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Adrenoleukodystrophy: heterogeneity in two brothers.
  1. G M Elrington,
  2. D E Bateman,
  3. M J Jeffrey,
  4. N F Lawton
  1. Wessex Neurological Centre, Southampton, UK.


    A man with hypoadrenalism died from a rapidly progressive pseudobulbar palsy, due to adult onset adrenoleukodystrophy. This diagnosis suggested that his brother, with a longstanding spastic paraparesis, suffered from adrenomyeloneuropathy. Both cases were confirmed biochemically. The heterogeneity of expression of this x-linked disorder is described, with the consequent difficulty in diagnosis and nomenclature.

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