Subacute sclerosing panencephalitis

Subacute sclerosing panencephalitis (SSPE) is a subacute encephalopathy of childhood and young adolescence. Infrequently, SSPE can occur in adults and pregnant women. It is caused by an aberrant measles virus, known as the SSPE virus. SSPE virus differs from wild-type measles viruses in the form of...

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Bibliographic Details
Published in:Journal of neurology 2008-12, Vol.255 (12), p.1861-1871
Main Author: Garg, Ravindra Kumar
Format: Article
Language:English
Subjects:
Age
Age Factors
Biological and medical sciences
Blindness, Cortical - complications
Blindness, Cortical - diagnosis
Blindness, Cortical - therapy
Epidemiology
Families & family life
Genes
Genomes
Genotype & phenotype
Human viral diseases
Humans
Industrialized nations
Infections
Infectious diseases
Inosine Pranobex - administration & dosage
Inosine Pranobex - therapeutic use
Measles
Measles Vaccine - administration & dosage
Measles Vaccine - therapeutic use
Measles virus
Measles virus - drug effects
Medical sciences
Medicine
Medicine & Public Health
Mutation
Nervous system
Neurology
Neuroradiology
Neurosciences
Original Communication
Pathogenesis
Phylogenetics
Proteins
Ribonucleic acid
RNA
RNA polymerase
Subacute Sclerosing Panencephalitis - complications
Subacute Sclerosing Panencephalitis - diagnosis
Subacute Sclerosing Panencephalitis - therapy
Vaccines
Viral diseases
Viral diseases of the nervous system
Viruses
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Description
Summary:Subacute sclerosing panencephalitis (SSPE) is a subacute encephalopathy of childhood and young adolescence. Infrequently, SSPE can occur in adults and pregnant women. It is caused by an aberrant measles virus, known as the SSPE virus. SSPE virus differs from wild-type measles viruses in the form of several mutations affecting the viral genome. The matrix gene is most commonly affected by these mutations. The characteristic clinical manifestations of SSPE include behavioral changes, cognitive decline, myoclonic jerks, seizures, abnormalities in vision, bilateral pyramidal signs and coma. Ocular changes may occur in up to 50 % of patients. The most characteristic ophthalmological lesion is necrotizing retinitis. Cortical blindness can be the early feature of SSPE. The diagnosis of SSPE is often difficult in the early stages. In a typical case diagnosis is based on clinical, electroencephalographic, and cerebrospinal fluid findings. At present, there is no effective treatment to completely cure SSPE. Oral isoprinosine and intrathecal or intraventricular alpha-interferon may prolong survival to some extent. Immunization against measles is currently the most effective strategy against SSPE.
ISSN:0340-5354
1432-1459
DOI:10.1007/s00415-008-0032-6