PHAKOMATOSIS, STURGE WEBER
\fˌakəmɐtˈə͡ʊsɪs], \fˌakəmɐtˈəʊsɪs], \f_ˌa_k_ə_m_ɐ_t_ˈəʊ_s_ɪ_s]\
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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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Nearby Words
- phakitis
- phako-
- phakoidoscope
- phakomatoses
- phakomatosis
- Phakomatosis, Sturge Weber
- phakoscope
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- phalacrocorax
- phalacrocorax carbo
- phalacrosis