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.
Currently, there is no cure for SSPE. Showing of 2 View All. Brain inflammation. Do you have more information about symptoms of this disease? We want to hear from you. Cause Cause. Subacute sclerosing panencephalitis SSPE is caused by a measles infection that is acquired earlier in life often years prior to the onset of SSPE symptoms.
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 brain and spinal cord.
SSPE affects males more often than females and is generally diagnosed in children and adolescents. The risk of developing SSPE may be higher for a person who gets measles before they are two years of age.
Diagnosis Diagnosis. A diagnosis of subacute sclerosing panencephalitis is often suspected based on the presence of characteristic signs and symptoms in a person with a history of the measles.
Additional testing can then be offered to confirm the diagnosis. Treatment Treatment. Unfortunately, there is currently no cure for subacute sclerosing panencephalitis SSPE. Treatment is supportive and primarily based on the signs and symptoms present in each person. For example, anticonvulsant and antispasmodic drugs may be given, as needed, to address some of the motor disturbances associated with the condition.
Although these drugs can prolong life, the best treatment regimen and their long-term effects in people with SSPE are currently unknown. Prognosis Prognosis. The long-term outlook prognosis for people with subacute sclerosing panencephalitis SSPE is poor. The condition is always fatal. Although most affected people die within one to three years, the average lifespan following diagnosis can vary. Studies show that a small group of people with SSPE will have a rapidly progressive form of the condition, leading to death within three months of diagnosis.
Although rare, another small group will have a chronic, slowly progressive form that is associated with a relapsing and remitting course. Statistics Statistics. In developed countries, subacute sclerosing panencephalitis SSPE is considered a rare disease. For example, fewer than 10 cases per year are reported in the United States. Furthermore, studies show that the number of SSPE cases diagnosed each year has declined by at least 90 percent in countries that have practiced widespread immunization with measles vaccine.
The risk of having the condition is significantly higher in developing countries, such as India over 20 cases per million people each year and Eastern Europe. Do you have updated information on this disease? Find a Specialist Find a Specialist. Healthcare Resources To find a medical professional who specializes in genetics, you can ask your doctor for a referral or you can search for one yourself.
You can also learn more about genetic consultations from MedlinePlus Genetics. Am J Epidemiol. The epidemiology of subacute sclerosing panencephalitis in England and Wales Int J Epidemiol. Environmental risk factors for subacute sclerosing panencephalitis SSPE.
Acta Neurol Scand. Epidemiology of subacute sclerosing panencephalitis. J Pediatr. Risk factors in subacute sclerosing panencephalitis: age- and sex-dependent host reactions or intensive exposure? Rev Infect Dis. Changing character of subacute sclerosing panencephalitis in the United States. Pediatr Neurol. Clonal expansion of hypermutated measles virus in a SSPE brain. Cerebral endothelial cell infection by measles virus in subacute sclerosing panencephalitis: ultrastructural and in situ hybridization evidence.
Neuropathol Appl Neurobiol. Measles virus infection of cells in perivascular infiltrates in the brain in subacute sclerosing panencephalitis: confirmation by non-radioactive in situ hybridization, immunocytochemistry and electron microscopy. Acta Neuropathol. Clinical spectrum of measles. Curr Top Microbiol Immunol. Weekly clinicopathological exercises.
Case A year-old woman with a three-month history of progressive mental deterioration. N Engl J Med. Measles virus spread between neurons requires cell contact but not CD46 expression, syncytium formation, or extracellular virus production. J Virol. Subacute sclerosing panencephalitis virus dominantly interferes with replication of wild-type measles virus in a mixed infection: implication for viral persistence.
Defective translation of measles virus matrix protein in a subacute sclerosing panencephalitis cell line. Mutated and hypermutated genes of persistent measles viruses which caused lethal human brain diseases. Functional and nonfunctional measles virus matrix genes from lethal human brain infections. Functional analysis of matrix proteins expressed from cloned genes of measles virus variants that cause subacute sclerosing panencephalitis reveals a common defect in nucleocapsid binding.
Defective measles virus in human subacute sclerosing panencephalitis brain. Generation and properties of measles virus mutations typically associated with subacute sclerosing panencephalitis. Ann N Y Acad Sci. The human CD46 molecule is a receptor for measles virus Edmonston strain. Cell fusion by the envelope glycoproteins of persistent measles viruses which caused lethal human brain disease. Subacute sclerosing panencephalitis is typically characterized by alterations in the fusion protein cytoplasmic domain of the persisting measles virus.
Measles viruses with altered envelope protein cytoplasmic tails gain cell fusion competence. In advanced stages, there is marked cerebral atrophy.
Definitive diagnosis requires demonstration of elevated measles antibody titers in cerebrospinal fluid CSF. Many drugs have been used to stabilize the course of the disease but without evidence from randomized clinical trials.
Six percent of patients may experience prolonged spontaneous remission. Fusion inhibitor peptide may, in the future, be exploited to treat SSPE. A universal vaccination against measles is the only proven way to tackle this menace currently.
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