U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

Subacute sclerosing panencephalitis(SSPE)

MedGen UID:
52527
•Concept ID:
C0038522
•
Disease or Syndrome
Synonym: SSPE
SNOMED CT: Measles inclusion body encephalitis (416154000); Immunosuppressive measles encephalitis (416154000); MIBE - Measles inclusion body encephalitis (416154000); Van Bogaert sclerosing leukoencephalitis (359686005); Subacute sclerosing panencephalitis (192685000); SSPE - Subacute sclerosing panencephalitis (192685000); Dawson's inclusion body encephalitis (192685000); Van Bogaert's sclerosing leukoencephalitis (359686005)
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
•Concept ID:
CN307044
•
Finding
Source: Orphanet
clinical entity without genetic inheritance.
See: This record
Not genetically inherited (Orphanet)
 
Monarch Initiative: MONDO:0009835
OMIM®: 260470
Orphanet: ORPHA2806

Definition

Subacute sclerosing panencephalitis (SSPE) is a fatal neurodegenerative disease caused by persistent central nervous system infection with the measles virus (summary by Torisu et al., 2004). [from OMIM]

Clinical features

From HPO
Infectious encephalitis
MedGen UID:
108917
•Concept ID:
C0596773
•
Disease or Syndrome
A disorder of the brain caused by an infectious agent that presents with fever, headache, and an altered level of consciousness. There may also be focal or multifocal neurologic deficits, and focal or generalized seizure activity.
See: Feature record | Search on this feature

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVSubacute sclerosing panencephalitis
Follow this link to review classifications for Subacute sclerosing panencephalitis in Orphanet.

Professional guidelines

PubMed

Virus and Immune-Mediated Encephalitides: Epidemiology, Diagnosis, Treatment, and Prevention.
Bale JF Jr
Pediatr Neurol 2015 Jul;53(1):3-12. Epub 2015 Mar 19 doi: 10.1016/j.pediatrneurol.2015.03.013. PMID: 25957806
Treatment of subacute sclerosing panencephalitis.
Gascon GG
J Child Neurol 1997 Nov;12(8):469-70. doi: 10.1177/088307389701200801. PMID: 9430310
Treatment of subacute sclerosing panencephalitis: an overview.
Taylor WJ, DuRant RH, Dyken PR
Drug Intell Clin Pharm 1984 May;18(5):375-81. doi: 10.1177/106002808401800503. PMID: 6202474

Recent clinical studies

Etiology

Measles Virus and Subacute Sclerosing Panencephalitis.
Krumova S, Andonova I, Stefanova R, Nenkova G, Genova-Kalou P
Clin Lab 2022 Sep 1;68(9) doi: 10.7754/Clin.Lab.2021.211147. PMID: 36125141
Subacute sclerosing panencephalitis.
Garg RK, Mahadevan A, Malhotra HS, Rizvi I, Kumar N, Uniyal R
Rev Med Virol 2019 Sep;29(5):e2058. Epub 2019 Jun 24 doi: 10.1002/rmv.2058. PMID: 31237061
Subacute sclerosing panencephalitis: clinical phenotype, epidemiology, and preventive interventions.
Mekki M, Eley B, Hardie D, Wilmshurst JM
Dev Med Child Neurol 2019 Oct;61(10):1139-1144. Epub 2019 Jan 25 doi: 10.1111/dmcn.14166. PMID: 30680706
Measles.
Rota PA, Moss WJ, Takeda M, de Swart RL, Thompson KM, Goodson JL
Nat Rev Dis Primers 2016 Jul 14;2:16049. doi: 10.1038/nrdp.2016.49. PMID: 27411684
Viral induced demyelination.
Stohlman SA, Hinton DR
Brain Pathol 2001 Jan;11(1):92-106. doi: 10.1111/j.1750-3639.2001.tb00384.x. PMID: 11145206Free PMC Article

Diagnosis

Subacute sclerosing panencephalitis.
Garg RK, Mahadevan A, Malhotra HS, Rizvi I, Kumar N, Uniyal R
Rev Med Virol 2019 Sep;29(5):e2058. Epub 2019 Jun 24 doi: 10.1002/rmv.2058. PMID: 31237061
Subacute sclerosing panencephalitis and chronic viral encephalitis.
Anlar B
Handb Clin Neurol 2013;112:1183-9. doi: 10.1016/B978-0-444-52910-7.00039-8. PMID: 23622327
Subacute sclerosing panencephalitis.
Garg RK
J Neurol 2008 Dec;255(12):1861-71. Epub 2008 Oct 14 doi: 10.1007/s00415-008-0032-6. PMID: 18846316
Subacute sclerosing panencephalitis.
Garg RK
Postgrad Med J 2002 Feb;78(916):63-70. doi: 10.1136/pmj.78.916.63. PMID: 11807185Free PMC Article
Subacute sclerosing panencephalitis.
Gascon GG
Semin Pediatr Neurol 1996 Dec;3(4):260-9. doi: 10.1016/s1071-9091(96)80030-1. PMID: 8969008

Therapy

Measles.
Hübschen JM, Gouandjika-Vasilache I, Dina J
Lancet 2022 Feb 12;399(10325):678-690. Epub 2022 Jan 28 doi: 10.1016/S0140-6736(21)02004-3. PMID: 35093206
Subacute sclerosing panencephalitis.
Garg RK, Mahadevan A, Malhotra HS, Rizvi I, Kumar N, Uniyal R
Rev Med Virol 2019 Sep;29(5):e2058. Epub 2019 Jun 24 doi: 10.1002/rmv.2058. PMID: 31237061
Subacute sclerosing panencephalitis.
Garg RK
J Neurol 2008 Dec;255(12):1861-71. Epub 2008 Oct 14 doi: 10.1007/s00415-008-0032-6. PMID: 18846316
Subacute sclerosing panencephalitis.
Garg RK
Postgrad Med J 2002 Feb;78(916):63-70. doi: 10.1136/pmj.78.916.63. PMID: 11807185Free PMC Article
Subacute sclerosing panencephalitis.
Br Med J 1979 Nov 3;2(6198):1096. PMID: 93011Free PMC Article

