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  • 1
    Electronic Resource
    Electronic Resource
    Springer
    Journal of neurology 231 (1984), S. 75-78 
    ISSN: 1432-1459
    Keywords: Amyotrophic lateral sclerosis ; Cerebrospinal fluid ; Blood-brain barrier ; Isoelectric focusing
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Zusammenfassung In 90 Fällen von amyotrophischer Lateralsklerose und bei 50 auch im Alter entsprechenden Kontrollen wurde der Liquor untersucht. Der Eiweißgehalt war bei den Patienten mit ALS signifikant höher als bei den Kontrollen. Die Albuminfraktion und das IgG, die mit Immunodiffusionsmethoden quantitativ bestimmt wurden, waren bei der ALS im Liquor signifikant erhöht, während sie sich im Serum gleich wie bei den Kontrollfällen verhielten. Bei der isoelektrischen Fokusierung ließ sich ein eindeutiges „Finger-print-pattern“ in elf von zwölf Fällen beobachten. Diese Befunde sprechen dafür, daß bei der ALS eine Störung der Blut-Hirnschranke vorliegt. Es wird kurz auf die Beobachtung einer vermehrten Zahl mononukleärer Zellen bei jungen Patienten mit ALS eingegangen und im besonderen die Frage diskutiert, in wieweit ein exogenes Agents in der Pathogenese eine Rolle spielen könnte. Mindestens eines der obern erwähnten Parameter war bei 45% aller ALS Fälle im Liquor verändert.
    Notes: Summary The cerebrospinal fluid (CSF) was examined in 90 amyotrophic lateral sclerosis (ALS) patients and in 50 age-matched normal controls. Total protein concentration was significantly higher in ALS patients than in normal controls. CSF IgG and albumin, quantitatively determined by single radial immunodiffusion, were significantly increased in ALS. No difference in serum concentrations was observed between ALS patients and normal controls. On isoelectric focusing a clear-cut “fingerprint” pattern was observed in 11 of 12 cases. These findings support the hypothesis that blood-brain barrier damage occurs in ALS. The finding of a higher mononuclear cell count in young ALS patients is briefly discussed in the light of the hypothesis that an exogenous agent might be of some relevance in pathogenesis. An alteration of at least one of the CSF parameters considered was found in 45.5% of ALS cases.
    Type of Medium: Electronic Resource
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  • 2
    Electronic Resource
    Electronic Resource
    Springer
    Neurological sciences 11 (1990), S. 57-59 
    ISSN: 1590-3478
    Keywords: Globoid cell leukodystrophy ; Krabbe disease ; CSF immunoglobulins
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Sommario Gli autori riportano lo studio clinico e biochimico di un caso di malattia di Krabbe tardiva. L'esordio acuto dei sintomi neurologici, dopo una affezione febbrile respiratoria, il profilo immunochimico del liquor con produzione locale di IgG e la risposta iniziale al trattamento con ACTH hanno fatto supporre una genesi infiammatoria. È stata tuttavia diagnosticata in seguito una malattia di Krabbe. Gli Autori discutono brevemente il significato di una possibile produzione di IgG nel Sistema Nervoso Centrale in questo caso.
    Notes: Abstract During a febrile upper respiratory tract illness this 4 year old boy developed left hemiparesis, which progressed to loss of walking and even of sitting finally to tetraplegia. The cerebrospinal fluid protein pattern showed blood-brain barrier damage with additional intrathecal IgG synthesis. The symptoms responded to steroid therapy but resumed and worsened on withdrawal. Only late, when visual evoked potentials and nerve conduction velocity proved to be impaired, was Krabbe disease diagnosed on the assay of cultured fibroblasts for galactocerebroside-beta-galactosidase. We discuss the significance of possible endogenous production of IgG in the CNS.
    Type of Medium: Electronic Resource
    Library Location Call Number Volume/Issue/Year Availability
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