of one sibling, who died at the age of 30 months, revealed subtotal loss of neurons in the cerebral and cerebellar cortex and in the ventral pons, and secondary loss of myelin in the cerebral and cerebellar subcortical white matter. Sural nerve biopsy in the other sibling, who had a similar neurological affection, showed a lack of large myelinated fibers.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00318580
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    Paper (German National Licenses)
    Fulltext
  • 4
    Electronic Resource
    Electronic Resource
    Polyglucosan bodies in sural nerve biopsies (1990)
    Springer
    Acta neuropathologica 80 (1990), S. 554-557 
    Details
    ISSN: 1432-0533
    Keywords: Polyglucosan bodies ; Sural nerve biopsy ; Lafora's disease ; Ageing
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary The presence of polyglucosan bodies in sural nerves collected over a 16-year period was studied in relation to age, sex, presence of polyneuropathy, and concomitant presence of central nervous system disorder. Polyglucosan bodies have been seen in only one patient without a polyneuropathy. This patient was suffering from Lafora's disease. In all other sural nerves positive for polyglucosan bodies a polyneuropathy was present. Within this group the prevalence of polyglucosan bodies was positively correlated with age, and if a central nervous system disorder was associated, this prevalence was more distinct. With semiquantitative measurements of the surface of polyglucosan bodies a significant correlation was found between age and percentage of large bodies.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00294618
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    Paper (German National Licenses)
    Fulltext
  • 5
    Electronic Resource
    Electronic Resource
    Charcot-Marie-Tooth disease type 1A: morphological phenotype of the 17p duplication versus PMP22 point mutations (1995)
    Springer
    Acta neuropathologica 90 (1995), S. 645-649 
    Details
    ISSN: 1432-0533
    Keywords: Key words Charcot-Marie-Tooth disease ; Dejerine-Sottas disease ; Hereditary motor and sensory neuropathy ; PMP22 mutations ; Nerve biopsy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Charcot-Marie-Tooth disease type 1A (CMT1A) or hereditary motor and sensory neuropathy type Ia (HMSN type Ia) is an autosomal dominant demyelinating polyneuropathy, which may result from duplications as large as 1.5 Mb on chromosome 17p11.2–p12 encompassing the gene for the peripheral myelin protein PMP22, or from point mutations in this gene. In general, it is not possible to distinguish, by clinical and neurophysiological criteria, the cases associated with the duplication mutation from those associated with point mutations of the PMP22 gene, although the latter tend to be more severe. In this study we demonstrated that the two genotypes exhibit different morphological characteristics. In the PMP22 duplicated cases the mean g-ratio (axon diameter versus fibre diameter) is significantly lower than normal, while in cases of PMP22 point mutations nearly all myelinated fibres have an extremely high g-ratio. In cases with point mutations, onion bulbs are abundantly present from an early age, whereas onion bulbs in the duplicated cases develop gradually in the first years of life. Increase in total transverse fascicular area is most pronounced in the point mutation cases. The differences in pathology between these two very different types of mutations involving the same gene likely reflect differences in pathogenesis and may offer clues in understanding the function of PMP22.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00318579
    Library Location Call Number Volume/Issue/Year Availability
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    Paper (German National Licenses)
    Fulltext
  • 6
    Electronic Resource
    Electronic Resource
    Congenital muscular dystrophy and severe central nervous system atrophy in two siblings (1995)
    Springer
    Acta neuropathologica 90 (1995), S. 650-656 
    Details
    ISSN: 1432-0533
    Keywords: Congenital muscular dystrophy ; Cerebral atrophy ; Myelin deficiency ; Multisystem degeneration ; Sural nerve
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Severe degenerative features of the nervous system of a hitherto unknown kind, associated with a neuromuscular disorder with histopathological features of congenital muscular dystrophy, are reported in two female siblings. The clinical profile was characterized by generalized hypotonia followed by spastic tetraplegia, contractures, polyneuropathy, lack of cognitive development and progressive microcephaly. There was no involvement of the eyes. Neuropathological examination of the brain of one sibling, who died at the age of 30 months, revealed subtotal loss of neurons in the cerebral and cerebellar cortex and in the ventral pons, and secondary loss of myelin in the cerebral and cerebellar subcortical white matter. Sural nerve biopsy in the other sibling, who had a similar neurological affection, showed a lack of large myelinated fibers.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00318580
    Library Location Call Number Volume/Issue/Year Availability
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    Paper (German National Licenses)
    Fulltext
  • 7
    Electronic Resource
    Electronic Resource
