Incidence Of Subacute Sclerosing Panencephalitis Research Articles

Sub-Acute Sclerosing Panencephalitis Manifesting as Psychiatric Illness: A Case Report

Subacute sclerosing panencephalitis (SSPE) is a chronic progressive inflammatory central nervous system disorder manifesting as a rare complication of measles viral infection in childhood resulting in fatality in all cases. It is likely to have possibility of subclinical or undiagnosed measles in early childhood in absence of prior overt measles infection. Measles vaccine has a protective effect against SSPE and high vaccination gap is associated with higher incidence of SSPE. Children residing in areas with poor vaccination coverage are at increased risk of developing the disease. It is characterised by progressive intellectual deterioration, seizures, myoclonus, ataxia and visual disturbances. The onset of SSPE is insidious and can manifest as significant psychiatric ailments. A 17 years old girl with history of progressive scholastic deterioration for a year presented with focal seizures, myoclonic jerks and psychiatric manifestations like emotional outburst followed by progressive unresponsiveness, altered sensorium and memory lapses since two weeks. A diagnosis of Subacute sclerosing panencephalitis (SSPE) was made on the basis of clinical symptoms, typical EEG changes and presence of anti-measles antibody in cerebrospinal fluid. Patient received treatment for myoclonic jerks, spasticity along with supportive care. SSPE is often a chronic inflammatory and life threatening disease of central nervous system with no cure despite development of antiviral and immunomodulator drugs. There is urgent need to advocate policies to enhance vaccination coverage and for extensive research in field for potential therapies for treatment of such patients to improve clinical outcomes.

A recent surge of fulminant and early onset subacute sclerosing panencephalitis (SSPE) in the United Kingdom: An emergence in a time of measles.

Subacute Sclerosing Panencephalitis (SSPE) is a fatal progressive neurological disorder following measles infection. Cases were collated from Paediatric Neurology centres in the UK over 24 months from 2017 to 2019 and represent all cases referred to the National Viral Reference Department (VRD). Diagnosis was established with detection of a raised measles index, demonstrating intrathecal measles antibody production. Six children presented with SSPE over two years, with median age five years (range 2-7 years) and median latency period three years (range 2-6 years). The majority were exposed to measles during infancy. Atypical features were common, including visual impairment, focal and generalised tonic-clonic seizures, headache, vomiting and movement disorders. EEG demonstrated typical features in five cases, though not always at presentation. Initial MRI was normal in four cases, with two showing focal and widespread white matter changes. Antiviral and immunomodulatory treatment led to minimal or no improvement. All progressed to cognitive regression, seizures and neurological decline within six months. These cases demonstrate the highest incidence of SSPE in the UK since 2000, all progressing to acute fulminant disease, following younger age of onset, short latency period and atypical presentations. Recent global surges in measles cases raise the importance of clinician awareness of SSPE as a potential diagnosis in children with neurological regression. Herd immunity remains the key protective mechanism for infants and groups that cannot be vaccinated. Health care providers, educators and governments must ensure resources continue to target effective education and access to immunisation programmes, the only means to combat this devastating and fatal condition.

Diagnostic Approaches and Management of Subacute Sclerosing Panencephalitis in Children

Abstract: Subacute Sclerosing Panencephalitis (SSPE) is a progressive neurodegenerative disease that attacks the central nervous system, especially in the population of children and early adolescents, due to persistent measles virus infection. The incidence of SSPE is quite rare, and data shows that in developing countries it is still quite high compared to developed countries. Diagnosis is based on clinical, supporting examinations such as EEG, as well as increased antibodies against measles virus in serum and cerebrospinal fluid. Symptoms can include changes in behavior, myoclonus, memory problems, and persistent pyramidal or extrapyramidal movements. Management to date has not provided satisfactory results and is individualized. Most SSPE patients experience a progressive and gradual course, leading to death within 1-3 years. The challenge of SSPE in children is the approach to diagnosis and management. Until now, the approach to treating SSPE in children is still based on the pathophysiological mechanisms from several existing research studies. That why therapy guidelines for children with SSPE are still varied. This paper aims to discuss the topic of SSPE in children with a major focus on diagnostic and therapeutic approaches based on the latest scientific evidence. Keywords: Subacute Sclerosing Panencephalitis (SSPE), children, diagnosis, therapy

