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
367:
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
698:
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.
222:
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,
1274:
1259:
359:
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.
1962:
1209:
653:
825:
Gascon G, Yamanis S, Crowell J, et al. Combined oralisoprinosine-intraventricular alpha-interferon therapy for subacute sclerosing panencephalitis. Brain Dev. 1993; 15:346–55.
466:
829:
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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1085:
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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.
1378:
1196:
953:
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)".
2420:
1982:
1508:
1465:
880:
Cianchetti C, Marrosu MG, Muntoni F; et al. (1998). "Intraventricularalpha-interferon in subacute sclerosing panencephalitis".
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1221:
346:, but the response to these drugs varies from patient to patient, and the only accepted treatments are supportive measures such as
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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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432:
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"
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Grunewald T, Lampe J, Weissbrich B; et al. (1998). "A 35-year old bricklayer with hemimyoclonic jerks".
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737:"Defective translation of measles virus matrix protein in a subacute sclerosing panencephalitis cell line"
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434:"Subacute Sclerosing Panencephalitis: The Devastating Measles Complication Is More Common Than We Think"
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1144:"Subacute sclerosing panencephalitis, a measles complication, in an internationally adopted child"
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Stage 2: This stage may involve jerking, muscle spasms, seizures, loss of vision, and dementia.
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207:, which can also be caused by the measles virus, but has a very different timing and course.
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2010:
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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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909:
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Bellini WJ, Rota JS, Lowe LE, Katz RS, Dyken PR, Zaki SR, Shieh WJ, Rota PA (2005).
2316:
2166:
1844:
1705:
339:
290:
196:
667:
1305:
681:
National
Institute of Neurological Disorders and Stroke (NINDS) (27 March 2019).
2356:
2305:
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2213:
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1954:
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117:
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793:
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561:
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2005:
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494:
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1114:
1063:
1008:
901:
850:
778:
467:"New data shows a deadly measles complication is more common than thought"
2349:
1996:
1738:
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1346:
974:
893:
842:
611:
595:"Review of the effect of measles vaccination on the epidemiology of SSPE"
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552:
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have restricted expression. As a result, infectious particles like the
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1263:
1214:
760:
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90:
406:
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Intrathecal interferon alpha, intravenous ribavirin, isoprinosine
1294:
302:
94:
1367:
534:
Jafri, Sidra K; Kumar, Raman; Ibrahim, Shahnaz H (2018-06-26).
350:. Following onset of stage 2, the disease is invariably fatal.
210:
SSPE is caused by the wild-type virus, not by vaccine strains.
1588:
536:"Subacute sclerosing panencephalitis – current perspectives"
735:
Carter, M. J.; Willcocks, M. M.; Ter Meulen, V. (1983).
593:
Campbell, H; Andrews, N; Brown, K E; Miller, E (2007).
712:
Jawetz. Melnick & Adelberg's
Medical Microbiology
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32:
239:Symptoms progress through the following 4 stages:
800:, Treasure Island (FL): StatPearls Publishing,
264:, which may be preceded by or concomitant with
368:disease or even the initial infection itself.
218:SSPE is characterized by a history of primary
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8:
1084:: CS1 maint: multiple names: authors list (
922:: CS1 maint: multiple names: authors list (
871:: CS1 maint: multiple names: authors list (
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540:Pediatric Health, Medicine and Therapeutics
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1643:Progressive multifocal leukoencephalopathy
1613:
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276:, or the brain’s inability to control the
199:caused by a persistent infection with the
46:
29:
1514:Extranodal NK/T-cell lymphoma, nasal type
1167:
1016:
940:"SSPE information page www.ninds.nih.gov"
768:
610:
569:
551:
510:
449:
405:
493:Fisher DL, Defres S, Solomon T (2015).
377:
104:6-15 years after infection with measles
1142:Bonthius D, Stanek N, Grose C (2000).
1077:
915:
864:
792:Rocke, Zoe; Belyayeva, Mariya (2022),
1197:Merck Manual of Diagnosis and Therapy
993:"Subacute sclerosing panencephalitis"
794:"Subacute Sclerosing Panencephalitis"
599:International Journal of Epidemiology
7:
967:10.1001/archneur.1979.00500460044004
205:acute disseminated encephalomyelitis
132:EEG, Serologic testing, brain biopsy
55:Subacute sclerosing panencephalitis.
1664:Subacute sclerosing panencephalitis
1219:Subacute sclerosing panencephalitis
1205:Subacute Sclerosing Panencephalitis
185:Subacute sclerosing panencephalitis
33:Subacute sclerosing panencephalitis
394:The Journal of Infectious Diseases
25:
2411:Unsolved problems in neuroscience
1983:Severe acute respiratory syndrome
1509:Follicular dendritic cell sarcoma
342:(Inosiplex) and intraventricular
1969:Middle East respiratory syndrome
195:, is a rare form of progressive
1568:Adult T-cell leukemia/lymphoma
1554:Splenic marginal zone lymphoma
495:"Measles-induced encephalitis"
465:Sun, Lena (October 28, 2016).
