Indirect, evidence has suggested that subacute sclerosing panencephalitis sspe, dawsons encephalitis, van bogaerts leukoencephalitishardened inflammation of the brainis associated with. Comparative studies between two measles virus strains isolated from patients with subacute sclerosing panencephalitis sspe and a prototype low tissue culture passage edmonston measles virus are described. Objective to determine the clinical and demographic characteristics of children diagnosed with subacute sclerosing panencephalitis sspe. The diagnosis of sspe is based on characteristic clinical and eeg findings periodic complexes and demonstration of elevated antibody titres against measles in cerebrospinal fluid. Fulminant subacute sclerosing panencephalitis mimicking. Subacute sclerosing panencephalitis, seminars in pediatric. Attempts to transmit subacute sclerosing panencephalitis and isolate a measles related agent, with a study of the immune response in patients and experimental animals. Measles virus mv inserts one guanosine g residue at a specific site in a subpopulation of the mrna transcribed from the phosphoprotein p gene to. Subacute sclerosing panencephalitis sspe is a chronic encephalitis occurring after infection with measles virus.
Long survival in subacute sclerosing panencephalitis. Unusual symptoms, especially in the early periods of disease, included hemiparesis 7 patients, headache 3, generalized tonicclonic seizures 6, absence seizure 1, nausea 3, and vomiting 3. Subacute sclerosing panencephalitis sspe is a progressive neurological disorder of children and young adults that affects the central nervous system cns. Early and latestate subacute sclerosing panencephalitis. Subacute sclerosing panencephalitis with bilateral. Subacute sclerosing panencephalitis sspe is now considered to be caused by measles virus.
Subacute sclerosing panencephalitis sspe is so rare in developed countries with measles immunization programs that national active surveillance is now needed to capture sufficient number of cases for meaningful analysis of data. Clinical and laboratory characteristics of 39 patients with adult onset subacute sclerosing panencephalitis sspe are described and compared to those of juvenile onset patients regarding preceding measles, age at onset, gender, interval between onset and diagnosis, clinical profile, and course during follow up. Certain atypical features can occur at onset or during the course of illness which can be misleading. Subacute sclerosing panencephalitis was diagnosed in a 10yearold girl who had suffered from atypical absence attacks for 3 months. Subacute sclerosing panencephalitis sspe is a rare chronic, progressive encephalitis affecting primarily children and young adults, caused by a persistent infection of immune resistant measles virus. Mri findings in subacute sclerosing panencephalitis. A recent report on acute encephalitis associated with measles included diffusion mr findings. 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. The devastating measles complication is more common than we think. 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. Subacute sclerosing panencephalitis sspe is a rare progressive neuroinfectious disease due to a late complication of the measles virus. Subacute sclerosing panencephalitis information page.
Normal responses of measles specific immunoglobulins m and g igm and igg were defined in 10 children with measles. It has been estimated that about 1 in 10,000 people who get measles will eventually develop sspe. Lesions of high signal intensity on t 2weighted images are the most common finding. Most case reports of subacute sclerosing panencephalitis are described in nonvaccinated patients. Diagnosis was based on clinical and electroencephalographic findings and. Subacute sclerosis panencephalitis sspe is a persistent and chronic encephalitis secondary to measles virus infection that causes widespread demyelination of the central nervous system cns. Subacute sclerosing panencephalitis and lymphocytes nejm. Subacute sclerosing panencephalitis subacute sclerosing panencephalitis gascon, generoso g. Classical sspe, first described by dawson in 1934, is caused by latent measles virus 68 years after the primary measles infection. Later, in 1945, van bogaert 3 described another individual with the same clinical presentation but. Subacute sclerosing panencephalitis and lymphocytes. Subacute sclerosing panencephalitis sspe is caused by a measles infection that is acquired earlier in life often 710 years prior to the onset of sspe symptoms. The condition primarily affects children, teens, and young adults. It is a slow, but persistent, viral infection caused by defective measles virus.
Histories obtained in 350 of 375 clinically confirmed cases of subacute sclerosing panencephalitis sspe reported to a national registry showed that 292 patients had measles and 58 had no history of measles. Three children with subacute sclerosing panencephalitis sspe were treated with alphainterferon. The findings described in this report suggest that strains of measles virus associated with sspe have different biological properties and. Subacute sclerosing panencephalitis sspe a rare condition that is caused by a measles infection acquired earlier in life. Abnormal responses of measles igm and igg were found in both sera and cerebrospinal fluids from three cases of subacute sclerosing panencephalitis.
Evolution of certain typical and atypical features in a. Medication was administered via an ommaya reservoir into the cerebral ventricular system over a period of to 24 months in increasing amounts up to 1. Register of cases of subacute sclerosing panencephalitis. However, a 2016 study estimated that the rate for babies who contracted measles was as high as 1 in 609. Subacute sclerosing panencephalitis sspe is a devastating, progressive, destructive process of the central nervous system caused by the mutant measles virus infection of neurons. 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. Mr imaging findings have been described in the disease 14, and several proton mr spectroscopy reports exist in the literature 57. This disease develops following infection with a rubeola virus that has mutated. We collected data of patients with sspe during 20042010 who. Who subacute sclerosing panencephalitis and measles. The epidemiology of subacute sclerosing panencephalitis in.
