SSPE
MCID: PNN005
MIFTS: 44

Panencephalitis, Subacute Sclerosing (SSPE)

Categories: Immune diseases, Infectious diseases, Mental diseases, Neuronal diseases, Rare diseases

Aliases & Classifications for Panencephalitis, Subacute Sclerosing

MalaCards integrated aliases for Panencephalitis, Subacute Sclerosing:

Name: Panencephalitis, Subacute Sclerosing 57
Subacute Sclerosing Panencephalitis 57 12 76 53 54 59 37 55 44 15 73
Sspe 57 76 53 59
Dawson Disease 53 54
Van Bogaert's Sclerosing Leukoencephalitis 12
Subacute Sclerosing Panencephalitis; Sspe 57
Subacute Sclerosing Leukoencephalopathy 12
Immunosuppressive Measles Encephalitis 12
Subacute Sclerosing Leukoencephalitis 59
Subacute Inclusion Body Encephalitis 59
Van Bogaert Encephalitis 59
Dawson's Encephalitis 59
Dawson Encephalitis 53
Van Bogaert Disease 59

Characteristics:

Orphanet epidemiological data:

59
subacute sclerosing leukoencephalitis
Inheritance: Not applicable; Prevalence: <1/1000000 (United States),1-9/100000 (India); Age of onset: All ages; Age of death: any age;

OMIM:

57
Inheritance:
autosomal recessive


HPO:

32
panencephalitis, subacute sclerosing:
Inheritance autosomal recessive inheritance


Classifications:

Orphanet: 59  
Rare neurological diseases


External Ids:

OMIM 57 260470
Disease Ontology 12 DOID:8970
ICD10 33 A81.1
ICD9CM 35 046.2
MeSH 44 D013344
NCIt 50 C85171
SNOMED-CT 68 84196008
Orphanet 59 ORPHA2806
UMLS via Orphanet 74 C0038522
ICD10 via Orphanet 34 A81.1
MedGen 42 C0038522
KEGG 37 H01696
SNOMED-CT via HPO 69 258211005 45170000
UMLS 73 C0038522

Summaries for Panencephalitis, Subacute Sclerosing

NINDS : 54 Subacute sclerosing panencephalitis (SSPE) is a progressive neurological disorder of children and young adults that affects the central nervous system (CNS). It is a slow, but persistent, viral infection caused by defective measles virus. SSPE has been reported from all parts of the world, but it is considered a rare disease in developed countries, with fewer than 10 cases per year reported in the United States. The incidence of SSPE declined by at least 90 percent in countries that have practiced widespread immunization with measles vaccine. The incidence of SSPE is still high in developing countries such as India and Eastern Europe. There is a higher incidence among males than females (male/female: 3/1). Most youngsters with SSPE have a history of measles infection at an early age, usually younger than 2 years, followed by a latent period of 6 to 8 years before neurological symptoms begin. Despite the long interval between the measles infection and the onset of SSPE, researchers think that the infection of the brain occurs soon after the primary bout with measles and progresses slowly. Why it persists and progresses still isn't clear. 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. 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.

MalaCards based summary : Panencephalitis, Subacute Sclerosing, also known as subacute sclerosing panencephalitis, is related to multiple sclerosis and acute disseminated encephalomyelitis. An important gene associated with Panencephalitis, Subacute Sclerosing is CD46 (CD46 Molecule). The drug Calcium Carbonate has been mentioned in the context of this disorder. Affiliated tissues include brain, brain and heart, and related phenotypes are encephalitis and vision/eye

Disease Ontology : 12 A viral infectious disease that results in infection located in brain, has material basis in Measles virus which is immune resistant (which can be a result of a mutation of the virus itself).

NIH Rare Diseases : 53 Subacute sclerosing panencephalitis (SSPE) a rare condition that is caused by a measles infection acquired earlier in life. Signs and symptoms of the condition primarily affect the central nervous system and often develop approximately 7 to 10 years after a person recovers from the measles. Affected people may initially experience behavioral changes, dementia, and disturbances in motor function. In the late stages of the disease, affected people often progress to a comatose state, and then to a persistent vegetative state. Ultimately, many people with SSPE succumb to fever, heart failure, or the brain's inability to continue controlling the autonomic nervous system. It is unclear why some people develop SSPE after they have seemingly recovered from the measles while others do not. Researchers suspect that SSPE may be due to an abnormal immune response or a mutant form of the measles virus that causes a persistent infection within the central nervous system. Treatment is supportive and primarily based on the signs and symptoms present in each person. Recent studies have shown that certain medications (called antiviral and immunomodulatory drugs) may slow the progression of the condition, although the best treatment regimen and their long-term effects in people with SSPE are currently unknown.

