Chapter 50 - Sleep, neuroimaging, and polysomnography of Wilson’s disease

Summary

Involuntary movements dominate the clinical profile in patients with Wilson's disease (WD) but nearly one-third of these patients also have psychiatric and behavioral abnormalities. While sleep abnormalities have been reported in a wide variety of extrapyramidal disorders, they are expected and reported in patients with WD. Nevertheless further well-designed studies among patients with WD are important for a better understanding, and possible improvement with de-coppering therapy. Patients with WD have protean manifestations, magnetic resonance imaging (MRI) changes, multiple genetic mutations, and variable therapeutic response and course of illness, which might explain the variability. The imaging and pathological features of WD suggest that critical areas involved in the regulation of sleep are affected. There is no neuro anatomical correlation between polysomnography (PSG) parameters and MRI abnormalities. Recognition and treatment of sleep abnormalities in the WD patients may improve their quality of life.