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  • Ro 4-4602  (1)
  • early onset cerebellar ataxia with retained tendon reflexes  (1)
  • 1
    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
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  • 2
    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
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