Wilson's Disease in an Early Adolescent: Classic Magnetic Resonance Imaging Findings

Cureus. 2024 Apr 12;16(4):e58092. doi: 10.7759/cureus.58092. eCollection 2024 Apr.

Abstract

Wilson's disease (WD), alternatively termed hepatolenticular degeneration, represents a rare autosomal recessive disorder typified by disrupted copper metabolism, culminating in copper accumulation across various organs. WD commonly manifests with early-onset liver cirrhosis, with notable involvement of the central nervous system, particularly impacting the midbrain and basal ganglia. This case report delineates the clinical presentation of an early adolescent female with WD, accentuating classical magnetic resonance imaging (MRI) findings. These MRI findings, which include the "face of a giant panda sign" and the "Face of a miniature panda sign," are pivotal for expeditious diagnosis. Recognition of these classical signs underscores the indispensable role of MRI in elucidating the neurological dimensions of WD.

Keywords: basal ganglia; copper; hepatolenticular degeneration; liver cirrhosis; magnetic resonance imaging.

Publication types

  • Case Reports