Wikipedia : 76 Subacute sclerosing panencephalitis (SSPE) is a rare and chronic form of progressive brain inflammation... more...

Description from OMIM: 260470

Related Diseases for Panencephalitis, Subacute Sclerosing

Diseases related to Panencephalitis, Subacute Sclerosing via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 93)
# Related Disease Score Top Affiliating Genes
1 multiple sclerosis 31.2 MX1 MOG MBP
2 acute disseminated encephalomyelitis 30.6 MOG MBP
3 optic neuritis 30.6 MOG MBP
4 neuritis 30.6 MOG MBP
5 nervous system disease 30.2 MOG MBP MAPT
6 central nervous system disease 30.2 MOG MBP MAPT
7 neuromyelitis optica 30.2 MOG MBP
8 viral encephalitis 30.1 MBP MOG MX1
9 peripheral nervous system disease 30.0 MYOM2 MOG MBP
10 viral infectious disease 10.7
11 chorioretinitis 10.5
12 retinitis 10.5
13 dementia 10.4
14 epilepsy 10.4
15 measles 10.4
16 cortical blindness 10.3
17 encephalopathy 10.3
18 creutzfeldt-jakob disease 10.3
19 schizophrenia 10.3
20 rubella 10.3
21 encephalitis 10.3
22 myoclonus 10.3
23 intracranial hypertension, idiopathic 10.2
24 mumps 10.2
25 diphtheria 10.2
26 aseptic meningitis 10.2
27 status epilepticus 10.2
28 dystonia 10.2
29 acquired immunodeficiency syndrome 10.2
30 progressive multifocal leukoencephalopathy 10.2
31 meningitis 10.2
32 toxoplasmosis 10.2
33 necrotizing fasciitis 10.1 MYOM2 CD46
34 allergic encephalomyelitis 10.1 MOG MBP
35 myelitis 10.1 MOG MBP
36 balo concentric sclerosis 10.1 MOG MBP
37 internuclear ophthalmoplegia 10.1 MOG MBP
38 alzheimer disease 10.1
39 amyotrophic lateral sclerosis 1 10.1
40 parkinson disease, late-onset 10.1
41 hydrocephalus due to congenital stenosis of aqueduct of sylvius 10.1
42 3-methylglutaconic aciduria, type iii 10.1
43 macular degeneration, age-related, 1 10.1
44 aceruloplasminemia 10.1
45 epilepsy, idiopathic generalized 10 10.1
46 cerebral palsy 10.1
47 thrombocytopenia 10.1
48 aphasia 10.1
49 hypoparathyroidism 10.1
50 listeriosis 10.1

Graphical network of the top 20 diseases related to Panencephalitis, Subacute Sclerosing:



Diseases related to Panencephalitis, Subacute Sclerosing

Symptoms & Phenotypes for Panencephalitis, Subacute Sclerosing

Symptoms via clinical synopsis from OMIM:

57
Neuro:
subacute sclerosing panencephalitis


Clinical features from OMIM:

260470

Human phenotypes related to Panencephalitis, Subacute Sclerosing:

32
# Description HPO Frequency HPO Source Accession
1 encephalitis 32 HP:0002383

MGI Mouse Phenotypes related to Panencephalitis, Subacute Sclerosing:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 vision/eye MP:0005391 9.02 CD46 MAPT MBP MOG OCA2

Drugs & Therapeutics for Panencephalitis, Subacute Sclerosing

Drugs for Panencephalitis, Subacute Sclerosing (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):


# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Calcium Carbonate Approved, Investigational Not Applicable 471-34-1

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 Pupil Dilation and Analgesia Nociception Index Completed NCT03280238 Not Applicable
2 Prevention of Serious Adverse Events in Acute Care Hospitals by Afferent Limb and Response Method Intervention - the ALARM Intervention Study. Completed NCT01949025 Not Applicable

Search NIH Clinical Center for Panencephalitis, Subacute Sclerosing

Cochrane evidence based reviews: subacute sclerosing panencephalitis

Genetic Tests for Panencephalitis, Subacute Sclerosing

Anatomical Context for Panencephalitis, Subacute Sclerosing

The Foundational Model of Anatomy Ontology organs/tissues related to Panencephalitis, Subacute Sclerosing:

