[Prolonged remission in subacute sclerosing panencephalitis: 2 cases]. 1990

A Furby, and L Vallée, and M Rousseaux, and J P Nuyts, and A Destée
Service de Clinique Neurologique, C.H.U., Lille.

Long survival in subacute sclerosing panencephalitis (SSPE), including total disappearance of clinical signs, is rare. Two cases are reported. They concern a girl and a boy who, at age 13 and 15, developed SSPE and are still in remission 6 and 5 years later. After a typical onset and course over periods of 12 and 18 months, clinical improvement was observed and periodic EEG complexes disappeared. However, the electrophoretic oligoclonal pattern of CSF proteins and the elevated measles titers persisted (in one case specific CSF IgM were still increased 6 years after the onset). MRI showed asymmetrical areas of high-intensity signal in both white and gray matter, predominant in the temporal, parietal and occipital regions. The age at which SSPE begins and the interval between measles and SSPE onset are not prognostic factors. On the other hand, in reported cases with lasting remission SSPE did not progress beyond Jabbour's stage II. The second typical feature of these long-term improvements is disappearance of EEG periodic complexes and emergence of a normal basic background activity. No other prognostic factor has been reported.

UI MeSH Term Description Entries
D008297 Male Males
D012074 Remission Induction Therapeutic act or process that initiates a response to a complete or partial remission level. Induction of Remission,Induction, Remission,Inductions, Remission,Remission Inductions
D004569 Electroencephalography Recording of electric currents developed in the brain by means of electrodes applied to the scalp, to the surface of the brain, or placed within the substance of the brain. EEG,Electroencephalogram,Electroencephalograms
D005260 Female Females
D005500 Follow-Up Studies Studies in which individuals or populations are followed to assess the outcome of exposures, procedures, or effects of a characteristic, e.g., occurrence of disease. Followup Studies,Follow Up Studies,Follow-Up Study,Followup Study,Studies, Follow-Up,Studies, Followup,Study, Follow-Up,Study, Followup
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000293 Adolescent A person 13 to 18 years of age. Adolescence,Youth,Adolescents,Adolescents, Female,Adolescents, Male,Teenagers,Teens,Adolescent, Female,Adolescent, Male,Female Adolescent,Female Adolescents,Male Adolescent,Male Adolescents,Teen,Teenager,Youths
D013344 Subacute Sclerosing Panencephalitis 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) Encephalitis, Inclusion Body, Measles,Leukoencephalitis, Subacute Sclerosing,Measles Inclusion Body Encephalitis,Panencephalitis, Subacute Sclerosing,SSPE,Van Bogaert's Leukoencephalitis,Inclusion Body Encephalitis, Measles,Leukoencephalitis, Van Bogaert's,Sclerosing Leukoencephalitis, Subacute,Sclerosing Panencephalitis, Subacute,Leukoencephalitides, Subacute Sclerosing,Leukoencephalitis, Van Bogaert,Leukoencephalitis, Van Bogaerts,Panencephalitides, Subacute Sclerosing,Sclerosing Leukoencephalitides, Subacute,Sclerosing Panencephalitides, Subacute,Subacute Sclerosing Leukoencephalitides,Subacute Sclerosing Leukoencephalitis,Subacute Sclerosing Panencephalitides,Van Bogaert Leukoencephalitis,Van Bogaerts Leukoencephalitis
D013997 Time Factors Elements of limited time intervals, contributing to particular results or situations. Time Series,Factor, Time,Time Factor

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