Typically, affected persons have a history of measles infection in infancy period 1. Subacute sclerosing panencephalitis sspe is a progressive neurological disorder characterized by inflammation of the brain encephalitis. Feb 17, 2015 sub acute sclerosing panencephalitis 1. This paper describes the fundus changes in 2 patients with subacute sclerosing panencephalitis sspe. Subacute sclerosing panencephalitis postgraduate medical. Subacute sclerosing panencephalitis is a progressive, usually fatal brain disorder occurring months to usually years after an attack of measles. Other articles where subacute sclerosing panencephalitis is discussed. The former described under the name of subacute inclusion body ancephalitis while the latter called it sclerosing leucoencephalitis.
Subacute sclerosing panencephalitis sspe, also known as dawson disease, is a gradually developing neurological disorder that affects children and young adults. Subacute sclerosing panencephalitis sspe is a rare and deadly brain disorder. Subacute sclerosing panencephalitis sspe also known as dawson disease is a rare form of chronic progressive brain inflammation caused by slow infection with certain defective strains of hypermutated measles virus. Ct of a patient with subacute sclerosing panencephalitis sspe. The condition primarily affects children, teens, and young adults.
Subacute sclerosing panencephalitis definition subacute sclerosing panencephalitis is a rare, progressive brain disorder caused by an abnormal immune response to the measles virus. The clinical course is characterized by progressive. 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. It is a slow, but persistent, viral infection caused by defective measles virus. Subacute sclerosing panencephalitis measles virus in the brain jayamaha, j. Subacute sclerosing panencephalitis sspe facts and. A chronic brain disease of children and adolescents that occurs months to often years after an attack of measles, causing convulsions, motor abnormalities, mental retardation and, usually, death. Affected people may initially experience behavioral changes, dementia, and disturbances in motor. This fatal condition is a complication of measles, and affects children and. Early diagnosis may help in medical interventions and counseling. This report documents current data relating to the clinical phenotype, epidemiology, and understanding of sspe, inclusive of pre ventive.
May 26, 2016 subacute sclerosing panencephalitis sspe is a brain disorder. Subacute sclerosing panencephalitis was first des cribed by dawson 1933 and later van bogaert 1945. Nov 15, 2005 subacute sclerosing panencephalitis sspe is a progressive fatal disease of the central nervous system that is caused by a persistent measles virus infection. Subacute sclerosing panencephalitis sspe, a neurodegenerative disease caused by a persistent slow virus infection with a mutated measles virus, is endemic in much of the developing world. Subacute sclerosing panencephalitis penn state hershey. 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. The measles vaccine remains highlyeffective in preventing the numbers of children affected by subacute sclerosing panencephalitis sspe. Hemagglutininspecific neutralization of subacute sclerosing. Virus nucleocapsids are present in the brain and the patient is in a state of hyperimmunization towards. It is a slow virus infection caused by a genetically altered form of the. Affected people may initially experience behavioral changes, dementia, and disturbances in motor function. 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. Mar 27, 2019 subacute sclerosing panencephalitis sspe is a progressive neurological disorder of children and young adults that affects the central nervous system cns.
Early clinical characteristics of sspe may be variable, but they often include behavioral changes, cognitive deterioration, sporadic episodes of falling, and such optic disturbances as. It is caused by persistent defective measles virus. It usually occurs in boys more often than in girls. Encephalitis that affects both gray and white matter of the brain, resulting in progressive loss of mental and motor functions. Subacute sclerosing panencephalitis sspe is thought to be an encephalitis secondary to reactivation of a latent 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. Its pathogenesis is not well understood but may involve persistent infection with a genetic variant of measles virus within the central nervous system. Measlesvirus antibody and antigen in subacute sclerosing panencephalitis. Diagnosis involves electroencephalography, ct or mri, cerebrospinal fluid examination, and measles serologic testing. 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.
