HEPATOCEREBRAL DEGENERATION
\hˈɛpɐtˌə͡ʊsɹɛbɹə͡l dɪd͡ʒˌɛnəɹˈe͡ɪʃən], \hˈɛpɐtˌəʊsɹɛbɹəl dɪdʒˌɛnəɹˈeɪʃən], \h_ˈɛ_p_ɐ_t_ˌəʊ_s_ɹ_ɛ_b_ɹ_əl d_ɪ_dʒ_ˌɛ_n_ə_ɹ_ˈeɪ_ʃ_ə_n]\
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A rare autosomal recessive disease characterized by the deposition of copper in the BRAIN; LIVER; CORNEA; and other organs. Clinical features include LIVER CIRRHOSIS; LIVER FAILURE; SPLENOMEGALY; TREMOR; bradykinesia; DYSARTHRIA; CHOREA; MUSCLE RIGIDITY; Kayser-Fleischer rings (pigmented corneal lesions); ATAXIA; and intellectual deterioration. Hepatic dysfunction may precede neurologic dysfunction by several years. (From Adams et al., Principles of Neurology, 6th ed pp969-71)
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