Library

X
feed icon rss

Your email was sent successfully. Check your inbox.

An error occurred while sending the email. Please try again.

Proceed reservation?

Export
  • 1
    Electronic Resource
    Electronic Resource
    Is the sensory neuropathy in ataxia-telangiectasia distinguishable from that in Friedreich's ataxia? (1986)
    Springer
    Acta neuropathologica 69 (1986), S. 213-219 
    Details
    ISSN: 1432-0533
    Keywords: Ataxia ; Telangiectasia ; Friedreich's ataxia ; Peripheral sensory neuropathy ; Sural nerve biopsy ; Loss of large fibers
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary The bioptical morphometric and ultrastructural study of sural nerve in a 17-year-old boy with ataxia-telangiectasia (AT) is reported. Our findings include a loss of fibers, particularly of large ones, axonal degenerative changes, Schwann cell inclusions of various type, and rare signs of primary demyelination. Teased-fiber study showed paranodal myelin enlargements, segmental demyelination, shortening, and/or variability of internodal length. This picture is similar to that in Friedreich's ataxia (FA), although they differ in degree and time of onset. A correct neuropathologic diagnosis of AT cannot be made on the basis of sural nerve biopsy alone.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00688296
    Library Location Call Number Volume/Issue/Year Availability
    BibTip Others were also interested in ...
    Paper (German National Licenses)
    Fulltext
  • 2
    Electronic Resource
    Electronic Resource
    Correlation of clinical symptoms, HVA and 5-HIAA in CSF and plasma L-DOPA in parkinsonian patients treated with L-DOPA and L-DOPA + RO 4-4602 (1977)
    Springer
    European journal of clinical pharmacology 11 (1977), S. 255-261 
    Details
    ISSN: 1432-1041
    Keywords: HVA ; 5-HIAA ; L-DOPA ; DOPA decarboxylase inhibitor ; Ro 4-4602 ; parkinsonism
    Source: Springer Online Journal Archives 1860-2000
    Topics: Chemistry and Pharmacology , Medicine
    Notes: Summary Two groups of 6 patients with Parkinson's disease were treated: (Group A) with L-DOPA for 65 days, or (Group B) with L-DOPA+Ro 4-4602 for 30 days. At stated intervals, both groups underwent clinical evaluation, lumbar puncture and measurement of L-DOPA in plasma. A significant improvement in total disability (Webster scale) and akinesia was observed in both groups, but rigidity was improved only in Group B. HVA in CSF increased from its low basal value much more in Group A than in B; 5-HIAA did not change. L-DOPA curves in plasma were similar in the two groups. Baseline Webster scale and akinesia were inversely correlated with basal HVA in all 12 patients. Basal HVA was also inversely correlated with the same clinical parameters evaluated at the end of the trial in all patients. Furthermore, L-DOPA peaks were directly related to CSF HVA level and to improvement (W. R. S. and akinesia) only in patients receiving the combined treatment (Group B). It was concluded that in parkinsonian syndromes clinical improvement was inversely related to severity of the illness and basal HVA in CSF, independent of the type of treatment; treatment with L-DOPA+Ro 4-4602 gave better clinical results than L-DOPA alone, and it restored CSF HVA to a more normal level; the combination with Ro 4-4602 made it possible to administer only one fifth of the quantity of L-DOPA and still to obtain the same L-DOPA level in plasma with better clinical results; and, in patients treated with L-DOPA+Ro 4-4602, the L-DOPA plasma peak was directly related to CSF HVA, Webster scale and akinesia. This means that, once peripheral decarboxylation of L-DOPA has been abolished, plasma levels are very important for L-DOPA availability in the brain.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00607673
    Library Location Call Number Volume/Issue/Year Availability
    BibTip Others were also interested in ...
    Paper (German National Licenses)
    Fulltext
  • 3
    Electronic Resource
    Electronic Resource
    Drugs Affecting Movement Disorders (1987)
    Palo Alto, Calif. : Annual Reviews
    Annual Review of Pharmacology 27 (1987), S. 113-136 
    Details
    ISSN: 0362-1642
    Source: Annual Reviews Electronic Back Volume Collection 1932-2001ff
    Topics: Medicine , Chemistry and Pharmacology
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1146/annurev.pa.27.040187.000553
    Library Location Call Number Volume/Issue/Year Availability
    BibTip Others were also interested in ...
    Paper (German National Licenses)
    Fulltext
  • 4
    Electronic Resource
    Electronic Resource
    Glutamate Dehydrogenase in Human Brain: Regional Distribution and Properties (1986)
    Oxford, UK : Blackwell Publishing Ltd
    Journal of neurochemistry 46 (1986), S. 0 
    Details
    ISSN: 1471-4159