Thirtyfour mri studies of 26 patients with subacute sclerosing panencephalitis are reported. Pdf subacute sclerosing panencephalitis measles virus. L of rnase free water, and the rna concentration was determined by ultraviolet spectroscopy rtpcr and sequence analysis to prepare templates for sequencing, rtpcr was performed using the. Its incidence will increase in the usa, not only in immigrants, but also because of the.
Affected people may initially experience behavioral changes, dementia, and disturbances in motor. We present a case of a fully vaccinated 3yearold boy who was diagnosed with and treated for autoimmune encephalitis before arriving at a diagnosis of sspe. One such complication, subacute sclerosing panencephalitis sspe, is a devastating, progressive, central nervous system destructive process caused by mutant measles virus infection of neurons. Subacute sclerosing panencephalitis genetic and rare. Characteristic eeg findings included diffuse spike and wave complexes of about 2. In patients with a history of measles, measles illness occurred before age 2 years in 46%, and a mean of. Subacute sclerosing panencephalitis pubmed central pmc. In resourceconstrained countries, in such patients, subacute sclerosing panencephalitis should be considered before doing expensive autoimmune antibody profile. Brain biopsies or postmortem histopathological examination show evidence of astrogliosis, neuronal loss, degeneration of dendrites, demyelination, neurofibrillary tangles, and.
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 sspe is a progressive fatal disease of the central nervous system. Subacute sclerosing panencephalitis is a lethal complication of measles that is not as rare as previously believed. Place and duration of study the aga khan university hospital, karachi, from january 2000 to june 2012. Epidemiologic studies of measles, measles vaccine, and. Pdf subacute sclerosing panencephalitis researchgate.
The neurovirulence of two wild type wt and seven subacute sclerosing panencephalitis sspe measles virus strains was tested in young adult ferrets by intracerebral ic inoculation of. 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 is a rare, progressive, inflammatory neurodegenerative disease. Intraventricular interferon treatment for subacute.
The mean of the duration from the infection to the onset of subacute sclerosing panencephalitis was 9 years. The hallmark clinical features of this disease include. 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. Subacute sclerosing panencephalitis sspe is a progressive neurological disorder of childhood and early adolescence. 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. Usually fatal, it develops 2 to 10 years after the patient recovers from rubeola.
However, early onset of sspe with a fulminant course has been described in children. Cureus subacute sclerosing panencephalitis in a child. The patient, aged 14 years, had reportedly been vaccinated against measles in the philippines at age 8 months. The aim of the present study is to describe the clinical profile and natural history of patients with sspe.
An 8yearold boy presented with progressive behavioral changes, cognitive decline and myoclonic jerks, progressing to a bed bound state over 2 months. We discuss the challenges of diagnosing sspe in developed countries. Subacute sclerosing panencephalitis findings at mr imaging. Clinical features at onset are very subtle and nonspecific. Typically, affected persons have a history of measles infection in infancy period 1. Subacute sclerosing panencephalitis sspe is a fatal complication of measles infection. Subacute sclerosing panencephalitis is a progressive neurologic disorder that is considered to be caused by persistent measles virus. Sspe can have atypical clinical features at the onset. Subacute sclerosing panencephalitis sspe is a slowly progressive inflammatory disease of the central nervous system caused by a persistent measles virus usually affecting the childhood and adolescent age group. The prevalence of the disease varies depending on uptake of measles vaccination, with the virus disproportionally affecting regions with low vaccination rates. Brain biopsies or postmortem histopathological examination show evidence of astrogliosis. Subacute sclerosing panencephalitis sspe is a progressively fatal degenerative disease of the central nervous system.
High incidence of subacute sclerosing panencephalitis in. A devastating complication of measles infection, subacute sclerosing panencephalitis sspe is also called dawson encephalitis. Subacute sclerosing panencephalitis postgraduate medical. Brain biopsies or postmortem histopathological examination show evidence of astrogliosis, neuronal loss, degeneration of dendrites, demyelination, neurofibrillary tangles, and infiltration of inflammatory cells. A total of 47 cases were identified, 31 male and 16 female. Magnetic resonance imaging fluidattenuated inversion recovery flair images show hyperintensity in left hippocampus, parahippocampal gyrus, and medial temporal lobe. Subacute sclerosing panencephalitis sspe is a slowly progressing inflammatory and degenerative disorder of the brain caused by a mutant measles virus. To assess the impact of measlesmumpsrubella mmr vaccine on the epidemiology of subacute sclerosing panencephalitis sspe in england and wales. It is caused by persistent defective measles virus. Sspe has been reported from all parts of the world, but it is considered a rare disease in developed. There was an average annual decline of 14% in sspe. 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.
Subacute sclerosing panencephalitis sspe is chronic encephalitis occurring after infection with measles virus. It is a slow virus infection caused by a genetically altered form of the. Clinical, electroencephalographic, and other laboratory features and subacute sclerosing panencephalitis sspe were studied in eight patients by the same group of investigators. Expression and properties of the v protein in acute. There are four diagnostic criteria, namely the clinical picture, a characteristic eeg, serology of serum and csf and brain histology. An insight into the diagnostic errors from a tertiary care university hospital. Forty of the latter patients received live, attenuated measles virus vaccine. The relatively high frequency of the disease within a small geographical area in a short period of time suggests that.
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