19
Brain

MalaCards organs/tissues related to Panencephalitis, Subacute Sclerosing:

41
Brain, Heart, T Cells, Cortex, Eye, Thymus, Spinal Cord

Publications for Panencephalitis, Subacute Sclerosing

Articles related to Panencephalitis, Subacute Sclerosing:

(show top 50) (show all 1008)
# Title Authors Year
1
Unilateral macular chorioretinitis in subacute sclerosing panencephalitis studied by spectral domain optical coherence tomography. ( 29942191 )
2018
2
Subacute sclerosing panencephalitis and immune thrombocytopenia: More than a coincidence? ( 29407001 )
2018
3
Neuroleptic malignant syndrome as a presenting feature of subacute sclerosing panencephalitis. ( 29243130 )
2018
4
Analysis of a subacute sclerosing panencephalitis (SSPE) Genotype B3 virus from the 2009/10 South African measles epidemic shows hyperfusogenic F proteins contribute to measles virus infection in the brain. ( 30487282 )
2018
5
Genetic characterization of measles virus genotype D6 subacute sclerosing panencephalitis case, Alberta, Canada. ( 30291564 )
2018
6
Retinitis as the presenting feature of subacute sclerosing panencephalitis in an Indian male: A case report. ( 30249850 )
2018
7
Subacute sclerosing panencephalitis presenting as rapidly progressive dementia in a young adult. ( 28318730 )
2018
8
Pisa Syndrome in a Child With Subacute Sclerosing Panencephalitis. ( 29297046 )
2018
9
Primary Psychiatric Manifestations of Subacute Sclerosing Panencephalitis: A Case Report and Literature Review. ( 29402453 )
2018
10
Hemagglutinin-specific neutralization of subacute sclerosing panencephalitis viruses. ( 29466428 )
2018
11
Dystonic Storm: An Atypical Presentation of Subacute Sclerosing Panencephalitis. ( 29845970 )
2018
12
Fulminant subacute sclerosing panencephalitis: Not only a disease of the past. ( 29923642 )
2018
13
Subacute sclerosing panencephalitis - current perspectives. ( 29985487 )
2018
14
Teaching NeuroImages: Isolated pontine involvement in subacute sclerosing panencephalitis. ( 30012660 )
2018
15
Subacute sclerosing panencephalitis mortality, United States, 1979-2016: Vaccine-induced declines in SSPE deaths. ( 30057285 )
2018
16
IL28B, IL29 and micro-RNA 548 in subacute sclerosing panencephalitis as a rare disease. ( 30077763 )
2018
17
An Atypical Electroencephalographic Finding in a Child with Subacute Sclerosing Panencephalitis. ( 30090161 )
2018
18
Senile-Onset Subacute Sclerosing Panencephalitis, Presenting With Peculiar Findings. ( 30099908 )
2018
19
Maintaining Concentration of Ribavirin in Cerebrospinal Fluid by a New Dosage Method; Three Cases of Subacute Sclerosing Panencephalitis Treated Using a Subcutaneous Continuous Infusion Pump. ( 30153227 )
2018
20
Subacute Sclerosing Panencephalitis: A Disease Not to be Forgotten. ( 30218526 )
2018
21
A long-term subacute sclerosing panencephalitis survivor treated with intraventricular interferon-alpha for 13 years. ( 30304904 )
2018
22
Fulminant subacute sclerosing panencephalitis in an immunized 20-month-old Indian boy. ( 30351295 )
2018
23
Diffuse White Matter Involvement in Subacute Sclerosing Panencephalitis. ( 30453354 )
2018
24
A case of fulminant subacute sclerosing panencephalitis presenting with acute myoclonic-astatic epilepsy. ( 28617265 )
2017
25
Ophthalmic examination as a means to diagnose Subacute Sclerosing Panencephalitis: an optical coherence tomography and ultrawide field imaging evaluation. ( 28116334 )
2017
26
Chorioretinitis: a potential clue to the early diagnosis of subacute sclerosing panencephalitis. ( 28512208 )
2017
27
An unusual case of acute encephalitic syndrome: Is it acute measles encephalitis or subacute sclerosing panencephalitis? ( 29133711 )
2017
28
Subacute sclerosing panencephalitis should be eliminated by measles vaccination. ( 28750182 )
2017
29
Subacute Sclerosing Panencephalitis: The Devastating Measles Complication That Might Be More Common Than Previously Estimated. ( 28387784 )
2017
30
A Case of Early Onset Subacute Sclerosing Panencephalitis Presented as Juvenile Myoclonic Epilepsy. ( 29284817 )
2017
31
Fulminant Early Onset Subacute Sclerosing Panencephalitis. ( 27578309 )
2017
32
Subacute Sclerosing Panencephalitis. ( 28387848 )
2017
33
Early Longitudinal Spectral Domain Optical Coherence Tomography Findings in Subacute Sclerosing Panencephalitis. ( 28423386 )
2017
34
Subacute Sclerosing Panencephalitis in a Child Suffering from Human Immunodeficiency Virus on "Highly Active Antiretroviral Therapy" - Can This be Another Instance of Immune Reconstitution Inflammatory Syndrome? ( 28713662 )
2017
35
Subacute Sclerosing Panencephalitis Presenting with Isolated Positive Psychotic and Catatonic Symptoms. ( 28852257 )
2017
36
Marked Atrophic Changes of the Brain in a Patient with Subacute Sclerosing Panencephalitis. ( 29062617 )
2017
37
Subacute Sclerosing Panencephalitis of the Brainstem as a Clinical Entity. ( 29112137 )
2017
38
50 Years Ago in The Journal of Pediatrics: Subacute Sclerosing Panencephalitis. ( 29173295 )
2017
39
The behavior pattern of parents of patients with subacute sclerosing panencephalitis concerning alternative medicine. ( 29376574 )
2017
40
Subacute sclerosing panencephalitis in pregnancy. ( 26809815 )
2016
41
Increased insulin-like growth factor-1 levels in cerebrospinal fluid of advanced subacute sclerosing panencephalitis patients. ( 27061773 )
2016
42
Subacute Sclerosing Panencephalitis in a Child with Human Immunodeficiency Virus Co-Infection. ( 27777245 )
2016
43
A case of subacute sclerosing panencephalitis in a 23-year-old recent immigrant to Australia. ( 27078597 )
2016
44
Subacute sclerosing panencephalitis presenting as acute cerebellar ataxia and brain stem hyperintensities. ( 26968729 )
2016
45
BILATERAL SUBRETINAL FLUID AND RETINAL VASCULOPATHY ASSOCIATED WITH SUBACUTE SCLEROSING PANENCEPHALITIS. ( 27124791 )
2016
46
Notes from the Field: Subacute Sclerosing Panencephalitis Death - Oregon, 2015. ( 26765654 )
2016
47
Interleukin-12 (-1188) A/C and interferon-I^ (+874) A/T gene polymorphisms in subacute sclerosing panencephalitis patients. ( 27103395 )
2016
48
Atypical magnetic resonance imaging features in subacute sclerosing panencephalitis. ( 27293348 )
2016
49
A Novel Peptide Derived from the Fusion Protein Heptad Repeat Inhibits Replication of Subacute Sclerosing Panencephalitis Virus In Vitro and In Vivo. ( 27612283 )
2016
50
Subacute Sclerosing Panencephalitis: The Foothold in Undervaccination. ( 27634625 )
2016