Subacute Sclerosing Panencephalitis Causing Rapidly Progressive Dementia and Myoclonic Jerks in a Sexagenarian Woman

<strong>Background:</strong> Subacute sclerosing panencephalitis (SSPE) is a disease of childhood and adolescence, but can affect adults. Rapidly progressive cognitive decline, seizures including myoclonic jerks, spasticity, ataxia, visual disturbances, and incontinence are typical manifestations. <strong>Case report:</strong> A 62-year-old woman who presented with rapidly progressive dementia and myoclonus was diagnosed with SSPE. There was resolution of the movement disorder with clonazepam and valproic acid treatment and some amelioration of cognitive decline after 3 months of therapy with interferon alfa and isoprinosine. <strong>Discussion:</strong> With the recent rise in measles cases worldwide, any increased incidence of SSPE would require vigilance for early interventions.

Subacute sclerosing panencephalitis.

Subacute sclerosing panencephalitis (SSPE) is a slowly progressive brain disorder caused by mutant measles virus. SSPE affects younger age groups. SSPE incidence is proportional to that of measles. High-income countries have seen substantial decline in SSPE incidence following universal vaccination against measles. SSPE virus differs from wild measles virus. Measles virus genome recovered from the autopsied brain tissues demonstrates clustered mutations in virus genome particularly in the M gene. These mutations destroy the structure and functioning of the encoded proteins. Complete infectious virus particle has rarely been recovered from the brain. Human neurons lack required receptor for entry of measles virus inside the neurons. Recent in vitro studies suggest that mutations in F protein confer hyperfusogenic properties to measles virus facilitating transneuronal viral spread. The inflammatory response in the brain leads to extensive tissue damage. Clinically, SSPE is characterized by florid panencephalitis. Clinically, SSPE is characterized by cognitive decline, periodic myoclonus, gait abnormalities, vision loss, and ultimately to a vegetative state. Chorioretinitis is a common ocular abnormality. Electroencephalography (EEG) shows characteristic periodic discharges. Neuroimaging demonstrates periventricular white matter signal abnormalities. 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.

Interferon alpha in subacute sclerosing panencephalitis: Case report and review of the literature

Subacute Sclerosing Panencephalitis (SSPE) is a rare, rapidly progressive neurodegenerative disease caused by the measles virus. Spontaneous remission and survival longer than 4 years is rare. The disease commonly affects children and adolescents from less privileged socio-economic backgrounds. Prior to immunisation, the incidence of SSPE in South Africa (SA) was 2.6/100 000 people per year, which is higher than the global estimate of 1/1 000 000 per year. The post-immunisation incidence has dropped to 0.43/100 000 people per year. Currently, there is no curative treatment for SSPE. In developed countries, patients have been treated with antiviral therapy and interferon alpha (IFNα) with promising results. There have been no reported studies of children with SSPE treated with IFNα in South Africa. We report on a patient successfully treated with intraventricular INFα.

Interferon alpha in subacute sclerosing panencephalitis: Case report and review of the literature

Subacute Sclerosing Panencephalitis (SSPE) is a rare, rapidly progressive neurodegenerative disease caused by the measles virus. Spontaneous remission and survival longer than 4 years is rare. The disease commonly affects children and adolescents from less privileged socio-economic backgrounds. Prior to immunisation, the incidence of SSPE in South Africa (SA) was 2.6/100 000 people per year, which is higher than the global estimate of 1/1 000 000 per year. The post-immunisation incidence has dropped to 0.43/100 000 people per year. Currently, there is no curative treatment for SSPE. In developed countries, patients have been treated with antiviral therapy and interferon alpha (IFNα) with promising results. There have been no reported studies of children with SSPE treated with IFNα in South Africa. We report on a patient successfully treated with intraventricular INFα.

