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. 1985 Nov;48(11):1097–1102. doi: 10.1136/jnnp.48.11.1097

A study of the relationship between neurological function and serum vitamin E concentrations in patients with cystic fibrosis.

H J Willison, D P Muller, S Matthews, S Jones, A Kriss, R J Stead, M E Hodson, A E Harding
PMCID: PMC1028567  PMID: 4078574

Abstract

A patient with cystic fibrosis and undetectable serum vitamin E concentrations is described who developed a progressive spinocerebellar syndrome and pigmentary retinopathy with abnormal somatosensory and visual evoked potentials (SSEPs and VEPs). In order to assess the relationship between neurological function and serum vitamin E concentrations in cystic fibrosis, 29 unselected patients who had no neurological symptoms were examined neurologically. Ten were randomly selected for neurophysiological assessment by recording SSEPs and VEPs. Electroretinograms (ERGs) were also performed in five cases. The findings were correlated with serum vitamin E concentrations which were unknown to the neurological investigators prior to completion of the study. Only one patient had definite reflex and sensory abnormalities, and the remaining 28 were clinically normal. The ERG was abnormal in two cases, one of whom had abnormal VEPs. SSEPs were normal in all 10 cases. Twenty six patients had serum vitamin E concentrations below the normal range. In two of the three patients who had definite neurological or electrophysiological abnormalities serum vitamin E concentrations were below the median value for the whole group.

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Selected References

These references are in PubMed. This may not be the complete list of references from this article.

