ENCEPHALOFACIAL HEMANGIOMATOSIS SYNDROMES
\ɛnsˈɛfɐlˌɒfe͡ɪʃə͡l hˌɛmɐŋɡɪˌɒmɐtˈə͡ʊsɪs sˈɪndɹə͡ʊmz], \ɛnsˈɛfɐlˌɒfeɪʃəl hˌɛmɐŋɡɪˌɒmɐtˈəʊsɪs sˈɪndɹəʊmz], \ɛ_n_s_ˈɛ_f_ɐ_l_ˌɒ_f_eɪ_ʃ_əl h_ˌɛ_m_ɐ_ŋ_ɡ_ɪ__ˌɒ_m_ɐ_t_ˈəʊ_s_ɪ_s s_ˈɪ_n_d_ɹ_əʊ_m_z]\
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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
- encephaloceles
- encephalochysis
- encephalocoel
- encephalocoele
- encephalofacial hemangiomatosis syndrome
- Encephalofacial Hemangiomatosis Syndromes
- encephalogram
- encephalography
- encephalohaemia
- encephaloid
- encephaloid cancer