Fulminant Subacute Sclerosing Panencephalitis in an Individual With a Perinatally Acquired Human Immunodeficiency Virus Infection

BACKGROUND Case reports of subacute sclerosing panencephalitis (SSPE) in individuals with human immunodeficiency virus (HIV) infection are scarce, and the natural history is unclear. To our knowledge, a fulminant presentation has not yet been described. OBJECTIVE To describe a case of fulminant SSPE in an individual with a perinatally acquired HIV infection. DESIGN Case report and literature review. SETTING Christian Medical College Hospital, Vellore, India. PATIENT A 17-year-old boy with a perinatally acquired HIV infection. RESULTS The patient presented with subacute-onset cognitive decline and myoclonic jerks with rapid deterioration of health (the patient died within 12 weeks of onset). The findings from magnetic resonance imaging and electroencephalography and the cerebrospinal fluid and serum measles antibody titers were suggestive of SSPE. The fulminant presentation in this case needs to be noted. CONCLUSIONS Along with the better life expectancy of HIV-infected individuals, there may be an increase in the incidence of SSPE in this population. Fulminant SSPE may be added to the spectrum of measles-associated neurological disorders in HIV.

Subacute sclerosing panencephalitis in Italy. An epidemiological study.

An epidemiological inquiry in Italy led to the collection of 207 cases of subacute sclerosing panencephalitis (SSPE) with onset during the decade 1972-1981, with a mean incidence rate of 0.37 cases per year per million total population (CYMTP) and 1.24 cases per year per million young population aged 0-19 years (CYMYP). A striking difference was found among the 20 Italian regions, with a minimum of no cases in three regions, and a maximum incidence of 2.46 CYMTP (6.67 CYMYP) in Sardinia. The male/female ratio was 1.8/1. The mean age at SSPE onset was 10.6 (+/- 4.0) years in Italy, and was significantly lower in Sardinia (8.8 +/- 2.8). The mean age of measles was 2.8 +/- 1.7 years (in 106 cases), significantly lower than the mean age of measles in the general population. Moreover, the age of measles was significantly lower in males than in females, and this could represent a factor contributing to the higher SSPE incidence in males. The interval between measles and SSPE onset was significantly lower in Sardinia (mean 5.6 +/- 2.5 years). In Italy, as in other Mediterranean countries, SSPE incidence is higher than expected and unevenly distributed, suggesting that environmental factors play a role.

Review of the effect of measles vaccination on the epidemiology of SSPE

When measles vaccines were widely introduced in the 1970s, there were concerns that they might cause subacute sclerosing panencephalitis (SSPE): a very rare, late-onset, neurological complication of natural measles infection. Therefore, SSPE registries and routine measles immunization were established in many countries concurrently. We conducted a comprehensive review of the impact of measles immunization on the epidemiology of SSPE and examined epidemiological evidence on whether there was any vaccine-associated risk. Published epidemiological data on SSPE, national SSPE incidence, measles incidence and vaccine coverage, reports of SSPE in pregnancy or shortly post partum were reviewed. Potential adverse relationships between measles vaccines and SSPE were examined using available data. Epidemiological data showed that successful measles immunization programmes protect against SSPE and, consistent with virological data, that measles vaccine virus does not cause SSPE. Measles vaccine does not: accelerate the course of SSPE; trigger SSPE or cause SSPE in those with an established benign persistent wild measles infection. Evidence points to wild virus causing SSPE in cases which have been immunized and have had no known natural measles infection. Perinatal measles infection may result in SSPE with a short onset latency and fulminant course. Such cases are very rare. SSPE during pregnancy appears to be fulminant. Infants born to mothers with SSPE have not been subsequently diagnosed with SSPE themselves. Successful measles vaccination programmes directly and indirectly protect the population against SSPE and have the potential to eliminate SSPE through the elimination of measles. Epidemiological and virological data suggest that measles vaccine does not cause SSPE.

