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A family with adult-onset cerebral adrenoleucodystrophy

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Abstract

A family in which three members presented with cerebral adrenoleucodystrophy in adulthood is described. The implications on our understanding for the mechanisms controlling the phenotypic expression of this genetic disorder are discussed.

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References

  1. Antoku Y, Koike F, Ohtsuka Y, Sakai T, Tskamoto K, Nagara H, Iwashita H, Goto I (1991) Adrenoleukodystrophy: a correlation between saturated very long chain fatty acids in mononuclear cells and phenotype. Ann Neurol 30:101–103

    Google Scholar 

  2. Aubourg P, Adamsbaum C, Lavallard-Rousseau M-C, Rocchiccioli F, Cartier N, Jambaque I, Jakobezak C, Lemaitre A, Boureau F, Wolf C, Bougneres P-F (1993) A two-year trial of oleic and erucic acids (“Lorenzo's oil”) as treatment for adrenomyeloneuropathy. Engl J Med 329:745–752

    Google Scholar 

  3. Joshi M, Clark WE, Uphoff DF (1991) Adrenoneuropathy: characteristic gross findings and association with Hashimoto's thyroiditis and accelerated atherosclerosis. Mod Pathol 4:683–686

    Google Scholar 

  4. Moser HW, Naidu S, Kumar A, Rosenbaum AE (1987) The adrenoleukodystrophies. In: Nelson JS (ed) Critical reviews in neurobiology, vol 3, issue 1. CRC Press, Boca Raton, pp 29–88

    Google Scholar 

  5. Moser HW, Moser AB, Naidu S, Bergin A (1991) Clinical aspects of adrenoleukodystrophy and adrenomyeloneuropathy. Dev Neurosci 13:254–261

    Google Scholar 

  6. Moser HW, Moser AB, Smith KD, Bergin A, Borel J, Shankroff J, Stine OC, Merette C, Ott J, Krivit W (1992) Adrenoleukodystrophy: phenotypic variability and implications for therapy. J Inherit Metab Dis 15:645–664

    Google Scholar 

  7. Mosser J, Douar A-M, Sarde C-O, Kioschis P, Feil R, Moser H, Poustka A-M, Mandel J-L, Aubourg P (1983) Putative X-linked adrenoleukodystrophy gene shares unexpected homology with ABC transporters. Nature 361:726–730

    Google Scholar 

  8. Sadeghi-Nejad A, Senior B (1990) Adrenomyeloneuropathy presenting as Addison's disease in childhood. N Engl J Med 322:13–16

    Google Scholar 

  9. Uziel G, Bertini E, Bardelli P, Rimoldi M, Gambett M (1991) Experience on therapy of adrenoleukodystrophy and adrenomyeloneuropathy. Dev Neurosci 13:274–279

    Google Scholar 

  10. Weller M, Liedtke W, Petersen D, Opitz H, Poremba M (1992) Very-lateonset adrenoleukodystrophy: possible precipitation of demyelination by cerebral contusion. Neurology 42:367–370

    Google Scholar 

  11. Zwetsloot CP, Padberg GW, Seters AP van, Maaswinkel-Mooy PD, Onkenhout W (1992) Adult adrenoleukodystrophy: the clinical spectrum in a large Dutch family. J Neurol 239:107–111

    Google Scholar 

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Angus, B., de Silva, R., Davidson, R. et al. A family with adult-onset cerebral adrenoleucodystrophy. J Neurol 241, 497–499 (1994). https://doi.org/10.1007/BF00919712

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  • DOI: https://doi.org/10.1007/BF00919712

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