Abstract
Abstract: Subacute Sclerosing Panencephalitis (SSPE) is a progressive neurodegenerative disease that attacks the central nervous system, especially in the population of children and early adolescents, due to persistent measles virus infection. The incidence of SSPE is quite rare, and data shows that in developing countries it is still quite high compared to developed countries. Diagnosis is based on clinical, supporting examinations such as EEG, as well as increased antibodies against measles virus in serum and cerebrospinal fluid. Symptoms can include changes in behavior, myoclonus, memory problems, and persistent pyramidal or extrapyramidal movements. Management to date has not provided satisfactory results and is individualized. Most SSPE patients experience a progressive and gradual course, leading to death within 1-3 years. The challenge of SSPE in children is the approach to diagnosis and management. Until now, the approach to treating SSPE in children is still based on the pathophysiological mechanisms from several existing research studies. That why therapy guidelines for children with SSPE are still varied. This paper aims to discuss the topic of SSPE in children with a major focus on diagnostic and therapeutic approaches based on the latest scientific evidence. Keywords: Subacute Sclerosing Panencephalitis (SSPE), children, diagnosis, therapy
Highlights
Subacute Sclerosing Panencephalitis (SSPE) is a progressive neurodegenerative disease that attacks the central nervous system, especially in the population of children and early adolescents due to persistent measles virus infection
The approach to treating SSPE in children is still based on the pathophysiological mechanisms from several existing research studies
This paper aims to discuss the topic of SSPE in children with a major focus on diagnostic and therapeutic approaches based on the latest scientific evidence
Summary
Subacute Sclerosing Panencephalitis (SSPE) is a progressive neurodegenerative disease that attacks the central nervous system, especially in the population of children and early adolescents due to persistent measles virus infection. Maternal antibodies to wild type measles virus persist until 9 months of age, measles virus infection at 1 year of age has a potential to alter its self expression Both intracellular infection and cytokine-mediated inflammatory response can triggers apoptosis programs leading to neuronal cell death, including oligodendrocyte cells in SSPE patients.[10] The latency period for measles virus infection usually lasts 7-10 years after measles infection, but this usually occurs between 1 month to 27 years time frame. The diagnostic criteria refer to clinical findings, EEG source imaging, cerebrospinal fluid examination, antibody levels against measles and brain biopsy (Table 1).[4]
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