Analysis of serum and cerebrospinal fluid cytokine levels in subacute sclerosing panencephalitis in Papua New Guinea

Background Subacute sclerosing panencephalitis (SSPE) is a rare progressive inflammatory disease characterized by the persistent infection of the brain by the measles virus. However, the immunological pathophysiology of SSPE is still unclear. Methods We measured the concentrations of interferon-γ (IFN-γ), tumor necrosis factor-α (TNF-α), interleukin-2 (IL-2), IL-4, IL-6, IL-10, and soluble TNF receptor 1 (sTNFR1) in the serum and cerebrospinal fluid (CSF) of 23 patients with SSPE in Papua New Guinea (PNG), a country with a high incidence of SSPE, and Japanese controls by cytometric bead array or ELISA. Results The serum IL-6 and IL-10 levels of SSPE patients were significantly higher than those of controls ( p = 0.0075, and p = 0.0019, respectively). The serum IL-6 and IL-10 levels of SSPE patients with fever were significantly higher than those without fever ( p = 0.0107, and p = 0.0006, respectively). The CSF IL-6 levels of SSPE patients were significantly higher than those of controls ( p = 0.0218). The CSF IL-6 levels of SSPE patients with myoclonic jerks were significantly higher than those without myoclonic jerks ( p = 0.0189). There were no differences in serum IFN-γ, TNF-α, IL-2, IL-4, and sTNFR1, or CSF IFN-γ, TNF-α, IL-2, IL-4, IL-10, and sTNFR1 levels between the affected patients and controls. Conclusion Our present study suggests that serum IL-6 and IL-10 levels are related to fever, and the CSF IL-6 level, myoclonic jerks, in SSPE patients in PNG.

Subacute sclerosing panencephalitis surveillance study in Istanbul

The exact incidence rate of subacute sclerosing panencephalitis (SSPE) in Turkey (and in Istanbul) is not known. We have conducted an active surveillance study to determine the epidemiological characteristics and the incidence rate of SSPE in Istanbul between the dates July 1, 2002 and July 1, 2004. We found that the incidence of SSPE in Istanbul is 2 per million. By logistic regression analysis, risk factors in SSPE development are determined as being at younger ages (OR: 1.199, 95%CI=1.047–1.372, P=0.009), living in crowded households (OR: 1.430, 95%CI=1.039–1.968, P=0.028), low education level of the mother (OR: 0.123, 95%CI=0.034–0.447, P=0.001), low household income (OR: 0.413, 95%CI=0.234–0.728, P=0.002), infant's being born out of Marmara region (Istanbul is in Marmara region of Turkey) (OR: 0.358, 95%CI: 0.172–0.746, P=0.006), infant's not being vaccinated against measles (OR: 0.495, 95%CI: 0.312–0.786), infant's having had measles before (OR: 0.235, 95%CI: 0.135–0.411). As a result, it is found in this study that SSPE is mostly related to having measles infection, and measles vaccination is found to be highly protective against SSPE. This is the first epidemiological study in SSPE from Turkey that conveys the incidence rate in Istanbul.

Subacute Sclerosing Panencephalitis in Bulgaria (1978–2002)

The epidemiology of subacute sclerosing panencephalitis (SSPE) has changed substantially since the introduction of measles vaccine. We studied the incidence of SSPE in Bulgaria based on cases admitted to the Child Neurology Clinic, University Hospital of Neurology and Psychiatry, Sofia, for a 25-year period (1978–2002). The SSPE incidence prior to and during the period of routine measles immunization was analyzed. SSPE was diagnosed in 40 children (29 males and 11 females, mean age 8.5 years), 28 from 1978 to 1984 (average 4 patients/year), and 12 from 1995 to 2002 (average 1.7 patients/year). Thirty-eight cases (95%) were non-immunized and had early measles infection. Age at onset of SSPE ranged from 8 to 11 years (52.5%) with a mean latent period of 7 years following measles infection. The increase in SSPE incidence (1995–2002) following a 10-year disease-free period (1985–1994) appears to be related to early measles infection (mean age 11 months) during the measles epidemic of 1991–1992. During the period 1995–2002, children had earlier measles infection (average 11 months) and earlier onset of SSPE (mean age 8.4 years) than in the period 1978–1984 (mean age at measles infection 18 months, and of SSPE onset 11.2 years). The SSPE incidence in Bulgaria during the 25-year period from 1978 to 2002 confirms the importance of early measles infection as a risk factor for SSPE, and the role of routine measles immunization in SSPE prevention.