  1. Alvarez F., Landrieu P., Laget P., Lemonnier F., Odièvre M., Alagille D. Nervous and ocular disorders in children with cholestasis and vitamin A and E deficiencies. Hepatology. 1983 May-Jun;3(3):410–414. doi: 10.1002/hep.1840030321. [DOI] [PubMed] [Google Scholar]
  2. Bieri J. G., Tolliver T. J., Catignani G. L. Simultaneous determination of alpha-tocopherol and retinol in plasma or red cells by high pressure liquid chromatography. Am J Clin Nutr. 1979 Oct;32(10):2143–2149. doi: 10.1093/ajcn/32.10.2143. [DOI] [PubMed] [Google Scholar]
  3. Bye A. M., Muller D. P., Wilson J., Wright V. M., Mearns M. B. Symptomatic vitamin E deficiency in cystic fibrosis. Arch Dis Child. 1985 Feb;60(2):162–164. doi: 10.1136/adc.60.2.162-a. [DOI] [PMC free article] [PubMed] [Google Scholar]
  4. Cavalier S. J., Gambetti P. Dystrophic axons and spinal cord demyelination in cystic fibrosis. Neurology. 1981 Jun;31(6):714–718. doi: 10.1212/wnl.31.6.714. [DOI] [PubMed] [Google Scholar]
  5. Elias E., Muller D. P., Scott J. Association of spinocerebellar disorders with cystic fibrosis or chronic childhood cholestasis and very low serum vitamin E. Lancet. 1981 Dec 12;2(8259):1319–1321. doi: 10.1016/s0140-6736(81)91342-8. [DOI] [PubMed] [Google Scholar]
  6. Farrell P. M., Levine S. L., Murphy M. D., Adams A. J. Plasma tocopherol levels and tocopherol-lipid relationships in a normal population of children as compared to healthy adults. Am J Clin Nutr. 1978 Oct;31(10):1720–1726. doi: 10.1093/ajcn/31.10.1720. [DOI] [PubMed] [Google Scholar]
  7. Geller A., Gilles F., Shwachman H. Degeneration of fasciculus gracilis in cystic fibrosis. Neurology. 1977 Feb;27(2):185–187. doi: 10.1212/wnl.27.2.185. [DOI] [PubMed] [Google Scholar]
  8. Gouras P., Carr R. E., Gunkel R. D. Retinitis pigmentosa in abetalipoproteinemia: Effects of vitamin A. Invest Ophthalmol. 1971 Oct;10(10):784–793. [PubMed] [Google Scholar]
  9. Guggenheim M. A., Ringel S. P., Silverman A., Grabert B. E. Progressive neuromuscular disease in children with chronic cholestasis and vitamin E deficiency: diagnosis and treatment with alpha tocopherol. J Pediatr. 1982 Jan;100(1):51–58. doi: 10.1016/s0022-3476(82)80234-5. [DOI] [PubMed] [Google Scholar]
  10. Harding A. E., Muller D. P., Thomas P. K., Willison H. J. Spinocerebellar degeneration secondary to chronic intestinal malabsorption: a vitamin E deficiency syndrome. Ann Neurol. 1982 Nov;12(5):419–424. doi: 10.1002/ana.410120503. [DOI] [PubMed] [Google Scholar]
  11. Harries J. T., Muller D. P. Absorption of vitamin E in children with biliary obstruction. Gut. 1971 Jul;12(7):579–584. doi: 10.1136/gut.12.7.579. [DOI] [PMC free article] [PubMed] [Google Scholar]
  12. Howard L., Ovesen L., Satya-Murti S., Chu R. Reversible neurological symptoms caused by vitamin E deficiency in a patient with short bowel syndrome. Am J Clin Nutr. 1982 Dec;36(6):1243–1249. doi: 10.1093/ajcn/36.6.1243. [DOI] [PubMed] [Google Scholar]
  13. Muller D. P., Harries J. T., Lloyd J. K. The relative importance of the factors involved in the absorption of vitamin E in children. Gut. 1974 Dec;15(12):966–971. doi: 10.1136/gut.15.12.966. [DOI] [PMC free article] [PubMed] [Google Scholar]
  14. Muller D. P., Lloyd J. K. Effect of large oral doses of vitamin E on the neurological sequelae of patients with abetalipoproteinemia. Ann N Y Acad Sci. 1982;393:133–144. doi: 10.1111/j.1749-6632.1982.tb31239.x. [DOI] [PubMed] [Google Scholar]
  15. Muller D. P., Lloyd J. K., Wolff O. H. Vitamin E and neurological function. Lancet. 1983 Jan 29;1(8318):225–228. doi: 10.1016/s0140-6736(83)92598-9. [DOI] [PubMed] [Google Scholar]
  16. Nelson J. S., Fitch C. D., Fischer V. W., Broun G. O., Jr, Chou A. C. Progressive neuropathologic lesions in vitamin E-deficient rhesus monkeys. J Neuropathol Exp Neurol. 1981 Mar;40(2):166–186. doi: 10.1097/00005072-198103000-00008. [DOI] [PubMed] [Google Scholar]
  17. Robison W. G., Jr, Kuwabara T., Bieri J. G. Deficiencies of vitamins E and A in the rat. Retinal damage and lipofuscin accumulation. Invest Ophthalmol Vis Sci. 1980 Sep;19(9):1030–1037. [PubMed] [Google Scholar]
  18. Rosenblum J. L., Keating J. P., Prensky A. L., Nelson J. S. A progressive neurologic syndrome in children with chronic liver disease. N Engl J Med. 1981 Feb 26;304(9):503–508. doi: 10.1056/NEJM198102263040902. [DOI] [PubMed] [Google Scholar]
  19. Shwachman H., Kowalski M., Khaw K. T. Cystic fibrosis: a new outlook. 70 patients above 25 years of age. Medicine (Baltimore) 1977 Mar;56(2):129–149. [PubMed] [Google Scholar]
  20. Sperling M. A., Hiles D. A., Kennerdell J. S. Electroretinographic responses following vitamin A therapy in A-beta-lipoproteinemia. Am J Ophthalmol. 1972 Mar;73(3):342–351. doi: 10.1016/0002-9394(72)90063-3. [DOI] [PubMed] [Google Scholar]
  21. Sung J. H., Park S. H., Mastri A. R., Warwick W. J. Axonal dystrophy in the gracile nucleus in congenital biliary atresia and cystic fibrosis (mucoviscidosis): beneficial effect of vitamin E therapy. J Neuropathol Exp Neurol. 1980 Sep;39(5):584–597. doi: 10.1097/00005072-198009000-00007. [DOI] [PubMed] [Google Scholar]
  22. WOLFF O. H., LLOYD J. K., TONKS E. L. A-BETA-LIPOPROTEINAEMIA WITH SPECIAL REFERENCE TO THE VISUAL DEFECT. Exp Eye Res. 1964 Dec;3:439–442. doi: 10.1016/s0014-4835(64)80055-5. [DOI] [PubMed] [Google Scholar]

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