It causes mental deterioration, myoclonic jerks, and seizures. It has been estimated that about 1 in 10,000 people who get measles will eventually develop sspe. 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. It is a slow, but persistent, viral infection caused by defective measles virus. Subacute sclerosing panencephalitis occurs because the measles virus reactivates. It usually occurs 710 years after measles infection. Subacute sclerosing panencephalitiscausessymptomstreatment. Pdf on may 1, 1998, ravindra kumar garg and others published subacute sclerosing panencephalitis find, read and cite all the research you need on researchgate. Chronic encephalitis means a type of encephalitis that has a slow time course. Introduction subacute sclerosing panencephalitis is the most. Subacute sclerosing panencephalitis sspe is a progressively fatal degenerative disease of the central nervous system. 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. Subacute sclerosing panencephalitis sspe also known as dawson diseaseis a rare form of chronic progressive brain inflammation caused by slow infection with certain defective strains of hypermutated measles virus. Subacute sclerosing panencephalitis was first des cribed by dawson 1933 and later van bogaert 1945.
Mri findings in subacute sclerosing panencephalitis neurology. The matrix gene is most commonly affected by these. Infrequently, sspe can occur in adults and pregnant women. 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 sspe open government. The term subacute sclerosing panencephalitis has been used since green. Subacute sclerosing panencephalitis current perspectives sidra k jafri, raman kumar, shahnaz h ibrahim department of pediatrics and child health, aga khan university hospital, karachi, pakistan abstract. Subacute sclerosing panencephalitis sspe childrens.
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. Full text subacute sclerosing panencephalitis current. Subacute sclerosing panencephalitis sspe is a chronic encephalitis occurring after infection with measles virus. Motor incoordination and impairment of speech and sight subsequently develop. Affected people may initially experience behavioral changes, dementia, and disturbances in motor function. The condition primarily affects children, teens, and young adults. Despite the availablility of vaccination, a lar ge nationwide epidemic of measles occurred between mid1992 and mid1995. The term subacute sclerosing panencephalitis has been used since. Diagnosis involves electroencephalography, ct or mri, cerebrospinal fluid examination, and measles serologic testing. Subacute sclerosing panencephalitis sspe pediatrics. In a nonimmunized population, the average age at onset is 8 years.
Subacute sclerosing panencephalitis current perspectives phmt. Sspe is a degenerative neurological condition which progressively destroys nerve cells in the brain almost always leading to mental deterioration and death. The patient was found to have had subacute sclerosing panencephalitis at autopsy. However, a 2016 study estimated that the rate for babies who contracted measles was as high as 1 in 609. Apr 20, 2016 subacute sclerosing panencephalitis sspe generally develops approximately seven to ten years after a person recovers from the measles. Sspe virus differs from wildtype measles viruses in the form of several mutations affecting the viral genome. Subacute sclerosing panencephalitis and chorioretinitis. Measles is also the cause of a disease called subacute sclerosing panencephalitis sspe. Methodology children under 16 years of age discharged from pediatric unit of akuh with a diagnosis of sspe from january 2000 to june 2012 were included in the study. Subacute sclerosing panencephalitis radiology reference. 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. Journal of the neurological sciences 9 1996 227234. Pdf subacute sclerosing panencephalitis sspe is a subacute encephalopathy of childhood and young adolescence.
Know the causes, symptoms, treatment and prognosis of subacute sclerosing panencephalitis. May 26, 2016 subacute sclerosing panencephalitis sspe is a brain disorder. Sspe was not suspected in the differential diagnosis of three of the cases until results of measles testing were known. Sspe is a rare complication approximately 1 in 300,000 cases of measles, occurring predominantly in children in their first decade of life. Jun 24, 2019 subacute sclerosing panencephalitis sspe is a slowly progressive brain disorder caused by mutant measles virus. A case of subacute sclerosing panencephalitis in a 2yearold boy is reported.
Walop three different forms of infections in the central nervous system have been associated with the measles virus. Public health notifiable disease management guidelines. See the major article by wendorf et al on pages 22632. Brain biopsies or postmortem histopathological examination show evidence of astrogliosis, neuronal loss, degeneration of dendrites, demyelination, neurofibrillary tangles, and. Sspe has been reported from all parts of the world, but it is considered a rare disease in developed. Subacute sclerosing panencephalitis findings at mr imaging. Oct 26, 2004 the authors describe five cases of subacute sclerosing panencephalitis sspe identified through the california encephalitis project that emphasize the importance of considering sspe in the differential diagnosis of encephalitis, particularly among pediatric patients.
