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Subacute sclerosing panencephalitis

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329:"SSPE is suspected in young patients with dementia and neuromuscular irritability. EEG, CT or MRI, CSF examination, and measles serologic testing are done. EEG shows periodic complexes with high-voltage diphasic waves occurring synchronously throughout the recording. CT or MRI may show cortical atrophy or white matter lesions. CSF examination usually reveals normal pressure, cell count, and total protein content; however, CSF globulin is almost always elevated, constituting up to 20 to 60% of CSF protein. Serum and CSF contain elevated levels of measles virus antibodies. Anti-measles IgG appears to increase as the disease progresses. If test results are inconclusive, brain biopsy may be needed." 699:
Seizures may also occur. Some people may become blind. In advanced stages of the disease, individuals may lose the ability to walk, as their muscles stiffen or spasm. There is progressive deterioration to a comatose state, and then to a persistent vegetative state. Death is usually the result of fever, heart failure, or the brain's inability to continue controlling the autonomic nervous system.
203:. The condition primarily affects children, teens, and young adults. It has been estimated that about 2 in 10,000 people who get measles will eventually develop SSPE. However, a 2016 study estimated that the rate for unvaccinated infants under 15 months was as high as 1 in 609. No cure for SSPE exists, and the condition is almost always fatal. SSPE should not be confused with 48: 358:
In the classic presentation of the disease, death occurs in 1 to 3 years, but faster and slower progressions can occur. Faster deterioration in cases of acute fulminant SSPE leads to death within 3 months of diagnosis. Although the prognosis is bleak for SSPE past stage 1, there is a 5% spontaneous
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SSPE is a rare condition, although there is still relatively high incidence in Asia and the Middle East. However, the number of reported cases is declining since the introduction of the measles vaccine—eradication of the measles virus prevents the SSPE mutation and therefore the progression of the
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The initial symptoms of SSPE are subtle and include mild mental deterioration (such as memory loss) and changes in behavior (such as irritability) followed by disturbances in motor function, including uncontrollable involuntary jerking movements of the head, trunk or limbs called myoclonic jerks.
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infection, followed by an asymptomatic period that lasts 7 years on average but can range from 1 month to 27 years. After the asymptomatic period, progressive neurological deterioration occurs, characterized by behavior change, intellectual problems,
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remission rate—this may be either a full remission that may last many years or an improvement in condition giving a longer progression period or at least a longer period with the less severe symptoms.
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Gascon G, Yamanis S, Crowell J, et al. Combined oralisoprinosine-intraventricular alpha-interferon therapy for subacute sclerosing panencephalitis. Brain Dev. 1993; 15:346–55.
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Anlar B, Yalaz K, Oktem F; et al. (1997). "Long-term follow-up of patients with subacute sclerosing panencephalitis treated with intraventricular alpha-interferon".
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Stage 1: There may be personality changes, mood swings, or depression. Fever, headache, and memory loss may also be present. This stage may last up to 6 months.
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are not produced, and the virus is able to survive persistently without evoking an immune response. Eventually the infection will lead to SSPE.
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Risk WS, Haddad FS (1979). "The variable natural history of subacute sclerosing panencephalitis: a study of 118 cases from the Middle East".
719: 322: 390:"Subacute sclerosing panencephalitis: more cases of this fatal disease are prevented by measles immunization than was previously recognized" 2405: 1606: 204: 682: 1289: 268:, occurs in the final stage, during which breathing, heart rate, and blood pressure are affected. Death usually occurs as a result of 1093:
Santoshkumar B, Radhakrishnan K (1998). "Substantial spontaneous long-term remission in subacute sclerosing panencephalitis (SSPE)".
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Cianchetti C, Marrosu MG, Muntoni F; et al. (1998). "Intraventricularalpha-interferon in subacute sclerosing panencephalitis".
1897: 1221: 346:, but the response to these drugs varies from patient to patient, and the only accepted treatments are supportive measures such as 249:
Stage 3: Jerking movements are replaced by writhing (twisting) movements and rigidity. At this stage, complications may result in
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There is no cure available. If the diagnosis is made during stage 1 it may be possible to treat the disease with oral
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Wendorf, K, Winter, K, Harriman, K, Zipprich, J, Schechter, R, Hacker, J, Preas, C, Cherry JD, Glaser, C (2016).
683:"Subacute Sclerosing Panencephalitis Information Page | National Institute of Neurological Disorders and Stroke" 1901: 1813: 1718: 1548: 1521: 1493: 1426: 1300: 277: 1042:
Grunewald T, Lampe J, Weissbrich B; et al. (1998). "A 35-year old bricklayer with hemimyoclonic jerks".
1697: 1891: 1487: 2390: 2385: 2091: 2069: 1948: 1944: 1940: 1936: 1829: 1723: 1684: 737:"Defective translation of measles virus matrix protein in a subacute sclerosing panencephalitis cell line" 1351: 2076: 1657: 1527: 1079: 917: 866: 434:"Subacute Sclerosing Panencephalitis: The Devastating Measles Complication Is More Common Than We Think" 257: 1498: 2340: 2172: 1807: 1636: 748: 634: 310: 1758: 1479: 1278: 471: 1144:"Subacute sclerosing panencephalitis, a measles complication, in an internationally adopted child" 1861: 1617: 1118: 1067: 905: 854: 224: 76: 69: 1363: 246:
Stage 2: This stage may involve jerking, muscle spasms, seizures, loss of vision, and dementia.
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2 in 10,000 for all age groups; As high as 1 in 609 for unvaccinated infants under 15 months
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Bellini WJ, Rota JS, Lowe LE, Katz RS, Dyken PR, Zaki SR, Shieh WJ, Rota PA (2005).
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National Institute of Neurological Disorders and Stroke (NINDS) (27 March 2019).
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have restricted expression. As a result, infectious particles like the
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Intrathecal interferon alpha, intravenous ribavirin, isoprinosine
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Jafri, Sidra K; Kumar, Raman; Ibrahim, Shahnaz H (2018-06-26).
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SSPE is caused by the wild-type virus, not by vaccine strains.
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Carter, M. J.; Willcocks, M. M.; Ter Meulen, V. (1983).
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Campbell, H; Andrews, N; Brown, K E; Miller, E (2007).
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Jawetz. Melnick & Adelberg's Medical Microbiology
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Index

Dawson's encephalitis

Specialty
Neurology
Infectious Disease
Symptoms
seizures
spasticity
poor coordination
coma
Risk factors
Diagnostic method
Measles vaccine
Medication
Prognosis
brain inflammation
measles virus
acute disseminated encephalomyelitis
measles
myoclonic seizures
ataxia
blindness
loss of consciousness
persistent vegetative state
paralysis
fever
heart failure
autonomic nervous system
nucleocapsids
neurons

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