WESTPHAL STRUMPELL SYNDROMES
\wˈɛstfə͡l stɹˈʌmpɛl sˈɪndɹə͡ʊmz], \wˈɛstfəl stɹˈʌmpɛl sˈɪndɹəʊmz], \w_ˈɛ_s_t_f_əl s_t_ɹ_ˈʌ_m_p_ɛ_l s_ˈɪ_n_d_ɹ_əʊ_m_z]\
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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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Word of the day
tinctura quininae ammoniata
- A preparation made by dissolving quinin sulphate in alcohol [Br. Ph.].