438:Open Forum Infectious Diseases
1:
1107:10.1016/s0022-510x(97)00303-1
1056:10.1016/s0140-6736(98)04091-4
991:Garg, R K (1 February 2002).
1773:Tropical spastic paraparesis
1678:Lymphocytic choriomeningitis
997:Postgraduate Medical Journal
714:. Lange. 2010. p. 586.
2406:Neurodegenerative disorders
2136:Cytomegalovirus esophagitis
2056:Human parainfluenza viruses
1729:Ramsay Hunt syndrome type 2
301:. In these cells the viral
262:persistent vegetative state
2437:
18:Dawson's encephalitis
1800:Cytomegalovirus retinitis
54:
45:
2421:Rare infectious diseases
1902:Infectious mononucleosis
1719:Chandipura vesiculovirus
1549:Hepatocellular carcinoma
1494:Nasopharyngeal carcinoma
1427:Hepatocellular carcinoma
278:autonomic nervous system
231:, and eventually death.
1698:Encephalitis lethargica
1399:viral systemic diseases
2070:Human orthopneumovirus
1724:Herpesviral meningitis
1685:Arbovirus encephalitis
1160:10.3201/eid0604.000409
451:10.1093/ofid/ofw194.81
331:
1528:Merkel-cell carcinoma
1009:10.1136/pmj.78.916.63
327:
258:loss of consciousness
256:Stage 4: Progressive
235:Stages of Progression
2173:Adenovirus infection
1637:Human polyomavirus 2
1466:Oropharyngeal cancer
894:10.1212/wnl.50.1.315
843:10.1212/wnl.48.2.526
553:10.2147/PHMT.S126293
512:10.1093/qjmed/hcu113
293:are produced in the
150:Supportive treatment
2416:Slow virus diseases
1759:Post-polio syndrome
1395:Infectious diseases
753:1983Natur.305..153C
472:The Washington Post
2396:Viral encephalitis
1892:Epstein–Barr virus
1862:Respiratory system
1499:Burkitt's lymphoma
1327:External resources
1224:2014-08-08 at the
654:"merckmanuals.com"
612:10.1093/ije/dym207
289:A large number of
225:myoclonic seizures
214:Signs and symptoms
197:brain inflammation
81:Behavior changes,
70:Infectious Disease
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2017:Influenza virus A
1934:Human coronavirus
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1814:Herpes of the eye
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721:978-0-07-174271-9
400:(10): 1686–1693.
321:According to the
307:envelope proteins
191:), also known as
182:
181:
128:Diagnostic method
122:Measles infection
91:poor coordination
27:Medical condition
16:(Redirected from
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2099:
2094:digestive system
2011:Orthomyxoviridae
1977:SARS coronavirus
1963:MERS coronavirus
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1691:Orthomyxoviridae
1614:
1580:Immune disorders
1504:Hodgkin lymphoma
1480:Kaposi's sarcoma
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2193:Gastroenteritis
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2148:Gastroenteritis
2142:
2130:Cytomegalovirus
2093:
2082:
2050:Paramyxoviridae
2039:Avian influenza
1915:
1910:Cytomegalovirus
1874:viral pneumonia
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1869:nasopharyngitis
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1794:Cytomegalovirus
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1441:Cervical cancer
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1226:Wayback Machine
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747:(5930): 153–5.
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344:interferon alfa
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1830:Cardiovascular
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1461:Vaginal cancer
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961:(10): 610–14.
