Panencephalitis, Subacute Sclerosing malady
Categories: Rare diseases, Infectious diseases, Neuronal diseases, Immune diseases
Aliases & Descriptions for Panencephalitis, Subacute Sclerosing:
Orphanet epidemiological data:53
subacute sclerosing panencephalitis:
Inheritance: Not applicable; Prevalence: <1/1000000 (United States),1-9/100000 (India); Age of onset: All ages; Age of death: any age
panencephalitis, subacute sclerosing:
Inheritance: autosomal recessive inheritance
Global: Rare diseases, Infectious diseases
Anatomical: Neuronal diseases, Immune diseases
ICD10: 30 29
Rare neurological diseases
NINDS:48 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 measles and penis basal cell carcinoma, and has symptoms including encephalitis An important gene associated with Panencephalitis, Subacute Sclerosing is CD46 (CD46 Molecule), and among its related pathways is NF-kappaB Signaling. Affiliated tissues include brain, brain and heart, and related mouse phenotypes are hematopoietic system and immune system.
Disease Ontology:11 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:47 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. Last updated: 4/20/2016
Wikipedia:70 Subacute sclerosing panencephalitis (SSPE) also known as Dawson Disease, Dawson encephalitis, and... more...
Description from OMIM:51 260470
Interventional clinical trials:
Search NIH Clinical Center for Panencephalitis, Subacute Sclerosing
MalaCards organs/tissues related to Panencephalitis, Subacute Sclerosing:35
FMA organs/tissues related to Panencephalitis, Subacute Sclerosing:17
MGI Mouse Phenotypes related to Panencephalitis, Subacute Sclerosing:40
Search GEO for disease gene expression data for Panencephalitis, Subacute Sclerosing.
Biological processes related to Panencephalitis, Subacute Sclerosing according to GeneCards Suite gene sharing:
Molecular functions related to Panencephalitis, Subacute Sclerosing according to GeneCards Suite gene sharing:
30ICD10 via Orphanet
39MESH via Orphanet
52OMIM via Orphanet
62SNOMED-CT via Orphanet
66Tumor Gene Family of Databases
68UMLS via Orphanet