Infrequently, sspe can occur in adults and pregnant women. Brain biopsies or postmortem histopathological examination show evidence of astrogliosis, neuronal loss, degeneration of dendrites, demyelination, neurofibrillary tangles, and infiltration of inflammatory cells. Subacute sclerosing panencephalitis is a progressive neurodegenerative disease. Imaging demonstrates nonspecific atrophy and increased t2 signal within cerebral white matter. Subacute sclerosing panencephalitis sspe childrens. Subacute sclerosing panencephalitis sspe pediatrics msd. Pdf subacute sclerosing panencephalitis researchgate.
Subacute sclerosing panencephalitis sspe is thought to be an encephalitis secondary to reactivation of a latent measles infection. The matrix gene is most commonly affected by these. Apr 20, 2016 subacute sclerosing panencephalitis sspe generally develops approximately seven to ten years after a person recovers from the measles. However, an abnormal immune response to measles or, possibly, certain mutant forms of the. Subacute sclerosing panencephalitis sspe is a progressive neurologic disorder caused by measles virus that silently develops for several years after the primary measles virus infection and is inevitably fatal. Its incidence rates in developed countries have been reported as 711 cases of sspe100,000 cases of measles.
Subacute sclerosing panencephalitis sspe is a progressive, disabling, and deadly brain disorder related to measles rubeola infection. Walop three different forms of infections in the central nervous system have been associated with the measles virus. To analyze the profile of patients with a relatively benign course who survive beyond 3 years. Subacute sclerosing panencephalitis sspe a rare condition that is caused by a measles infection acquired earlier in life. Magnetic resonance mr imaging is the modality of choice for determining such changes in white matter. Sspe syn drome can affect people years from the acute measles virus infection, as result of the persistence of defective viral particles in brain cells. There is a higher incidence among males than females malefemale. The clinical course is characterized by progressive. A rare, fatal late complication of measles, sspe is often overlooked in assessments focused on the acute illness. Subacute sclerosing panencephalitis postgraduate medical.
Subacute sclerosing panencephalitis occurs because the measles virus reactivates. Subacute sclerosing panencephalitis in perspective w. Division of molecular biology, school of biology and biochemistry, the queens university of belfast, belfast bt9 7bl, n. Full text subacute sclerosing panencephalitis current. The patient, aged 14 years, had reportedly been vaccinated against measles in. Subacute sclerosing panencephalitis sspe is a rare and deadly brain disorder. Sclerosing means a reaction which damages and scars the brain. There are four diagnostic criteria, namely the clinical picture, a characteristic eeg, serology of serum and csf and brain histology. High incidence of subacute sclerosing panencephalitis in. We describe a fatal case of sspe in an internationally adopted child 9 years after he arrived in the united states. 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. D subacute sclerosing panencephalitis, the journal of infectious diseases, volume 121, issue 2, 1 february 1970, pages 227230, s.
Subacute sclerosing panencephalitis, is a progressive fatal neuroinfection caused by persistent mutated measles virus. Among persons who contracted measles during the resurgence in the united states u. Subacute sclerosing panencephalitis a case report presenting. Panencephalitis means that all areas of the brain are affected. Long term survival in subacute sclerosing panencephalitis.
On inquiry, there is a history of measles at 3 years of age. Subacute sclerosing panencephalitis sspe usually has a progressive stereotypic downhill course and results in premature death. The late symptoms of subacute sclerosing panencephalitis may include muscle rigidity, elevated body. Subacute sclerosing panencephalitis is a progressive, usually fatal brain disorder occurring months to usually years after an attack of measles. Panencephalitis definition of panencephalitis by the free. The reason why the measles virus persists in some leading to fatal sspe is unclear. It is a slow, but persistent, viral infection caused by defective measles virus. It is caused by an aberrant measles virus, known as the sspe virus. Its pathogenesis is not well understood but may involve persistent infection with a genetic variant of measles virus within the central nervous system. Other articles where subacute sclerosing panencephalitis is discussed. This page was last edited on 26 november 2016, at 18. High incidence of subacute sclerosing panencephalitis in south india volume 104 issue 1 v.