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Abstract: Glutamate dehydrogenase (GDH) activity was studied in 17 regions of six human brains. Duration and conditions of the postmortem period did not affect enzyme activity. Specific activity ranged between 103 and 377 nmoles/min/mg protein at 25°C and it was 10-fold higher than that found in leukocytes. Apart from exclusively white matter regions (corpus callosum and centrum ovale), there was a moderate regional distribution (2.5-fold variation), with highest values in the inferior olive and hypothalamus, and lowest in the cerebellum and lenticular nucleus. With α-ketoglutarate (α-KG), NADH, or NH4+ as variable substrate, the apparent Km values in human brain were Kmα-KG= 1.9 × 10−3 M, KmNADH= 0.21 × 10−3 M, and KmNH4+= 28 × 10−3 M, and in leukocytes they were Kmα-KG= 1.7 × 10−3 M, KmNADH= 0.24 × 10−3 M, and KmNH4+= 28 × 10−3 M. The effects of cofactors, inhibitor, and pH were similar in brain and leukocyte GDH.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1111/j.1471-4159.1986.tb12985.x
    Library Location Call Number Volume/Issue/Year Availability
    BibTip Others were also interested in ...
    Paper (German National Licenses)
    Fulltext
  • 5
    Electronic Resource
    Electronic Resource
    Similarities Between A.P.U.D. Cells and Monoaminergic Neurons: Evidence for a Dopamine Uptake Mechanism in C Cells of Chicken Ultimobranchial Gland (1980)
    Oxford, UK : Blackwell Publishing Ltd
    Journal of neurochemistry 34 (1980), S. 0 
    Details
    ISSN: 1471-4159
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Abstract: The uptake of [3H]dopamine was studied in the C cells of chicken ultimobranchial gland (UBG). DA uptake appears to be an active and saturable mechanism (Km= 8.6 μM). The C cells' affinity for DA is intermediate between dopaminergic synaptosomes and such extraneural tissues as platelets and heart. UBG incubation with DA leads to the synthesis of a noticeable quantity of DOPAC, indirectly showing the presence of MAO activity. Thus UBG C cells, besides having both an embryological derivation from the neural crest and some morphological aspects similar to synaptosomes, also possess an active uptake mechanism for a central neurotransmitter.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1111/j.1471-4159.1980.tb11173.x
    Library Location Call Number Volume/Issue/Year Availability
    BibTip Others were also interested in ...
    Paper (German National Licenses)
    Fulltext
  • 6
    Electronic Resource
    Electronic Resource
    Magnetic resonance imaging in “typical” and “late onset” Friedreich's disease and early onset cerebellar ataxia with retained tendon reflexes (1995)
    Springer
    Neurological sciences 16 (1995), S. 303-308 
    Details
    ISSN: 1590-3478
    Keywords: magnetic resonance imaging ; Friedreich's disease ; early onset cerebellar ataxia with retained tendon reflexes
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Sommario La Risonanza Magnetica permette lo studio morfologico “in vivo” dell'encefalo e del midollo spinale nei pazienti con atassia ereditaria. Noi abbiamo eseguito uno studio con immagini T1- e T2-pesate in 11 pazienti con Malattia di Freidreich (MF), 5 con MF ad esordio tardivo e 10 con atassia cerebellare ad esordio precoce con conservazione dei riflessi osteotendinei (Early Onset Cerebellar Ataxia with retained tendon reflexes, EOCA). L'atrofia del midollo cervicale era costante in MF ed MF ad esordio tardivo, spesso in associazione con atrofia cerebellare e del tronco. Le immagini T2-pesate mostravano degenerazione dei cordoni posteriori nel midollo cervicale. In EOCA il reperto più frequente era l'atrofia cerebellare, pura od in associazione con atrofia del midollo cervicale o del tronco. L'atrofia cerebellare in EOCA era marcata in pochi casi ed era correlata con la durata di malattia.
    Notes: Abstract MRI makes it possible to study the in vivo brain and spinal cord morphology of patients with hereditary ataxia. We performed T1-and T2-weighted studies in eleven patients with Friedreich's disease (FD), five with “late onset” FD and ten with early onset cerebellar ataxia with retained tendon reflexes (EOCA). Cervical cord atrophy was constant in FD and “late onset” FD and often associated with atrophy of the cerebellum and of the brainstem; T2-weighted studies showed posterior column degeneration in the cervical cord. The most frequent finding in EOCA was cerebellar atrophy, pure or associated with cervical cord or brainstem atrophy; the cerebellar atrophy was marked in a few cases and was related to disease duration.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF02249105
    Library Location Call Number Volume/Issue/Year Availability
    BibTip Others were also interested in ...
    Paper (German National Licenses)
    Fulltext
  • 7
    Electronic Resource
    Electronic Resource
    Update on genetics of Huntington's disease: availability of direct and accurate predictive test (1996)
    Springer
    Neurological sciences 17 (1996), S. 185-187 
    Details
    ISSN: 1590-3478
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF01995681