List three differential diagnoses for underlying etiology of catatonia as it pertains to this case 3. The information on demographics, dates of onset of sspe symptoms and diagnosis, history of measles disease and vaccination, sspe stage at the time of diagnosis. Its incidence will increase in the usa, not only in immigrants, but also because of the 19881990 measles epidemic. Subacute sclerosing panencephalitis sspe is a fatal complication of measles. Oct 14, 2008 subacute sclerosing panencephalitis sspe is a subacute encephalopathy of childhood and young adolescence. Subacute sclerosing panencephalitis radiology case. This emedtv article presents a detailed overview of this condition, with information on its symptoms, stages, treatment, and more. Despite the availablility of vaccination, a lar ge nationwide epidemic of measles occurred between mid1992 and mid1995. Pdf subacute sclerosing panencephalitis researchgate. 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 is a rare, progressive brain disorder caused by an abnormal immune response to the measles virus description.
Subacute sclerosing panencephalitis information mount. Subacute sclerosing panencephalitis definition of subacute. Subacute sclerosing panencephalitis pubmed central pmc. Subacute sclerosing panencephalitis sspe is a progressive neurological disorder of children and young adults that affects the central nervous system cns. Apr 20, 2016 subacute sclerosing panencephalitis sspe generally develops approximately seven to ten years after a person recovers from the measles. Subacute sclerosing panencephalitis manifesting as viral retinitis. Despite the availablility of vaccination, a lar ge nationwide epidemic of. It causes mental deterioration, myoclonic jerks, and seizures. 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 pediatrics. Sspe syn drome can affect people years from the acute measles virus infection. Subacute sclerosing panencephalitis sspe is a progressive inflammatory disorder of the central nervous system with both poor prognosis and high mortality. Subacute sclerosing panencephalitis information page. It is more common in males, as they are three times more likely.
Sspe has been reported from all parts of the world, but it is considered a rare disease in developed. The disease may develop due to reactivation of the measles virus or an inappropriate immune response to the measles virus. It has been estimated that about 1 in 10,000 people who. Subacute sclerosing panencephalitis current perspectives ncbi. 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 sspe is a slowly progressive brain disorder caused by mutant measles virus. Subacute sclerosing panencephalitis is a progressive neurodegenerative disease. It has been estimated that about 1 in 10,000 people who get measles will eventually develop sspe. Subacute sclerosing panencephalitis sspe is a rare, alwaysfatal, neurodegenerative disease caused by measles virus infection of the central nervous system, as a sequela of measles. A case of fulminant subacute sclerosing panencephalitis. The disease usually emerges 57 years after the child has had an uneventful measles infection. Get a printable copy pdf file of the complete article 990k, or click on a page.
Subacute sclerosing panencephalitis background subacute sclerosing panencephalitis sspe is a rare, late complication of measles and is invariably fatal. Subacute sclerosing panencephalitis occurs because the measles virus reactivates. Subacute sclerosing panencephalitis sspe is a subacute encephalopathy of childhood and young adolescence. 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. See the major article by wendorf et al on pages 22632. However, an abnormal immune response to measles or, possibly, certain mutant forms of the. The disease develops many years after the measles infection.
News report presented by ray martin following the story of a young girl affected by subacute sclerosing panencephalitis sspe also known as measles encephalitis. 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. Spinal cord injury subacute sclerosing panencephalitis sandhoff disease scott craniodigital syndrome smithlemliopitz syndrome spinal muscular atrophy subdural hematoma sandifer syndrome seckel syndrome smithmagenis syndrome spinal stenosis sydenham chorea sanfilippo syndrome seizures social anxiety disorder split handsplit foot. Definition of subacute sclerosing panencephalitis sspe. It is caused by an aberrant measles virus, known as the sspe virus. Catatonia as a clinical feature of subacute sclerosing panencephalitis 1. Sarah walton caught measles when she was 11 months old, and at the time recovered well. The viruses isolated from subacute sclerosing panencephalitis appear to be variants of measles virus when investigated either in cell cultures oyanagi, ter meulen, katz, and koprowski, 1971 or when inoculated intracerebrally into. The variable natural history of subacute sclerosing panencephalitis. Sub acute sclerosing panencephalitis linkedin slideshare. The patient, aged 14 years, had reportedly been vaccinated against measles in the philippines at age 8 months. Pdf ocular manifestations in subacute sclerosing panencephalitis ip innovative publication pvt. Subacute sclerosing panencephalitis is a serious infection caused by an altered form of the measles virus. Dec 15, 2005 one such complication, subacute sclerosing panencephalitis sspe, is a devastating, progressive, central nervous system destructive process caused by mutant measles virus infection of neurons.
