J Korean Neurol Assoc.  2006 Jun;24(3):282-285.

Adrenoleukodystrophy Presenting as Secondary Erythrocytosis

Affiliations
  • 1Department of Neurology, Ajou University School of Medicine, Suwon, Korea. dacda@hanmail.net
  • 2Department of Hematology, Armed Forces Capital Hospital, Seongnam, Korea.

Abstract

A 22-year-old man, who had complained of dizziness and morning headaches, was admitted because of persistent polycythemia with elevated erythropoietin. Subsequently during the work-up, he had frequent sleep apneas without respiratory efforts and a brain MRI revealed multiple bilateral leukoencephalopathies without enhancement. Adrenoleukodystrophy was diagnosed by clinical manifestations including adrenal insufficiency, brain imaging, and an elevated very long chain fatty acids (VLCFA) level. We report a case of adolescent cerebral type of adrenoleukodystrophy presenting with central sleep apnea and secondary erythrocytosis.

Keyword

Sleep apnea; Adrenoleukodystrophy; Very long chain fatty acids

MeSH Terms

Adolescent
Adrenal Insufficiency
Adrenoleukodystrophy*
Brain
Dizziness
Erythropoietin
Fatty Acids
Headache
Humans
Leukoencephalopathies
Magnetic Resonance Imaging
Neuroimaging
Polycythemia*
Sleep Apnea Syndromes
Sleep Apnea, Central
Young Adult
Erythropoietin
Fatty Acids
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