    Charcot-Marie-Tooth disease type 1A: morphological phenotype of the 17p duplication versus PMP22 point mutations (1995)
    Springer
    Acta neuropathologica 90 (1995), S. 645-649 
    Details
    ISSN: 1432-0533
    Keywords: Charcot-Marie-Tooth disease ; Dejerine-Sottas disease ; Hereditary motor and sensory neuropathy ; PMP22 mutations ; Nerve biopsy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Charcot-Marie-Tooth disease type 1A (CMT1A) or hereditary motor and sensory neuropathy type Ia (HMSN type Ia) is an autosomal dominant demyelinating polyneuropathy, which may result from duplications as large as 1.5 Mb on chromosome 17p11.2–p12 encompassing the gene for the peripheral myelin protein PMP22, or from point mutations in this gene. In general, it is not possible to distinguish, by clinical and neurophysiological criteria, the cases associated with the duplication mutation from those associated with point mutations of the PMP22 gene, although the latter tend to be more severe. In this study we demonstrated that the two genotypes exhibit different morphological characteristics. In the PMP22 duplicated cases the mean g-ratio (axon diameter versus fibre diameter) is significantly lower than normal, while in cases of PMP22 point mutations nearly all myelinated fibres have an extremely high g-ratio. In cases with point mutations, onion bulbs are abundantly present from an early age, whereas onion bulbs in the duplicated cases develop gradually in the first years of life. Increase in total transverse fascicular area is most pronounced in the point mutation cases. The differences in pathology between these two very different types of mutations involving the same gene likely reflect differences in pathogenesis and may offer clues in understanding the function of PMP22.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00318579
    Library Location Call Number Volume/Issue/Year Availability
    BibTip Others were also interested in ...
    Paper (German National Licenses)
    Fulltext
  • 8
    Electronic Resource
    Electronic Resource
    Peripheral nerve elongation by laser Doppler flowmetry controlled expansion: morphological aspects (1995)
    Springer
    Acta neuropathologica 89 (1995), S. 166-171 
    Details
    ISSN: 1432-0533
    Keywords: Peripheral nerve ; Expansion ; Elongation ; Paranodal widening ; Remyelination
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Peripheral nerve elongation by a tissue expander may offer an alternative to nerve grafting in the management of segmental nerve loss. We investigated the morphological changes in peripheral nerve following slow nerve elongation by laser Doppler flowmetry controlled expansion in a rabbit sciatic nerve model. The animals were randomly assigned to one of four groups, with an expander volume of 0, 5, 10 or 15 cm3, respectively. An elongation of up to 40% was possible with preservation of clinical function. Nerve conduction velocity decreased in relation to elongation. Paranodal widening, followed by remyelination of the node, were early and constant morphological features. Demyelination and remyelination of whole internodes, and axonal degeneration occurred sporadically and did not correlate with elongation, rate of elongation or neurophysiological parameters. The model of laser Doppler flowmetry controlled nerve expansion provides a method for remodelling of myelin sheaths and lengthening of nerve fibers without axonal damage.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00296361
    Library Location Call Number Volume/Issue/Year Availability
    BibTip Others were also interested in ...
    Paper (German National Licenses)
    Fulltext
  • 9
    Electronic Resource
    Electronic Resource
    Evidence for genetic heterogeneity underlying hereditary neuropathy with liability to pressure palsies (1994)
    Springer
    Human genetics 〈Berlin〉 93 (1994), S. 151-156 
    Details
    ISSN: 1432-1203
    Source: Springer Online Journal Archives 1860-2000
    Topics: Biology , Medicine
    Notes: Abstract Hereditary neuropathy with liability to pressure palsies (HNPP) is a disorder of the peripheral nervous system, the cause of which has recently been identified as a deletion on chromosome 17p. The deletion corresponds to the duplication that is commonly observed in patients with hereditary motor and sensory neuropathy type Ia (HMSNIa, 17p11.2–p12). Therefore, the gene for peripheral myelin protein 22 (PMP-22) is a candidate gene for both HMSNIa and HNPP. Here, we show that a similar deletion is present in one family with HNPP but is clearly absent in another family. Affected members of this family carry the expected two copies of the PMP-22 gene and the surrounding region. Furthermore, linkage analyses of this family exclude a large part of 17p, spanning the area deleted in other families with HNPP, as the location for the disease gene. These data strongly argue for the existence of genetic heterogeneity underlying HNPP. Results from two-point linkage analysis with markers on chromosome 1q are inconsistent with a possible involvement of the locus for HMSNIb in the present family.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00210601
    Library Location Call Number Volume/Issue/Year Availability
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    Paper (German National Licenses)
    Fulltext
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