The Egyptian Journal of Radiology and Nuclear Medicine (Aug 2024)
Case series on neuroimaging spectrum of Wilson’s disease: knowing the known and the uncommonly known
Abstract
Abstract Background Wilson’s disease is an inherited disease characterized by impaired copper metabolism that causes damage to many organs, including the brain. Patients having neurological involvement usually present with varied neuropsychiatric symptoms. Magnetic resonance imaging (MRI) Brain plays an indispensable role in identifying the structural involvement in these patients, aiding in early accurate diagnosis and timely management. Typically, basal ganglia, thalami and brainstem are involved, with bright claustrum sign, face of giant panda sign and miniature panda signs on MRI. Conclusions Having knowledge about the commonly encountered and known MRI brain findings in Wilson’s disease are essential in aiding accurate diagnosis and initiating early management. However, identifying the Atypical MRI brain characteristics is all the more imperative and should be considered in patients with prolonged or severe disease or in patients with rapid clinical progression and in patients showing poor response to treatment.
Keywords