Prognosis

Infectious leukoencephalopathies.
Jakabek D, Chaganti J, Brew BJ
Handb Clin Neurol 2024;204:431-453. doi: 10.1016/B978-0-323-99209-1.00016-8. PMID: 39322393
Subacute sclerosing panencephalitis.
Garg RK, Mahadevan A, Malhotra HS, Rizvi I, Kumar N, Uniyal R
Rev Med Virol 2019 Sep;29(5):e2058. Epub 2019 Jun 24 doi: 10.1002/rmv.2058. PMID: 31237061
Measles.
Rota PA, Moss WJ, Takeda M, de Swart RL, Thompson KM, Goodson JL
Nat Rev Dis Primers 2016 Jul 14;2:16049. doi: 10.1038/nrdp.2016.49. PMID: 27411684
Subacute sclerosing panencephalitis and chronic viral encephalitis.
Anlar B
Handb Clin Neurol 2013;112:1183-9. doi: 10.1016/B978-0-444-52910-7.00039-8. PMID: 23622327
Subacute sclerosing panencephalitis.
Garg RK
Postgrad Med J 2002 Feb;78(916):63-70. doi: 10.1136/pmj.78.916.63. PMID: 11807185Free PMC Article

Clinical prediction guides

Movement Disorders in Patients with Subacute Sclerosing Panencephalitis: A Systematic Review.
Garg D, Patel S, Sankhla CS, Holla VV, Paramanandam V, Kukkle PL, Pandey S, Schneider SA, Pal PK
Mov Disord Clin Pract 2024 Jul;11(7):770-785. Epub 2024 May 15 doi: 10.1002/mdc3.14062. PMID: 38748762Free PMC Article
Immune-dysregulation in subacute sclerosing panencephalitis: An exploratory case-control study.
Vijayavarman V, Malhotra HS, Rizvi I, Kumar N, Pandey S, Jain M, Ali W, Suresh V, Garg RK, Jain A, Verma R, Sharma P, Uniyal R
J Med Virol 2023 Feb;95(2):e28504. doi: 10.1002/jmv.28504. PMID: 36655739
Aprepitant in the Treatment of Subacute Sclerosing Panencephalitis: A Randomized, Double-Blind, Placebo-Controlled Study.
Oncel I, Sancar M, Konuskan B, Arioz F, Tezcan S, Arman-Kandirmaz E, Parlak S, Gumeler E, Anlar B
Pediatr Neurol 2020 Sep;110:59-63. Epub 2020 Jun 1 doi: 10.1016/j.pediatrneurol.2020.05.009. PMID: 32718528
Subacute sclerosing panencephalitis.
Khwaja GA, Gupta M, Sharma DK
J Assoc Physicians India 1991 Dec;39(12):928-33. PMID: 1816221
Notes on the pathogenesis of subacute sclerosing panencephalitis.
Poser CM
J Neurol Sci 1990 Feb;95(2):219-24. doi: 10.1016/0022-510x(90)90244-h. PMID: 2182785

Recent systematic reviews

Movement Disorders in Patients with Subacute Sclerosing Panencephalitis: A Systematic Review.
Garg D, Patel S, Sankhla CS, Holla VV, Paramanandam V, Kukkle PL, Pandey S, Schneider SA, Pal PK
Mov Disord Clin Pract 2024 Jul;11(7):770-785. Epub 2024 May 15 doi: 10.1002/mdc3.14062. PMID: 38748762Free PMC Article
Vision Loss in Subacute Sclerosing Panencephalitis: A Systematic Review of Case Reports and Case Series.
Garg RK, Paliwal V, Kumar N, Agrawal AA, Agrawal P, Rizvi I
Am J Trop Med Hyg 2023 Jun 7;108(6):1272-1276. Epub 2023 May 9 doi: 10.4269/ajtmh.23-0004. PMID: 37160278Free PMC Article
Estimating national-level measles case-fatality ratios in low-income and middle-income countries: an updated systematic review and modelling study.
Sbarra AN, Mosser JF, Jit M, Ferrari M, Ramshaw RE, O'Connor P, Krause LK, Rogowski ELB, Portnoy A
Lancet Glob Health 2023 Apr;11(4):e516-e524. doi: 10.1016/S2214-109X(23)00043-8. PMID: 36925172Free PMC Article
Obstetric outcomes in pregnant women with subacute sclerosing panencephalitis (SSPE): a systematic review of case reports and case series.
Garg RK, Paliwal V, Rizvi I, Pandey S, Uniyal R, Agrawal S, Khanna R
Neurol Sci 2023 Jun;44(6):1959-1968. Epub 2023 Feb 2 doi: 10.1007/s10072-023-06650-x. PMID: 36729186
TREM2 Variants and Neurodegenerative Diseases: A Systematic Review and Meta-Analysis.
Zhou SL, Tan CC, Hou XH, Cao XP, Tan L, Yu JT
J Alzheimers Dis 2019;68(3):1171-1184. doi: 10.3233/JAD-181038. PMID: 30883352

Supplemental Content

Table of contents

    Clinical resources

    Practice guidelines

    • PubMed
      See practice and clinical guidelines in PubMed. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.
    • Bookshelf
      See practice and clinical guidelines in NCBI Bookshelf. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Reviews

    Recent activity

    Clear Turn Off Turn On

    Your browsing activity is empty.

    Activity recording is turned off.

    Turn recording back on

    See more...