Clinical and epidemiological characteristics of subacute sclerosing panencephalitis in Bulgaria during the past 25 years (1978–2002)

The aims of the present study are to establish the subacute sclerosing panencephalitis (SSPE) incidence in Bulgaria for the 25-year period 1978–2002; to analyze the SSPE incidence prior to, and in the period of, routine measles immunization; and, to analyze the clinical characteristics of SSPE. SSPE was diagnosed in a total of 40 children; 28 of were diagnosed between 1978 and 1984, and 12 between 1995 and 2002. Thirty-eight cases (95%) were non-immunized and have had an early measles infection (mean age 16 months). The SSPE onset occurred primarily between 8 and 11 years of age (52.5%) with a mean latent period of about 7 years after the measles infection. After the 10-year disease-free period (1985–1994), the SSPE incidence increased between 1995 and 2002 because of the 1991–1992 measles epidemic. During the period 1995–2002 children with earlier measles infection and earlier SSPE onset predominated, compared to the period 1978–1984. The initial clinical manifestations included intellectual deterioration in 35%, extrapyramidal hyperkinesias in 29%, epileptic seizures in 15%, hemiparesis in 10%, and visual disturbances in 10% of the cases. Nine children (22.5%) demonstrated an atypical onset. A rapidly progressive course was observed in 4 children (10%) and a chronic progressive course with pseudo-remissions over 2 years-in 8 cases (20%). Our analysis of the SSPE incidence in Bulgaria for the 25-year period (1978–2002) supports the importance of early measles infection as a crucial risk factor for this persistent neuroinfection. Moreover, it confirms the role of routine measles immunization in SSPE prevention.

A continuing high incidence of subacute sclerosing panencephalitis (SSPE) in the Eastern Highlands of Papua New Guinea.

The aims of this descriptive study were to confirm the high incidence of subacute sclerosing panencephalitis (SSPE) previously reported from Papua New Guinea (PNG) and to relate SSPE to previous measles vaccination and measles illness. From February 1997 to April 1999 we diagnosed a total of 55 patients with SSPE at Goroka Base General Hospital in Eastern Highlands Province (EHP) of PNG. The diagnosis was based on high cerebrospinal fluid and serum measles virus antibody titres with progressive neurological disorder and myoclonic jerks. Of these 55 patients 42 were from EHP, including 32 whose onset was in the 2-year period 1997-1998. The annual incidence of SSPE in EHP in these 2 years was 98 per million population under 20 years of age, the highest ever reported. This incidence was more than ten times higher than the highest incidence in the prevaccine era reported from elsewhere. The mean age of onset of SSPE was 7.7 years (range 2.8-14.8 years) and the interval between measles and the onset of SSPE, where known, had a mean of 5.9 years and a range of 2.5-11.1 years. Among the SSPE patients 19 had a documented history of measles vaccination. Eight of these 19 also had documentation of previous measles illness; of these, seven were vaccinated after the development of measles and one was vaccinated 20 days before measles illness. Two non-SSPE children received vaccination twice which was documented and subsequently developed measles which was also substantiated by documentation. Two patients with SSPE yielded amplified nucleotide sequences of measles virus that were different from any of the vaccine strains. We found no evidence to implicate measles vaccination in the development of SSPE.

Panencefalitis esclerosante subaguda: una enfermedad que todavía existe

Subacute sclerosing panencephalitis (SSPE) is a rare entity with an invariably fatal course that progressively affects the central nervous system. It is caused by persistent infection with the wild-type measles virus. While rare in industrial countries, it is not infrequent in developing countries, where there are still areas of endemic measles infection and immunization is not yet generalized. We describe an eight-year-old Spanish girl who presented rhythmic and symmetric myoclonus. She contracted measles at 13 months and required hospitalization. No cognitive deterioration was found. Neuroimaging and the initial electroencephalogram were normal. Oligoclonal bands and high titers of measles antibodies were found in serum and cerebrospinal fluid. She was treated with oral metisoprinol and intraventricular alpha-interferon (IFN-) and showed no further progression of her symptoms. The importance of including SSPE in the differential diagnosis of patients consulting for school failure, neurological deterioration or movement disorders is highlighted. Special attention should be paid to the immigrant population from countries where the incidence of SSPE is greater than in Spain.