    Library Location Call Number Volume/Issue/Year Availability
    BibTip Others were also interested in ...
    Paper (German National Licenses)
    Fulltext
  • 8
    Electronic Resource
    Electronic Resource
    Genetic data and natural history of Friedreich's disease: a study of 80 Italian patients (1990)
    Springer
    Journal of neurology 237 (1990), S. 345-351 
    Details
    ISSN: 1432-1459
    Keywords: Spinocerebellar degeneration ; Friedreich's disease ; Diagnostic criteria ; Genetics ; Natural history
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary The clinical and genetic features of 80 patients with Friedreich's disease from 64 families are described. Diagnostic criteria were: no evidence of dominant inheritance, onset by the age of 20 years, progressive unremitting ataxia of limbs and gait, and absence of knee and ankle jerks. Furthermore, at least one of the following accessory signs was present: dysarthria, extensor plantar response and echocardiographic evidence of hypertrophic cardiomyopathy. Two peaks of onset age were evident at 6–9 and 12–15 years. Analysis of intrafamily variation of onset age and absence of clustering of cardiomyopathy and diabetes did not suggest genetic heterogeneity. Peripheral nerve impairment was an early finding and showed slight further progression, whereas involvement of the cerebellar and corticospinal pathways appeared later and mainly accounted for the progressive worsening of the disease.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00315657
    Library Location Call Number Volume/Issue/Year Availability
    BibTip Others were also interested in ...
    Paper (German National Licenses)
    Fulltext
  • 9
    Electronic Resource
    Electronic Resource
    Contamination environnementale par les métaux et maladie de Parkinson (1990)
    Springer
    Water, air & soil pollution 49 (1990), S. 197-203 
    Details
    ISSN: 1573-2932
    Source: Springer Online Journal Archives 1860-2000
    Topics: Energy, Environment Protection, Nuclear Power Engineering
    Description / Table of Contents: Résumé La prévalence de la maladie de Parkinson au Québec était de 84/100000 en 1985. Des études ont démontré une fluctuation importante dans la distribution régionale et évoqué des liens à des facteurs environnementaux. Nous avons voulu vérifier l'existence de ces liens, plus spéifiquement avec 3 métaux: le fer, ;e manganèse et l'aluminium. L'estimation de l'exposition environnementale à ces trois métaux et le risque qui en découle, ont 'eté évalués grâce à l'épinette rouge utilisée comme bio-indicateur. Les concentrations de métaux dans les arbres ont permis d'estimer les risques temporles pour chaque ville du territoire étudié. Ces risques, combinés à l'historique de résidence, ont permis de comparer l'exposition des parkinsoniens à celle des non-parkinsoniens. Il en ressort que l'exposition environnementale de ces deux groupes n'est significativement différente.
    Notes: Abstract Parkinson's disease (PD) has been proposed to result from the interaction of aging and environment in susceptible individuals. The prevalence of PD in Quebec was 84 per 100 000 in 1985. The regional variation observed has been linked with several environmental factors. We intended to verify the existence of a relation between the development of PD with exposure to 3 metal pollutants: Fe, Mn, and Al. The choice of these metals rests on their potential presence in the environment caused by the industrial activity in the region studied, and on the fact that it theoretically favors the prevalence of the illness. Environmental exposure to these metals and the resulting risks were evaluated using red spruce as a bio-indicator. The metal concentrations in tree rings were determined by neutron activation analysis. These concentrations permitted a temporal estimation of contamination in each of the territories studied, from 1938 to 1987. The risk of environmental exposure was assumed to be proportional to the concentration of the metal. The calculated risks combined with the history of residence allowed a comparison between parkinsonians and controls. It appeared that environmental exposure to the metals was not significantly different between those two groups.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00279521
    Library Location Call Number Volume/Issue/Year Availability
    BibTip Others were also interested in ...
    Paper (German National Licenses)
    Fulltext
  • 10
    Electronic Resource
    Electronic Resource
    Obituary (1986)
    Springer
    Neurological sciences 7 (1986), S. 467-469 
    Details
    ISSN: 1590-3478
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF02283027
    Library Location Call Number Volume/Issue/Year Availability
    BibTip Others were also interested in ...
    Paper (German National Licenses)
    Fulltext
Close ⊗
This website uses cookies and the analysis tool Matomo. More information can be found here...