A 14-year-old boy complains of a 4-month history of fatigue, abdominal pain, and yellowing of his eyes and skin. Physical examination shows tremor of both hands, lack of coordination, and mild jaundice. An ophthalmic examination reveals Kayser-Fleischer rings. Degenerative changes are present in which of the following anatomic regions of the CNS in this patient?
Wilson disease is an autosomal recessive condition in which excess copper may deposit in the liver and brain. Chronic hepatitis leads to cirrhosis in young people. Ocular lesions, so-called Kayser-Fleischer rings, represent deposition of copper in Descemet membrane in the iris. Lack of coordination and tremor (extrapyramidal neurologic symptoms) are related to degenerative changes in the corpus striatum. Deposition of copper is not observed in the other anatomic regions of the brain.Diagnosis: Wilson disease
Cerebellum
Corpus striatum
Paraventricular white matter
Pons
Central Nervous System