Subacute sclerosing panencephalitis resembling rasmussens. Pdf subacute sclerosing panencephalitis current perspectives. In 2015, the oregon health authority was notified of the death of a boy with subacute sclerosing panencephalitis sspe, a rare and fatal complication of measles. The disease is caused by the measles virus, with the particular disorder developing when a child is infected with a form of the virus that mutates and does not go away. Pdf subacute sclerosing panencephalitis sspe is a subacute encephalopathy of childhood and young adolescence. Normally, the measles virus does not cause brain damage. Paired serum 500 l minimum and csf 500 l minimum, collected at the same time if possible within an hour at most with total igg and total albumin concentrations mgl determined. Subacute means a slow start and, usually, a gradual progression. Subacute sclerosing panencephalitis sspe, also known as dawson disease, is a rare chronic, progressive and fatal encephalitis that affects primarily children and young adults, caused by a persistent infection of immune resistant measles virus.
In 1972, the uk government had serious concerns about the measles vaccines potential to cause vaccineinduced subacute sclerosing panencephalitis sspe. Longterm stabilization or remission is exceptional. Subacute sclerosing panencephalitis definition of subacute. Subacute sclerosing panencephalitis is a slowly progressive disease manifested by dementia and myoclonus accompanied. Subacute sclerosing panencephalitis is a rare neurological disease of childhood or young adulthood.
Subacute sclerosing panencephalitis sspe diagnosis guide. Subacute sclerosing panencephalitis genetic and rare. Subacute sclerosing panencephalitis, commonly known as sspe, is a progressive neurodegenerative disease caused by the persistence of. Subtle immunologic abnormalities in four boys with subacute sclerosing panencephalitis.
It usually occurs 710 years after measles infection. Georgia had 8377 and 11,495 reported measles cases during the 20042005 and 202015 outbreaks, respectively, but sspe. Subacute sclerosing panencephalitis is a form of chronic measles encephalitis that was once endemic in several parts of the world but has almost disappeared in countries that require routine measles immunization honarmand et al, 2004. Subacute sclerosing panencephalitis sspe facts and. The pathogenesis of subacute sclerosing panencephalitis. Jan 15, 2016 in 2015, the oregon health authority was notified of the death of a boy with subacute sclerosing panencephalitis sspe, a rare and fatal complication of measles. Subacute sclerosing panencephalitis sspe, a postinfectious neurologic complication of measles, can also occur.
Subacute sclerosis panencephalitis, also known as sspe, is a rare disorder that usually affects children and young adults. See the major article by wendorf et al on pages 22632. Subacute sclerosing panencephalitis sspe is a progressive neurological disorder of children and young adults that affects the central nervous system cns. Panencephalitis definition of panencephalitis by the. It usually occurs in boys more often than in girls. Subacute sclerosing panencephalitis pathology britannica. Subacute sclerosing panencephalitis clinical infectious. Subacute sclerosing panencephalitis background subacute sclerosing panencephalitis sspe is a rare, late complication of measles and is invariably fatal. Know the causes, symptoms, treatment and prognosis of subacute sclerosing panencephalitis. It is caused by persistent defective measles virus. Encephalitis that affects both gray and white matter of the brain, resulting in progressive loss of mental and motor functions. Sspe virus differs from wildtype measles viruses in the form of several mutations affecting the viral genome.
The average latency period following primary infection is 610 years but can range from 2 to 30 years, and the time from early. Sspe has been reported from all parts of the world, but it is considered a rare disease in developed. In a nonimmunized population, the average age at onset is 8 years. High risk of subacute sclerosing panencephalitis following. Subacute sclerosing panencephalitis is a slowly progressive disease manifested by dementia and myoclonus accompanied by characteristic electroencephalographic and. It causes problems with thinking, uncontrolled movements, and seizures that worsen over time.