Infrequently, sspe can occur in adults and pregnant women. Introduction subacute sclerosing panencephalitis is the most. Subacute sclerosing panencephalitis pathology britannica. 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. Motor incoordination and impairment of speech and sight subsequently develop. Subacute sclerosing panencephalitis sspe is a brain disorder.
Panencephalitis means that all areas of the brain are affected. Subacute sclerosing panencephalitis sspe is a rare, fatal complication of measles characterized by cognitive and motor deterioration, with death typically occurring within years of symptom onset. Chronic encephalitis means a type of encephalitis that has a slow time course. Subacute sclerosingpanencephalitis sclerosing panencephalitis which suggests that cells ofthe lymphocyte ormacrophage series are capable ofcarrying measles virus. The matrix gene is most commonly affected by these. Subacute sclerosing panencephalitis 78i suggest instead a morelocalized intraocular disease process table ii. Subacute sclerosing panencephalitis sspe a rare condition that is caused by a measles infection acquired earlier in life. Benefits for subacute sclerosing panencephalitis sspe. Description this fatal condition is a complication of measles, and affects children and young adults before the age of 20. Subacute sclerosing panencephalitis sspe is a progressive neurological disorder of childhood and early adolescence. Serologic diagnosis of measles in subacute sclerosing panencephalitis. Subacute sclerosing panencephalitis clinical infectious. Subacute sclerosing panencephalitis sspe is a progressive, disabling, and deadly brain disorder related to measles rubeola infection the disease develops many years after the measles infection. It usually occurs 710 years after measles infection.
Subtle immunologic abnormalities in four boys with subacute sclerosing panencephalitis. Subacute sclerosing panencephalitis sspe childrens. Sspe virus differs from wildtype measles viruses in the form of several mutations affecting the viral genome. Normally, the measles virus does not cause brain damage. 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. Twenty years later, however, she fell ill and was diagnosed with. Imaging demonstrates nonspecific atrophy and increased t2 signal within cerebral white matter. Subacute sclerosing panencephalitis wiley online library. There is a higher incidence among males than females malefemale. It causes problems with thinking, uncontrolled movements, and seizures that worsen over time. The usual age of onset of the disease is in childhood between the age of 10 to 14 years. Subacute sclerosing panencephalitis radiology reference. Subacute sclerosing panencephalitis sspe is a chronic encephalitis occurring after infection with measles virus. Subacute sclerosing panencephalitis sspe is a progressively fatal degenerative disease of the central nervous system.
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 progressive disease caused by wildtype measles virus leading to premature death. 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. An abnormal reaction by your immune system to the measles virus, or rubeola, is likely the cause.
Pdf subacute sclerosing panencephalitis sspe is a subacute encephalopathy of childhood and young adolescence. Subacute sclerosing panencephalitis sspe is a progressive, disabling, and deadly brain disorder related to measles rubeola infection. Measles complications are a risk in international adoptions. Subacute sclerosing panencephalitis definition subacute sclerosing panencephalitis sspe is a progressive, disabling, and deadly brain disorder related to measles rubeola infection the disease develops many years after the measles infection. Panencephalitis definition of panencephalitis by the free. In one patient a localized serous detachment in the macular region was associated with retinal infiltrates and in the second patient an atypical unilateral macular chorioretinitis was first diagnosed. Based on previous studies of subacute sclerosing panencephalitis suggesting a preserved th2 response and a defect of the th1 response in patients with sspe at the time of initial measles infection, inoue et al. The boy had most likely contracted wildtype measles in thailand.
Subacute sclerosing panencephalitiscausessymptomstreatment. Subacute sclerosing panencephalitis is a chronic progressive disease of the central nervous system in children and young adults, which produces an intracellular infection of the brain, retina, and lymphoid tissue. High risk of subacute sclerosing panencephalitis following. Table ii ophthalmological and ophthalmoscopic findings in subacute sclerosing panencephalitis which suggest an ocularproblem i. Subacute sclerosing panencephalitis postgraduate medical journal. Subacute sclerosing panencephalitis genetic and rare. Subacute sclerosing panencephalitis sspe diagnosis guide. Sspe affects the central nervous system and is a viral infection that can be caused by a malfunctioning measles virus. Subacute sclerosing panencephalitis mortality, united.
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