VAN BOGAERT LEUKOENCEPHALITIS
\vˈan bˈɒɡe͡ət lˌuːkə͡ʊnsˌɛfɐlˈa͡ɪtɪs], \vˈan bˈɒɡeət lˌuːkəʊnsˌɛfɐlˈaɪtɪs], \v_ˈa_n b_ˈɒ_ɡ_eə_t l_ˌuː_k_əʊ_n_s_ˌɛ_f_ɐ_l_ˈaɪ_t_ɪ_s]\
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A rare, slowly progressive encephalitis caused by chronic infection with the MEASLES VIRUS. The condition occurs primarily in children and young adults, approximately 2-8 years after the initial infection. A gradual decline in intellectual abilities and behavioral alterations are followed by progressive MYOCLONUS; MUSCLE SPASTICITY; SEIZURES; DEMENTIA; autonomic dysfunction; and ATAXIA. DEATH usually occurs 1-3 years after disease onset. Pathologic features include perivascular cuffing, eosinophilic cytoplasmic inclusions, neurophagia, and fibrous gliosis. It is caused by the SSPE virus, which is a defective variant of MEASLES VIRUS. (From Adams et al., Principles of Neurology, 6th ed, pp767-8)
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