STURGE SYNDROME
\stˈɜːd͡ʒ sˈɪndɹə͡ʊm], \stˈɜːdʒ sˈɪndɹəʊm], \s_t_ˈɜː_dʒ s_ˈɪ_n_d_ɹ_əʊ_m]\
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A congenital syndrome characterized by a port-wine nevus covering portions of the face and cranium (in the distribution of the ophthalmic division of the TRIGEMINAL NERVE) and angiomas of the meninges and choroid. Clinical manifestations include the onset of focal SEIZURES, progressive hemiparesis, GLAUCOMA, hemianopsia, and cognitive deficits in the first decade of life. By age two years, skull radiographs reveal "tramline calcifications" of the margins of the occipital and parietal lobes. Pathologically cortical neurons are replaced by glial tissue that undergoes calcification. (From Adams et al., Principles of Neurology, 6th ed, pp1018-9)
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Mustagh Range
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