Subacute sclerosing panencephalitis is an extremely rare but extremely serious and potentially fatal disorder of the brain which is basically caused by an abnormal reaction of the immune system of the body to the measles virus. The disease develops many years after the measles infection. Oct 14, 2008 subacute sclerosing panencephalitis sspe is a subacute encephalopathy of childhood and young adolescence. The measles vaccine remains highlyeffective in preventing the numbers of children affected by subacute sclerosing panencephalitis sspe. Subacute sclerosing panencephalitis sspe is now considered to be caused by measles virus. Serologic diagnosis of measles in subacute sclerosing panencephalitis. Sspe is a degenerative neurological condition, which affects a persons behaviour, memory and coordination, leading to fits, blindness and eventually death. Early signs and symptoms of the condition can include behavioral changes and mild mental deterioration affecting memory, thinking, language, and judgment. Subacute sclerosing panencephalitis sspe is a fatal, progressive degenerative disease of the central nervous system that usually occurs 7 to 10 years after natural measles virus infection. Subacute sclerosing panencephalitis radiology case. One rare longterm sequelae of measles virus infection is subacute sclerosing panencephalitis sspe, a fatal disease of the central nervous system that generally develops 710 years after infection. It causes mental deterioration, myoclonic jerks, and seizures.
Affected people may initially experience behavioral changes, dementia, and. In the past in the united states, for reasons that are not known, the disorder occurred in about 7 to 300 people per million people who had measles infection and in about 1 person per million people who received the measles vaccine. Motor incoordination and impairment of speech and sight subsequently develop. It is a slow virus infection caused by a genetically altered form of the. To describe cases and estimate subacute sclerosing panencephalitis sspe risk following largesale measles outbreaks in georgia. Subacute sclerosis panencephalitis and ssa disability. Subacute sclerosing panencephalitis radiology reference. Subacute sclerosing panencephalitis is a neurodegenerative disease secondary to measles infection that usually has a typical presentation with progressive myoclonia, cognitive decline, and periodic slowwave complexes on electroencephalography. May 26, 2016 subacute sclerosing panencephalitis sspe is a brain disorder. Diagnosis involves electroencephalography, ct or mri, cerebrospinal fluid examination, and measles serologic testing. Subacute sclerosing panencephalitis sspe is a progressively fatal degenerative disease of the central nervous system. An abnormal reaction by your immune system to the measles virus, or rubeola, is likely the cause. Despite the availablility of vaccination, a lar ge nationwide epidemic of measles occurred between mid1992 and mid1995.
Subacute sclerosing panencephalitis current perspectives ncbi. Presenting with imbalance and motor complaints for the last 3 years. Chronic encephalitis means a type of encephalitis that has a slow time course. Subacute sclerosing panencephalitis genetic and rare diseases. The patient, aged 14 years, had reportedly been vaccinated against measles in the philippines at age 8 months. A case of fulminant subacute sclerosing panencephalitis presenting. Subacute sclerosing panencephalitis sspe is a progressive, disabling, and deadly brain disorder related to measles rubeola infection the disease. The patient was apparently well until about 6 years of age. Subacute sclerosing panencephalitis sspe pediatrics. Subacute sclerosing panencephalitis is a progressive disease which results in personality changes, outbursts of temper, sleeplessness, disorientation, stupor, spasticity, loss of previously acquired intellectual skills, poor memory and judgment dementia, and general neurological deterioration.
The condition primarily affects children, teens, and young adults. Description this fatal condition is a complication of measles, and affects children and young adults before the age of 20. The average latency period following primary infection is 610 years but can range from 2 to 30 years. Subacute sclerosing panencephalitis information mount. Subacute sclerosing panencephalitis the journal of. Subacute sclerosing panencephalitiscausessymptomstreatment. Subacute sclerosing panencephalitis definition subacute sclerosing panencephalitis is a rare, progressive brain disorder caused by an abnormal immune response to the measles virus.
Subacute sclerosing panencephalitis is a progressive disease which results in personality changes, outbursts of temper, sleeplessness, disorientation, stupor, spasticity, loss of previously acquired intellectual skills, poor memory and judgment dementia, and. Subacute sclerosing panencephalitis information page. Subacute sclerosing panencephalitis sspe is a rare, slowly progressing but invariably fatal disease that is related to a prior measles virus infection and most commonly affects paediatric patients. Subacute sclerosing panencephalitis sspe diagnosis. Subacute sclerosing panencephalitis sspealso known as dawson diseaseis a rare form of chronic progressive brain inflammation caused by slow infection with certain defective strains of hypermutated measles virus. Mar 27, 2019 subacute sclerosing panencephalitis sspe is a progressive neurological disorder of children and young adults that affects the central nervous system cns. Subacute sclerosing panencephalitis sspe is a brain disorder.
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