Onset Of Subacute Sclerosing Panencephalitis Research Articles

Sub-Acute Sclerosing Panencephalitis at University Hospital of Constantine

Introduction Subacute sclerosing panencephalitis (SSPE) is a serious, late complication of measles, and the prognosis remains poor in the absence of effective treatment, underscoring the role of vaccination in its prevention. Objectives We describe the clinicobiological and evolutionary profile of 16 patients recruited from the B chu de Constantine pediatric department. Patients and methods Study of16 cases: epidemiological, clinical, EEG, biological and evolutionary profile. Results16 cases of SSPE were collected, 75% were male, 70% were aged between 24 and 59 months, almost all patients had had measles, 98% within the first 24 months of life, 93% had not been vaccinated against measles, SSPE occurred in more than 50% of cases between 24 and 48 months after measles, all had received treatment (100% corticosteroids and immunoglobulins), 87% antiepileptics and 25% rituximab, the course was characterized by stabilization: 27%, death: 33% deterioration:40% of cases. Comments and discussion SSPE is a rare complication occurring a few years after recovery from measles Almost all our patients had not been vaccinated against measles. in our patients, the median age at onset of SSPE was 4.5 years This earlier onset of SSPE in recent studies than reported in older studies suggests early onset of measles, viral mutation or a fragile immune system. Almost all patients were unvaccinated against measles; 40% of patients died, 31% worsened (stage IV) and 37% stabilized. Conclusion Given the poor prognosis of SSPE, the best way to control this disease is to vaccinate the target population against measles.

A Comprehensive Investigation of the Current Subacute Sclerosing Panencephalitis (SSPE) Treatment Options to Improve Patient Quality of Life.

Subacute sclerosing panencephalitis (SSPE) is a progressive, disabling, and deadly neurological disorder related to measles (rubeola) infection occurring primarily in children. The slow but persistent viral infection occurs in children or young adults and affects their central nervous system (CNS). There have been plenty of reports on SSPE throughout the world, but it is considered a rare disease in developed countries. This research focuses on comparing the current treatments available to prolong the life of patients for over three years after the onset of SSPE. The goal was to identify possible patterns or trends among the treatments in order to find the best possible method to lengthen a patient's life. The results indicated that interferon alpha, inosine pranobex, and ribavirin display the most effective treatment plan and indicate the most potential in discovering a more effective therapeutic for SSPE.

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.

Subacute sclerosing pan encephalitis in a tertiary care centre in North India

Background: Subacute sclerosing pan encephalitis (SSPE) is caused by persistent, aberrant measles virus infection presenting as rapid neurocognitive decline, associated with myoclonus. Diagnosis is by distinctive clinical presentation, characteristic EEG changes and measles serology. Methods and materials: Retrospective study from a tertiary care hospital SGPGI, Lucknow – a total of non-replicate 89 paired CSF and blood samples from patients presenting to OPD with clinical features suggestive of SSPE (i.e. dementia, myoclonic jerks and/or ataxia in a previously healthy individual) were analyzed from 2015 to 2018. Total IgG and measles-specific IgG levels for CSF and value of relative CSF/serum quotient ≥ 1.5 was accepted indicative for intrathecal measles antibody synthesis. History of previous attack of measles, immunization status, EEG pattern were recorded. Results: Presenting symptoms included myoclonus, behavioural changes, seizures, and cognitive, visual, and extrapyramidal disturbance. A total of 52.08% (47/89) patients had a high cerebrospinal fluid: serum anti-measles antibody ratio showing male preponderance. Age at onset of SSPE ranged from 6 years to 34 years, showing an increase in mean age at onset of SSPE. A sizable percentage of the patients were ≥18 years old and considered to have adult onset SSPE. A definitive history of measles could be elicited in 20% cases. Previous complete immunization history was present in about 40% and partial could be elicited in 35%. Characteristic EEG findings were present in about 80%. All patients received symptomatic therapy; 20% also received disease modifying agents with poor compliance. Demographic details showed more frequent clustering in some neighboring districts of UP. Conclusion: WHO and Indian association of Pediatrics recommends two doses of vaccine. High SSPE cases could be attributed either to referral bias, decrease in the potency of the vaccine at the receiving end/inefficient cold chain system, improper vaccine coverage, poor quality of vaccine and possible circulation of atypical measles virus strain cannot be ruled out.

Subacute Sclerosing Panencephalltis: Clinical and Laboratory Manifestations

We reviewed clinical and laboratory findings of 12 cases of Subacute sclerosing panencephalitis (SSPE) hospitalized at our department from 1985 to 1991. All cases were diagnosed and hospitalized at the 2nd stage. The principal clinical manifestations were mental changes, myoclonus, and frequent falls. Other clinical manifestations were ocular changes, involuntary movements, loss of social contact, and spasticity. Diagnosis was based on suppression-burst pattern in EEG and positive antibody titer to measles in serum and cerebrospinal fluid. CT scan was not diagnotic, since it was either normal or showed only non-specific cortical atrophy. Eleven patients (91, 7%) recalled a history of measles in the past. Age of onset of SSPE varied among cases and was difficult to specify precisely due to its subtle nature. None of the cases had been vaccinated against measles.
 SSPE is a rare disease, but is almost always fatal with prolonged suffering of the patient. Based on our experience with SSPE patients, we recommend to broaden the immunization programme against measles.

