Relative adrenal insufficiency in adult-onset cerebral X-linked adrenoleukodystrophy
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A 48-year-old man with adrenoleukodystrophy was admitted because of heavy sweating, sputum, and high fever. Brain MRI showed extensive cerebral white matter demyelination (figure, A). The patient was previously diagnosed with adult-onset cerebral X-linked adrenoleukodystrophy (ACALD) with ABCD1 gene mutation at age 46 years. Laboratory findings on admission showed inflammatory changes (white blood cell count 12,400/μL, C-reactive protein 2.1 mg/dL), hypotonic hyponatremia (Na 118 mEq/L, Osm 255 mOsm), hyperkalemia (K 5.6 mEq/L), and hypoglycemia (75 mg/dL). The serum cortisol level was higher than normal (cortisol 35.2 μg/dL). Chest CT showed mild consolidation in the right lower lobe (figure, B).
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- Received June 23, 2016.
- Accepted September 7, 2016.
- © 2016 American Academy of Neurology
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