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Abstract
Objective: To study the frequency of additional cerebral demyelination in Dutch patients with adrenomyeloneuropathy (AMN).
Methods: Consecutive patients with AMN from the Dutch X-linked adrenoleukodystrophy cohort without cerebral demyelination on MRI at inclusion, seen between January 1, 1992, and January 1, 1999, were included. Primary endpoints were brain involvement, death, or the end of follow-up on January 1, 2011. Three levels of certainty were used for cerebral demyelination: (1) signs and symptoms reported by relatives and treating physicians, confirmed by brain MRI; if follow-up MRIs were not available, (2) based upon information from treating physicians and relatives, and (3) based upon information obtained from relatives only. Results were compared with a study published in 2001, in which 13/68 (19.1%) patients with AMN developed cerebral demyelination in 9.5 ± 5.5 years. Differences of the proportions of patients with cerebral demyelination and their 95% confidence intervals (CIs) were calculated.
Results: Of 27 patients with AMN, 17 (63%) developed cerebral demyelination 10.2 ± 6.9 years after onset of myelo(neuro)pathy. Mean survival was 3.4 ± 2.9 years. Brain involvement was higher in Dutch patients with AMN (difference 44%, 95% CI 0.23–0.64).
Conclusions: Cerebral demyelination in AMN may be more frequent than previously reported. Survival is as poor as in childhood cerebral adrenoleukodystrophy. Therapies that can halt cerebral demyelination in these patients are needed.
GLOSSARY
- ALD=
- ATP-binding cassette, subfamily D;
- AMN=
- adrenomyeloneuropathy;
- CCALD=
- childhood cerebral adrenoleukodystrophy;
- CI=
- confidence interval;
- FLAIR=
- fluid-attenuated inversion recovery;
- VLCFA=
- very long-chain fatty acids;
- X-ALD=
- X-linked adrenoleukodystrophy
Footnotes
Go to Neurology.org for full disclosures. Funding information and disclosures deemed relevant by the authors, if any, are provided at the end of the article.
- Received April 23, 2014.
- Accepted in final form August 11, 2014.
- © 2014 American Academy of Neurology
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