Variations for Panencephalitis, Subacute Sclerosing

Expression for Panencephalitis, Subacute Sclerosing

Search GEO for disease gene expression data for Panencephalitis, Subacute Sclerosing.

Pathways for Panencephalitis, Subacute Sclerosing

GO Terms for Panencephalitis, Subacute Sclerosing

Biological processes related to Panencephalitis, Subacute Sclerosing according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 viral process GO:0016032 9.33 CD46 MOG NECTIN4
2 type I interferon signaling pathway GO:0060337 8.96 IRF1 MX1
3 viral entry into host cell GO:0046718 8.8 CD46 MOG NECTIN4

Molecular functions related to Panencephalitis, Subacute Sclerosing according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 virus receptor activity GO:0001618 8.8 CD46 MOG NECTIN4

Sources for Panencephalitis, Subacute Sclerosing

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 ExPASy
19 FMA
28 GO
29 GTR
30 HGMD
31 HMDB
32 HPO
33 ICD10
34 ICD10 via Orphanet
35 ICD9CM
36 IUPHAR
37 KEGG
38 LifeMap
40 LOVD
42 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
54 NINDS
55 Novoseek
57 OMIM
58 OMIM via Orphanet
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 SNOMED-CT via Orphanet
71 TGDB
72 Tocris
73 UMLS
74 UMLS via Orphanet
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