Subacute sclerosing panencephalitis background subacute sclerosing panencephalitis sspe is a rare, late complication of measles and is invariably fatal. In addition to asymmetric lesions in the parietotemporal lobes, right thalamus, and globus pallidus, symmetric patterns were notable in the brain stem, middle cerebellar pedincles, and dentate nuclei. Despite the availablility of vaccination, a lar ge nationwide epidemic of. The disease has been related to a persistent and aberrant measles virus infection and no. It is a slow virus infection caused by a genetically altered form of the. It is caused by persistent defective measles virus. Summary subacute sclerosing panencephalitis sspe is a slowly progressive brain disorder caused by mutant measles virus. Subacute sclerosing panencephalitis genetic and rare. Subacute sclerosing panencephalitis, a measles complication. Subacute sclerosing panencephalitis information page. Thirtyfour mri studies of 26 patients with subacute sclerosing panencephalitis are reported.
Subacute sclerosing panencephalitis in perspective w. The average latency period following primary infection is 610 years but can range from 2 to 30 years, and the time from early. 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. Lesions tend to start in the cortexsubcortical white matter and progress with periventricular white matter involvement and diffuse cerebral. Its pathogenesis is not well understood but may involve persistent infection with a genetic variant of measles virus within the central nervous system. Panencephalitis means that all areas of the brain are affected.
Subacute sclerosing panencephalitis clinical infectious. 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. Measles infection and encephalitis the encephalitis society. Sspe is a common and serious disease of the central nervous system cns, and is caused by a mutant measles virus.
Subacute sclerosing panencephalitis sspe is a serious disorder of the central nervous system. An abnormal reaction by your immune system to the measles virus, or rubeola, is likely the cause. Subacute sclerosing panencephalitis pubmed central pmc. A case of fulminant subacute sclerosing panencephalitis. Our aim was to determine the metabolic abnormalities of brain in early and latestage sspe by using mr spectroscopy and to assess areas of involvement in the early stages when mr imaging findings were normal. The clinical course is characterized by progressive. Any patient who did not fulfill the diagnostic criteria of sspe was excluded from study. Subacute sclerosing panencephalitis sspe a rare condition that is caused by a measles infection acquired earlier in life. 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 complication of measles. It is caused by an aberrant measles virus, known as the sspe virus. Subacute sclerosing panencephalitis sspe is a rare, progressive, inflammatory neurodegenerative disease. Subacute sclerosing panencephalitis sspe is a progressive neurological disorder of children and young adults that affects the central nervous system cns.
A case of fulminant subacute sclerosing panencephalitis presenting with acute myoclonicastatic epilepsy. Subacute sclerosing panencephalitis sspe is a progressive inflammatory disorder of the central nervous system with both poor prognosis and high mortality. Subacute sclerosing panencephalitis current perspectives ncbi. Subacute sclerosing panencephalitis sspe 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. Mar 27, 2019 subacute sclerosing panencephalitis sspe is a progressive neurological disorder of children and young adults that affects the central nervous system cns. Other articles where subacute sclerosing panencephalitis is discussed. Lesions of high signal intensity on t2weighted images are the most common finding. Subacute sclerosing panencephalitis is a progressive neurodegenerative disease. Early and latestate subacute sclerosing panencephalitis. Subacute sclerosing panencephalitis pathology britannica. Pdf subacute sclerosing panencephalitis and chorioretinitis. The patient, aged 14 years, had reportedly been vaccinated against measles in the philippines at age 8 months.
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. 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. Subacute sclerosing panencephalitis, commonly known as sspe, is a progressive neurodegenerative disease caused by the persistence of measles infection commonly seen in children and young adults. Subacute sclerosing panencephalitis sspe is a chronic, degenerative cns disorder attributed to persistent infection with a defective measles virus despite robust host immune responses. This is a rare condition that can develop some years after natural measles infection. 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. Proton mr spectroscopy revealed markedly decreased n acetylaspartate peaks and normal choline and myo. Subacute sclerosing panencephalitis sspe is a subacute encephalopathy of childhood and young adolescence. Subacute sclerosing panencephalitis is a progressive, usually fatal brain disorder occurring months to usually years after an attack of measles. The former described under the name of subacute inclusion body ancephalitis while the latter called it sclerosing leucoencephalitis. Subacute sclerosing panencephalitis sspe is a progressive neurological disorder of childhood and early adolescence. Pdf subacute sclerosing panencephalitis researchgate. Brain biopsies or postmortem histopathological examination show evidence of astrogliosis, neuronal loss, degeneration of dendrites, demyelination, neurofibrillary tangles, and infiltration of inflammatory cells.
Subacute means a slow start and, usually, a gradual progression. Subacute sclerosing panencephalitis oxford academic journals. Subacute sclerosing panencephalitis postgraduate medical. Subacute sclerosing panencephalitis sspe pediatrics msd. Sclerosing means a reaction which damages and scars the brain.
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