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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: Restricted Diffusion and Clinical Evolution.
Prasad C, Netravathi M, Kulanthaivelu K, Bhat MD, Pendharkar H
Neurol India 2022 Jan-Feb;70(1):275-280. doi: 10.4103/0028-3886.338737. PMID: 35263895
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

Diagnosis

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: Restricted Diffusion and Clinical Evolution.
Prasad C, Netravathi M, Kulanthaivelu K, Bhat MD, Pendharkar H
Neurol India 2022 Jan-Feb;70(1):275-280. doi: 10.4103/0028-3886.338737. PMID: 35263895
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
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

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
Electroencephalographic markers in dementia.
Malek N, Baker MR, Mann C, Greene J
Acta Neurol Scand 2017 Apr;135(4):388-393. Epub 2016 Jul 18 doi: 10.1111/ane.12638. PMID: 27430350
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.
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: Restricted Diffusion and Clinical Evolution.
Prasad C, Netravathi M, Kulanthaivelu K, Bhat MD, Pendharkar H
Neurol India 2022 Jan-Feb;70(1):275-280. doi: 10.4103/0028-3886.338737. PMID: 35263895
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.
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
Cortical activation and motor body representations in a patient with subacute sclerosing panencephalitis.
Tomasino B, Valente M, Del Negro I, De Colle MC, Guarracino I, Maieron M, Gigli GL
Neuropsychologia 2022 Aug 13;173:108299. Epub 2022 Jun 14 doi: 10.1016/j.neuropsychologia.2022.108299. PMID: 35714969
Spectrum of Movement Disorders Among Children With Subacute Sclerosing Panencephalitis: A Cross-Sectional Study.
Garg D, Kakkar V, Kumar A, Kapoor D, Abbey P, Pemde H, Mukherjee SB, Sharma S
J Child Neurol 2022 May;37(6):491-496. Epub 2022 Mar 9 doi: 10.1177/08830738221085158. PMID: 35262436
Subacute sclerosing panencephalitis.
Khwaja GA, Gupta M, Sharma DK
J Assoc Physicians India 1991 Dec;39(12):928-33. PMID: 1816221

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
Clinical features, pathogenesis, pathology, neuroimaging, clinical course and outcome of measles inclusion-body encephalitis: a systematic review of published case reports and case series.
Garg RK, Suresh V, Suvirya S, Rizvi I, Kumar N, Pandey S
Neurol Sci 2024 Jul;45(7):3069-3091. Epub 2024 Mar 21 doi: 10.1007/s10072-024-07480-1. PMID: 38512528
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
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

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