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727:
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635:"CDC pinkbook"
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2230:
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2119:
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2110:
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2104:
2100:
2097:
2095:
2089:
2079:
2078:
2074:
2072:
2071:
2067:
2063:
2062:Parainfluenza
2060:
2059:
2058:
2057:
2052:
2051:
2046:
2045:
2040:
2036:
2033:
2032:
2031:
2030:
2026:
2022:
2018:
2013:
2012:
2007:
2004:
2003:
1998:
1995:
1994:
1993:
1992:
1988:
1984:
1981:
1980:
1979:
1978:
1974:
1970:
1967:
1966:
1965:
1964:
1960:
1956:
1953:
1952:
1951:
1950:
1946:
1942:
1938:
1935:
1930:
1927:
1926:
1924:
1922:
1918:
1912:
1911:
1907:
1903:
1899:
1898:EBV infection
1896:
1895:
1894:
1893:
1889:
1888:
1886:
1884:
1880:
1877:
1875:
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1774:
1771:
1770:
1769:
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1764:
1760:
1757:
1755:
1754:Poliomyelitis
1752:
1751:
1750:
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1744:
1742:
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326:
324:
316:
314:
312:
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304:
300:
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292:
291:nucleocapsids
284:
279:
275:
274:heart failure
271:
267:
263:
259:
255:
252:
248:
245:
242:
241:
240:
234:
232:
230:
227:, blindness,
226:
221:
213:
211:
208:
206:
202:
201:measles virus
198:
194:
190:
186:
177:
173:
170:Usually fatal
169:
167:
163:
159:
157:
153:
149:
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142:
139:
135:
131:
129:
125:
121:
119:
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112:Measles virus
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67:
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62:
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2324:
2317:Pancreatitis
2299:
2287:
2275:
2263:
2256:
2237:
2212:
2205:
2198:
2186:
2165:
2128:
2114:
2075:
2068:
2054:
2048:
2015:
2009:
1989:
1975:
1961:
1932:
1908:
1890:
1845:Pericarditis
1837:
1806:
1792:
1765:
1746:
1704:
1689:
1670:
1663:
1656:
1635:
1618:Encephalitis
1587:
1560:
1541:
1520:
1486:
1472:
1433:
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1200:Home Edition
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1095:J Neurol Sci
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1080:cite journal
1047:
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1000:
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881:
867:cite journal
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470:
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397:
393:
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363:Epidemiology
357:
340:isoprinosine
337:
328:
323:Merck Manual
320:
305:that encode
288:
285:Pathogenesis
238:
217:
209:
192:
188:
184:
183:
118:Risk factors
2214:Coronavirus
1955:Common cold
1867:acute viral
1850:Myocarditis
1694:(probable)
1446:Anal cancer
1336:MedlinePlus
1101:(1): 83–8.
955:Arch Neurol
692:21 November
478:October 28,
299:glial cells
101:Usual onset
38:Other names
2380:Categories
2341:Urogenital
2207:Astrovirus
2167:Adenovirus
1991:SARS-CoV-2
1748:Poliovirus
1623:meningitis
1312:DiseasesDB
811:2023-03-10
798:StatPearls
372:References
156:Medication
137:Prevention
87:spasticity
2252:RNA virus
2233:DNA virus
2225:Hepatitis
2200:Norovirus
2188:Rotavirus
2182:RNA virus
2161:DNA virus
2107:Esophagus
2035:Influenza
1921:RNA virus
1883:DNA virus
1652:RNA virus
1631:DNA virus
1537:RNA virus
1415:DNA virus
1407:Oncovirus
1232:synd/1889
1072:206010725
882:Neurology
831:Neurology
562:1179-9927
546:: 67–71.
354:Prognosis
334:Treatment
317:Diagnosis
311:M protein
266:paralysis
253:or death.
251:blindness
175:Frequency
166:Prognosis
147:Treatment
66:Neurology
61:Specialty
2350:BK virus
2153:diarrhea
1997:COVID-19
1739:Myelitis
1347:Orphanet
1222:Archived
1217:Health:
1192:23-273l.
1178:10905971
1123:10635605
1027:11807185
910:33700234
859:20412574
806:32809508
621:18037676
580:29985487
521:24865261
416:16235165
297:and the
83:seizures
77:Symptoms
2401:Measles
2103:Pharynx
1607:Central
1306:D013344
1169:2640885
1115:9543327
1064:9654261
1018:1742261
902:9443512
851:9040751
779:6888557
770:7094927
749:Bibcode
571:6027681
295:neurons
260:into a
220:measles
1767:HTLV-I
1712:Rabies
1562:HTLV-I
1341:001419
1295:260470
1215:PubMed
1176:
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1044:Lancet
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229:ataxia
109:Causes
2363:Mumps
2122:Mumps
2092:Human
1522:MCPyV
1317:12597
1284:046.2
1269:A81.1
1210:NINDS
1119:S2CID
1068:S2CID
906:S2CID
855:S2CID
303:genes
270:fever
2077:hMPV
1949:OC43
1945:HKU1
1941:NL63
1937:229E
1595:AIDS
1474:KSHV
1352:2806
1301:MeSH
1290:OMIM
1279:9-CM
1174:PMID
1111:PMID
1086:link
1060:PMID
1023:PMID
971:PMID
924:link
898:PMID
873:link
847:PMID
802:PMID
775:PMID
716:ISBN
694:2021
617:PMID
576:PMID
558:ISSN
517:PMID
480:2016
412:PMID
189:SSPE
95:coma
2357:MuV
2326:CBV
2301:HEV
2289:HDV
2277:HCV
2265:HAV
2258:CBV
2239:HBV
2116:MuV
2047:V,
1839:CBV
1808:HSV
1785:Eye
1672:LCV
1658:MeV
1589:HIV
1543:HCV
1488:EBV
1435:HPV
1421:HBV
1275:ICD
1260:ICD
1235:at
1208:at
1194:at
1164:PMC
1156:doi
1103:doi
1099:154
1052:doi
1048:351
1013:PMC
1005:doi
963:doi
890:doi
839:doi
765:PMC
757:doi
745:305
607:doi
566:PMC
548:doi
507:doi
503:108
499:QJM
446:doi
402:doi
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1706:RV
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