Neuroprogressive disease of post-infectious origin: a review of a resurging subacute sclerosing panencephalitis (SSPE).

Subacute sclerosing panencephalitis (SSPE) is a progressive, essentially untreatable, disease of the nervous system. When first described in the 20th Century, it was characterized more for its neuropathological features than for its pathophysiology or cause. It was not until the 1960s that a clear relationship to the measles virus was established. It is now thought that this uncommon infectious encephalopathy is caused by a "slow," altered or persistent form of the wild measles virus which has harbored in the nervous system for years. Then a "breakout" occurs and the more lytic and virulent organisms produce the progressive and spreading inflammatory and destructive lesions which are confined to the nervous system. Epidemiological study of the disease confirms its relationship to measles. In the years since the development of national measles immunization programs, there has been a dramatic decline in the incidence of measles exanthem and until recently a corresponding decline in the incidence of SSPE. In recent years there has been a mild to moderate increase in cases of SSPE as reported to the USA/International SSPE Registry. As yet, there has not been a totally effective treatment. The purpose of this paper is to give an overall review on SSPE and its relationship to measles. This review will include a prospectus of its history, considerations as to its etiology, correlation of clinicopathological features, and thoughts on the past and present epidemiology and treatment.

Subacute sclerosing panencephalitis in wales

Twenty cases of subacute sclerosing panencephalitis (SSPE) occurring over an 18-year period in Wales are described and used as the basis for a comparison of measles infection, vaccination rates and the incidence of SSPE in England and Wales. Rates of measles infection were higher in Wales in all age groups, and fewer Welsh children were vaccinated, which maintained a high risk of SSPE per case of measles. Following vaccination, there was a more pronounced change in the age distribution of measles infection in Wales than in England, and it is proposed that one contribution to the high frequency of SSPE in the 1980s was the reservoir of measles in unvaccinated 2-4-year-olds, acting as a source of infection for children aged < 2 years, in whom the risk of SSPE following measles is known to be higher than in other groups.

Observations of subacute sclerosing panencephalitis in South Africa

Analysis of 75 cases of subacute sclerosing panencephalitis (SSPE) reported to the National Institute for Virology, South Africa, in 1984–1990 does not support the role of intensive exposure to measles virus in the pathogenesis of SSPE. The incidence of SSPE per million population was similar in Blacks and Whites, although that of reported measles is up to 10 times greater in Blacks. The age of SSPE follows the distribution of measles cases; thus, significantly more younger SSPE cases were found in Blacks than in Whites. The distribution between males and females was approximately equal. These data suggest SSPE to be a fortuitous complication of measles infection associated with as yet unidentified risk factors rather than a consequence of an excessive dose of infecting virus or immunological immaturity.

Subacute sclerosing panencephalitis (SSPE) in Papua New Guinea: a high incidence in young children.

Eighty-seven cases of subacute sclerosing panencephalitis (SSPE) were diagnosed from September 1988 to April 1991 in Papua New Guinea (PNG), by demonstration of high-titre measles-specific antibodies in cerebrospinal fluid (CSF). For 1990 the annual incidence of SSPE, for the study provinces, was calculated to be 56 cases per million under 20 years of age and it is expected that this figure will be higher in 1991. The mean age of presentation was 4.9 years, with a male to female ratio of 1.8:1. An elevation in the ratio of immunoglobulin G as a percentage of total protein in CSF and an increase in the CSF:serum immunoglobulin G ratio was shown in SSPE patients. The dramatic appearance and high frequency of the disease in PNG might relate to the early age of measles infection encountered in children in this country.