MCID: PNN005
MIFTS: 43

Panencephalitis, Subacute Sclerosing

Categories: Rare diseases, Infectious diseases, Neuronal diseases, Immune 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
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 allergic encephalomyelitis and balo concentric sclerosis. 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

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, Endothelial, Thymus

Publications for Panencephalitis, Subacute Sclerosing

Articles related to Panencephalitis, Subacute Sclerosing:

(show top 50) (show all 587)
# 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
A case of fulminant subacute sclerosing panencephalitis presenting with acute myoclonic-astatic epilepsy. ( 28617265 )
2017
5
Ophthalmic examination as a means to diagnose Subacute Sclerosing Panencephalitis: an optical coherence tomography and ultrawide field imaging evaluation. ( 28116334 )
2017
6
Chorioretinitis: a potential clue to the early diagnosis of subacute sclerosing panencephalitis. ( 28512208 )
2017
7
Subacute sclerosing panencephalitis in pregnancy. ( 26809815 )
2016
8
Increased insulin-like growth factor-1 levels in cerebrospinal fluid of advanced subacute sclerosing panencephalitis patients. ( 27061773 )
2016
9
Subacute Sclerosing Panencephalitis in a Child with Human Immunodeficiency Virus Co-Infection. ( 27777245 )
2016
10
A case of subacute sclerosing panencephalitis in a 23-year-old recent immigrant to Australia. ( 27078597 )
2016
11
Subacute sclerosing panencephalitis presenting as acute cerebellar ataxia and brain stem hyperintensities. ( 26968729 )
2016
12
BILATERAL SUBRETINAL FLUID AND RETINAL VASCULOPATHY ASSOCIATED WITH SUBACUTE SCLEROSING PANENCEPHALITIS. ( 27124791 )
2016
13
Notes from the Field: Subacute Sclerosing Panencephalitis Death - Oregon, 2015. ( 26765654 )
2016
14
Interleukin-12 (-1188) A/C and interferon-I^ (+874) A/T gene polymorphisms in subacute sclerosing panencephalitis patients. ( 27103395 )
2016
15
Diffusion restriction in fulminant subacute sclerosing panencephalitis: Report of an unusual finding. ( 26053831 )
2015
16
Subacute sclerosing panencephalitis in a child with recurrent febrile seizures. ( 25802788 )
2015
17
Subacute sclerosing panencephalitis in a child with human immunodeficiency virus (HIV) infection on antiretroviral therapy. ( 25745323 )
2015
18
Amino acid substitutions in the heptad repeat A and C regions of the F protein responsible for neurovirulence of measles virus Osaka-1 strain from a patient with subacute sclerosing panencephalitis. ( 26524513 )
2015
19
Mania as a presenting clinical feature of subacute sclerosing panencephalitis. ( 25840934 )
2015
20
Stem cells and their potential therapeutic use in subacute sclerosing panencephalitis. ( 25857704 )
2015
21
MRI and MR spectroscopy findings of a case of subacute sclerosing panencephalitis affecting the corpus callosum. ( 26163552 )
2015
22
Subacute sclerosing panencephalitis presenting with hypersexual behavior. ( 26600593 )
2015
23
A Multinational Survey on Actual Diagnostics and Treatment of Subacute Sclerosing Panencephalitis. ( 26479761 )
2015
24
Subacute sclerosing panencephalitis with parkinsonian features in a child: A case report. ( 25736973 )
2015
25
A 29-year-old pregnant woman with worsening left hemiparesis, encephalopathy, and hemodynamic instability: a case report of subacute sclerosing panencephalitis. ( 25943270 )
2015
26
Subacute sclerosing panencephalitis resembling Rasmussen's encephalitis on magnetic resonance imaging. ( 26451061 )
2015
27
Mesenchymal stem cell application in children with subacute sclerosing panencephalitis. ( 25846604 )
2015
28
Adult-onset subacute sclerosing panencephalitis manifesting as slowly progressive dementia. ( 25851779 )
2015
29
Subacute sclerosing panencephalitis masquerading as rapid-onset dystonia-Parkinsonism in a child. ( 25751483 )
2015
30
The phosphoprotein genes of measles viruses from subacute sclerosing panencephalitis cases encode functional as well as non-functional proteins and display reduced editing. ( 26428304 )
2015
31
Measles Virus: Identification in the M Protein Primary Sequence of a Potential Molecular Marker for Subacute Sclerosing Panencephalitis. ( 26587021 )
2015
32
Subacute sclerosing panencephalitis in South African children following the measles outbreak between 2009 and 2011. ( 26428963 )
2015
33
Optic neuritis and rapidly progressive necrotizing retinitis as the initial signs of subacute sclerosing panencephalitis: a case report with clinical and histopathologic findings. ( 24522882 )
2014
34
Neuronal loss is an early component of subacute sclerosing panencephalitis. ( 25085642 )
2014
35
Expression Patterns of Micro-RNAs 146a, 181a, and 155 in Subacute Sclerosing Panencephalitis. ( 24789113 )
2014
36
May TLR4 Asp299Gly and IL17 His161Arg polymorphism be associated with progression of primary measles infection to subacute sclerosing panencephalitis? ( 24690400 )
2014
37
Isolated vision loss and disappearing lesions as the manifestation of subacute sclerosing panencephalitis. ( 25506180 )
2014
38
Fulminant subacute sclerosing panencephalitis presenting with acute ataxia and hemiparesis in a 15-year-old boy. ( 25324886 )
2014
39
Subacute sclerosing panencephalitis: clinical and demographic characteristics. ( 25149833 )
2014
40
Granzyme B gene polymorphism associated with subacute sclerosing panencephalitis. ( 24875585 )
2014
41
Adult onset subacute sclerosing panencephalitis - Lessons learnt from an atypical presentation. ( 25002787 )
2014
42
Catatonia as presenting clinical feature of subacute sclerosing panencephalitis. ( 24891908 )
2014
43
Blindness, Dancing Extremities, and Corpus Callosum and Brain Stem Involvement: An Unusual Presentation of Fulminant Subacute Sclerosing Panencephalitis. ( 24700664 )
2014
44
A Decrease of Regulatory T Cells and Altered Expression of NK Receptors Are Observed in Subacute Sclerosing Panencephalitis. ( 25379970 )
2014
45
Apoptosis in subacute sclerosing panencephalitis: possibility for treatment. ( 23399111 )
2013
46
Subacute Sclerosing Panencephalitis Presenting as Schizophrenia With an Alpha Coma Pattern in a Child. ( 24141274 )
2013
47
Role of carbamazepine in the symptomatic treatment of subacute sclerosing panencephalitis: a case report and review of the literature. ( 23533854 )
2013
48
Epidemiology of subacute sclerosing panencephalitis (SSPE) in Germany from 2003 to 2009: a risk estimation. ( 23874807 )
2013
49
Subacute sclerosing panencephalitis presenting as acute disseminated encephalomyelitis and pseudotumour cerebri. ( 23964034 )
2013
50
Subacute sclerosing panencephalitis: rapidly progressive cognitive decline in a young patient. ( 23665789 )
2013

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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