Literature DB >> 3984680

Antioxidant treatment in Spielmeyer-Sjögren's disease.

P Santavuori, T Westermarck, J Rapola, P Pohja, R Moren, M Lappi, U Vuonnala.   

Abstract

The data for 125 patients with Spielmeyer-Sjögren's disease is presented. Antioxidant therapy was given to 49. 27 received a combination of vitamin E, vitamin C, methionine and BHT. As the disease began to progress, the treatment was changed to a combination of sodium selenite and vitamin E in 14 of the 27 patients. The same therapy was also given to 22 children who had not received previous antioxidant supplementation. The number of positive and negative responses was nearly equal in the 2 treatment groups. However, the quality of the response was better in the selenite group and it has been possible in some cases to stop for several years, at least, the deterioration which began during the original therapy.

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Year:  1985        PMID: 3984680     DOI: 10.1111/j.1600-0404.1985.tb03178.x

Source DB:  PubMed          Journal:  Acta Neurol Scand        ISSN: 0001-6314            Impact factor:   3.209


  8 in total

1.  Clinical trials in rare disease: challenges and opportunities.

Authors:  Erika F Augustine; Heather R Adams; Jonathan W Mink
Journal:  J Child Neurol       Date:  2013-09       Impact factor: 1.987

2.  [18F]fluorodopa PET shows striatal dopaminergic dysfunction in juvenile neuronal ceroid lipofuscinosis.

Authors:  H M Ruottinen; J O Rinne; M Haaparanta; O Solin; J Bergman; V J Oikonen; I Järvelä; P Santavuori
Journal:  J Neurol Neurosurg Psychiatry       Date:  1997-06       Impact factor: 10.154

3.  Short-Term Administration of Mycophenolate Is Well-Tolerated in CLN3 Disease (Juvenile Neuronal Ceroid Lipofuscinosis).

Authors:  Erika F Augustine; Christopher A Beck; Heather R Adams; Sara Defendorf; Amy Vierhile; Derek Timm; Jill M Weimer; Jonathan W Mink; Frederick J Marshall
Journal:  JIMD Rep       Date:  2018-06-20

4.  Normal ascorbic acid in cerebrospinal fluid of patients with infantile neuronal ceroid-lipofuscinosis.

Authors:  J O Sass; D Skladal; M Brunner-Krainz
Journal:  Metab Brain Dis       Date:  1999-03       Impact factor: 3.584

5.  Spielmeyer-Vogt disease: clinical and neurophysiological aspects.

Authors:  L Piattella; C Cardinali; N Zamponi; O Papa
Journal:  Childs Nerv Syst       Date:  1991-08       Impact factor: 1.475

6.  Selenium in chronic neurologic diseases. Multiple sclerosis and Batten's disease.

Authors:  J Clausen; G E Jensen; S A Nielsen
Journal:  Biol Trace Elem Res       Date:  1988 Jan-Apr       Impact factor: 3.738

7.  Characterizing upper limb function in the context of activities of daily living in CLN3 disease.

Authors:  Hanna Hildenbrand; Jordan Wickstrom; Rebecca Parks; Cris Zampieri; Thuy-Tien Nguyen; Audrey Thurm; Kisha Jenkins; Katharine E Alter; Jesse Matsubara; Dylan Hammond; Ariane Soldatos; Forbes D Porter; An N Dang Do
Journal:  Am J Med Genet A       Date:  2021-02-08       Impact factor: 2.578

Review 8.  Application of Anticonvulsants, Antiepileptic Drugs, and Vitamin C in the Treatment and Analysis of Batten Disease.

Authors:  Shreya Reddy; Hetal Brahmbhatt
Journal:  Cureus       Date:  2022-01-30
  8 in total

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