Pediatric Neurology
Volume 30, Issue 1 , Pages 57-60, January 2004

Atypical childhood Wilson's disease

  • Martha D Carlson, MD, PhD

      Affiliations

    • Division of Pediatric Neurology, Departments of Pediatrics and Neurology, University of Michigan, Ann Arbor, Michigan, USA
    • Corresponding Author InformationCommunications should be addressed to:Dr. Carlson; Pediatric Neurology Metabolic Clinic; Box 0202/L3222; Women's Hospital; University of Michigan Health System; 1500 East Medical Center Drive; Ann Arbor, MI 48109-0202, USA.
    • ,
  • Majeed Al-Mateen, MD

      Affiliations

    • Mary Bridge Children's Hospital, Tacoma, Washington, USA
    • Department of Neurology, University of Washington, Seattle, Washington, USA
    • ,
  • George J Brewer, MD

      Affiliations

    • Departments of Human Genetics and Internal Medicine, University of Michigan, Ann Arbor, Michigan, USA

Received 25 March 2003; accepted 28 May 2003.

Abstract 

Wilson's disease is a genetic disorder of copper metabolism with a hepatic or neurologic presentation. A hepatic presentation is more common in young children. Neurologic Wilson's disease often manifests as a movement disorder with dystonia, tremor, and dysarthria. Psychiatric or behavioral symptoms can also be a presenting feature of Wilson's disease. We describe an atypical neurologic presentation in a prepubertal child with minimal hepatic involvement; in which transient hemiparesis and encephalopathy dominated her initial neurologic presentation. Brain magnetic resonance imaging revealed extensive cortical and subcortical signal change, in addition to the classical basal ganglia signal abnormality observed in Wilson's disease. She was treated with oral tetrathiomolybdate anticopper therapy, followed by zinc maintenance. Her clinical status and brain imaging improved considerably at 1 year after treatment initiation. Neurologic Wilson's disease may have diverse presentations, and should be considered in children who present with cortical features and signal change on magnetic resonance imaging.

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PII: S0887-8994(03)00422-3

doi:10.1016/S0887-8994(03)00422-3

Pediatric Neurology
Volume 30, Issue 1 , Pages 57-60, January 2004

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