Subacute sclerosing panencephalitis. 1978

M H Bellman, and G Dick

Subacute sclerosing panencephalitis (SSPE) is now considered to be caused by measles virus. There are four diagnostic criteria, namely the clinical picture, a characteristic EEG, serology of serum and CSF and brain histology. A register of cases in the U.K. has been kept since 1971, and up to September 1977, ninety-six patients have been reported. The male/female ratio is 2 : 1. The disease most commonly affects children between the ages of nine and eleven years who usually have had measles at a very early age. The average delay between the measles infection and onset of SSPE was 6.8 years and of the thirty-four patients known to have died the average survival times was 1.2 years. There are still many questions about the pathogenesis and epidemiology of SSPE that have yet to be answered.

UI MeSH Term Description Entries
D008297 Male Males
D008457 Measles A highly contagious infectious disease caused by MORBILLIVIRUS, common among children but also seen in the nonimmune of any age, in which the virus enters the respiratory tract via droplet nuclei and multiplies in the epithelial cells, spreading throughout the MONONUCLEAR PHAGOCYTE SYSTEM. Rubeola
D012042 Registries The systems and processes involved in the establishment, support, management, and operation of registers, e.g., disease registers. Parish Registers,Population Register,Parish Register,Population Registers,Register, Parish,Register, Population,Registers, Parish,Registers, Population,Registry
D002648 Child A person 6 to 12 years of age. An individual 2 to 5 years old is CHILD, PRESCHOOL. Children
D002675 Child, Preschool A child between the ages of 2 and 5. Children, Preschool,Preschool Child,Preschool Children
D005260 Female Females
D006113 United Kingdom Country in northwestern Europe including Great Britain and the northern one-sixth of the island of Ireland, located between the North Sea and north Atlantic Ocean. The capital is London. Great Britain,Isle of Man
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
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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