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Subacute sclerosing panencephalitis
Open accessAbstract
Subacute sclerosing panencephalitis (SSPE) is a progressive neurological disorder of childhood and early adolescence. It is caused by persistent defective measles virus. Brain biopsies or postmortem histopathological examination show evidence of astrogliosis, neuronal loss, degeneration of dendrites, demyelination, neurofibrillary tangles, and infiltration of inflammatory cells. Patients usually have behavioral changes, myoclonus, dementia, visual disturbances, and pyramidal and extrapyramidal signs. The disease has a gradual progressive course leading to death within 1-3 years. The diagnosis is based upon characteristic clinical manifestations, the presence of characteristic periodic EEG discharges, and demonstration of raised antibody titre against measles in the plasma and cerebrospinal fluid. Treatment for SSPE is still undetermined. A combination of oral isoprinosine (Inosiplex) and intraventricular interferon alfa appears to be the best effective treatment. Patients responding to treatment need to receive it life long. Effective immunisation against measles is the only solution presently available to the problem of this dreaded disease.
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Citations
Cited by
Schmahmann, J. D., & Pandya, D. N. (2006) · doi:10.1093/acprof:oso/9780195104233.001.0001 · Cited by 1,157
Neuroinflammation During RNA Viral Infections
Klein, R. S., Garber, C., Funk, K. E., Salimi, H., Soung, A., Kanmogne, M., Manivasagam, S., Agner, S., & Cain, M. (2019) · Annual Review of Immunology · doi:10.1146/annurev-immunol-042718-041417 · Cited by 160
Daroszewska, A., van 't Hof, R. J., Rojas, J. A., Layfield, R., Landao-Basonga, E., Rose, L., Rose, K., & Ralston, S. H. (2011) · Human Molecular Genetics · doi:10.1093/hmg/ddr172 · Cited by 131
Cundy, T. (2018) · Metabolism · doi:10.1016/j.metabol.2017.06.010 · Cited by 76
Measles Virus Spreads in Rat Hippocampal Neurons by Cell-to-Cell Contact and in a Polarized Fashion
Ehrengruber, M. U., Ehler, E., Billeter, M. A., & Naim, H. Y. (2002) · Journal of Virology · doi:10.1128/jvi.76.11.5720-5728.2002 · Cited by 73
Metadata
- Authors
- Garg, R. K.
- Venue
- Postgraduate Medical Journal
- Publisher
- Oxford University Press (OUP)
- Published
- 2002-02-01
- Volume
- 78
- Issue
- 916
- Pages
- 63-70
- ISSN
- 0032-5473 (ISSN-L)
- 1469-0756
- OpenAlex
- W2147619731
- Cited by
- 401
- References
- 0
- FWCI
- 12.60
- Type
- journal article
- Language
- en
- Open access
- bronze
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