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Neurologic manifestations of Wilsons disease
What are the neurologic manifestations of Wilson’s disease?
Wilson’s disease is a rare disorder of copper metabolism resulting in accumulation of copper in the liver, kidneys, and central nervous system (CNS).
In almost half of patients, neurologic manifestations are present, including tremors, dysarthria, clumsiness, drooling, and gait instability in order of decreasing frequency.
Neuropsychiatric symptoms, including those of dementia, mania, depression, or psychosis, may dominate the presentation in up to 20% of patients.
Kaiser–Fleischer rings, copper deposits in Descemet’s membrane of the cornea, are present in 98% of patients with neurologic manifestations and are visualized by slit-lamp examination.
Neurologic manifestations invariably follow liver involvement, even in silent, unrecognized liver disease.
