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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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  • 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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  • 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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  • 4
    Electronic Resource
    Electronic Resource
    Electron-microscopic heterogeneity of onion-bulb neuropathies of the Déjerine-Sottas type (1974)
    Springer
    Acta neuropathologica 27 (1974), S. 105-118 
    Details
    ISSN: 1432-0533
    Keywords: Dejerine-Sottas Polyneuropathy ; Onion-Bulbs ; Demyelination and Remyelination ; Basement Membranes
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary The microscopic and electron microscopic findings in sural nerve biopsies of a brother and sister with chronic polyneuropathy of the Dejerine-Sottas type resemble those reported by Lyon (1969). A genetically determined variant appears to be involved. The relationship between this syndrome and onion-bulb neuropathies is discussed. It seems unlikely that only segmental demyelination and remyelination is responsible for onionbulb formation. The relationship between axons in the layers of onion-bulbs and the central fibre is discussed, and between basement membranes and Schwann cell processes and the central fibre. Significant quantitative evidence of axon degeneration and regeneration after primary axon degeneration is pointed out. Earlier probable cases in the literature are reviewed.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00687161
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  • 5
    Electronic Resource
    Electronic Resource
    Connatal Pelizaeus-Merzbacher Disease with congenital stridor in two maternal cousins (1981)
    Springer
    Acta neuropathologica 54 (1981), S. 11-17 
    Details
    ISSN: 1432-0533
    Keywords: Connatal Pelizaeus-Merzbacher disease ; Congenital stridor ; X-linked inheritance ; Psychomotor retardation ; Quantified cerebral morphology
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Two maternal cousins are described with the connatal form of Pelizaeus-Merzbacher Disease (PMD) and congenital stridor. Study of brain biopsy material confirms the diagnosis of PMD. The neuropathological findings are suggestive for the transitional form of this disease. Quantitative morphology gives support to the hypothesis that PMD is a disturbance in maturation of neurons and in myelin formation rather than an active degenerative process. The hereditary transmission is most consistent with a sex-linked recessive pattern. Different X-linked signs seem combined in the presented cases.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00691328
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  • 6
    Electronic Resource
    Electronic Resource
    Polyglucosan bodies in intramuscular motor nerves (1989)
    Springer
    Acta neuropathologica 77 (1989), S. 629-633 
    Details
    ISSN: 1432-0533
    Keywords: Polyglucosan bodies ; Muscle ; Nerve ; Lafora's disease ; Ageing
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary The presence of polyglucosan bodies was studied in intramuscular motor nerves of 292 muscle biopsies. These biopsies were classified into five diagnostic categories and investigated for the presence of polyglucosan bodies in relation to age and sex. Their presence was nonspecific in patients over 20 years, the only correlation being with ageing. Under 20 years, their presence pointed to the diagnosis of Lafora's disease. In cases in which both a muscle biopsy and a sural nerve biopsy were performed, the former appeared to contain these polyglucosans more frequently.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00687891
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  • 7
    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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  • 8
    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
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  • 9
    Electronic Resource
    Electronic Resource
    Neuropathological findings in muscle-eye-brain disease (MEB-D) (1991)
    Springer
    Acta neuropathologica 83 (1991), S. 55-60 
    Details
    ISSN: 1432-0533
    Keywords: Muscle-eye-brain disease ; Fukuyama type of congenital muscular dystrophy ; Neuropathological differentiation
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Congenital muscular dystrophy (CMD) associated with cerebro-ocular dysplasia named muscle-eye-brain disease (MEB-D) is described in two sisters. Progressive hypotonia, mental retardation and severe visual failure appeared immediately after birth. Pathological examination demonstrated muscular dystrophy, hydrocephalus, type II lissencephaly and defective eye development of foetal origin. The great similarity of the clinical and neuropathological picture of both sisters is in agreement with an autosomal recessive inheritance. Neuropathological distinction between Fukuyama-CMD and MEB-D is a more severe and earlier cerebral developmental defect and the association with ocular dysplasia in MEB-D.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00294430
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  • 10
    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
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