WESTPHAL STRUMPELL SYNDROME
\wˈɛstfə͡l stɹˈʌmpɛl sˈɪndɹə͡ʊm], \wˈɛstfəl stɹˈʌmpɛl sˈɪndɹəʊm], \w_ˈɛ_s_t_f_əl s_t_ɹ_ˈʌ_m_p_ɛ_l s_ˈɪ_n_d_ɹ_əʊ_m]\
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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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Quinones
- Hydrocarbon rings which contain two moieties position. They can be substituted in any position except at the ketone groups.