Adult Onset Subacute Sclerosing Panencephalitis: A case report

Subacute sclerosing panencephalitis (SSPE) is a chronic encephalitis of childhood and young adolescence due to persistent measles virus infection of the central nervous system (CNS). In majority of cases, onset occurs between 5-10 years of age. SSPE generally occurs 5-10 years after measles virus infection1. The diagnosis of SSPE is based on characteristic clinical and electroencephalogram (EEG) findings, increase measles antibody titer in cerebrospinal fluid (CSF) and serum. As onset of SSPE in adults is rare and may have atypical feature it requires high index of suspicion for early and accurate diagnosis. Herein, we report a case of SSPE in a male of 26 years with recurrent episodes of myoclonic jerks. Journal of National Institute of Neurosciences Bangladesh, 2016;2(1): 40-42

Epilepsy in children with subacute sclerosing panencephalitis

Subacute sclerosing panencephalitis (SSPE) is a rare, progressive, fatal neurodegenerative disease of childhood and early adolescence caused by defective measles virus. The initial symptoms of SSPE usually involve regression in cognitive functioning and behavior or recurrent myoclonic jerks. Seizures revealing SSPE and epilepsy during the clinical course can occur. The aim of the study was to analyze clinical and EEG characteristics of both initially occurred seizures and epilepsy which developed in the course of the disease. Retrospective study was carried out on 19 children (14 boys, 5 girls) with SSPE diagnosed and treated at our Clinic from 1995 to 2010. Seizures revealed SSPE in our patients aged from 6.5 to 11.5 years (mean 8.6 years). SSPE onset ranged from 4.5 to 16.5 years (mean 10.05). Complete vaccination was performed in nine patients. Cognitive and behavioral decline was preceeded by 6-18 months in two children with intractable focal motor seizures with secondary generalization, one child with complex partial seizures and one with atypical absences. During the clinical course of the disease epilepsy developed in 10 (52.6%) cases, including four patients with seizures as the initial SSPE sign. It occurred mainly in the first year, while in three cases seizures appeared between 1 and 5 years of the disease evolution. Myoclonus was present independently from seizures. No significant inter-group differences were found relating to the type of SSPE progression and history of epilepsy. The only child with fulminant SSPE presented with initial seizures. Favorable seizure control was achieved in 60.0% patients. Intractable epilepsy developed in four patients. Atypical SSPE presentation can include mainly focal intractable seizures. Epilepsy developed during clinical course in 52.6% cases. No significant influence was found of the history of epilepsy on the type of SSPE progression.

Challenges in diagnosing SSPE

The typical clinical presentation of subacute sclerosing panencephalitis (SSPE) includes behavioral and intellectual changes followed by myoclonia. However, there are a considerable number of SSPE cases which present with distinct clinical features that can lead to a diagnostic difficulty. In this report, we summarize the clinical features of patients with SSPE who have uncommon presentations or features of the disease or coexisting medical conditions which may lead to diagnostic difficulties. We studied 173 patients, all under the age of 17. Patients were included in the study group according to following criteria: onset of the disease before age 2years, seizures occurring before the onset of myoclonia and/or behavioral symptoms, extrapyramidal or cerebellar signs and ocular manifestations as initial presenting symptoms, fulminant course including coma or death within 6months. Additionally, patients with onset of SSPE at the setting of a known neurological disorder are defined as another group in the study. Out of 173 patients with SSPE who were followed in two neurology centers, 31 (17.9%) met our criteria. We found a relative high frequency of these clinical features. Our findings suggest that clinicians should be aware of this clinical characteristics and rule out the disease in cases were other common causes have been excluded, especially in countries with insufficient measles immunization.

Predictors of clinical course of subacute sclerosing panencephalitis: experience at the Children's Hospital, Lahore.

To determine the clinical course of Subacute Sclerosing Panencephalitis (SSPE) and different factors affecting the clinical course. Descriptive study. The Children's Hospital, Lahore, from October 2005 to May 2008. All serologically confirmed patients of SSPE were registered and clinical staging of these patients were done from stage-I to stage-IV. Clinical course of these patients was classified by using neurological disability index as fulminant, acute, subacute, and chronic course. Clinical course was analyzed for any difference with age, gender, immunization for measles, measles infection, nutritional status and correlation with age of onset of SSPE, (Spearman's correlation), using statistic package for social science (SPSS) V. 14. A total of 57 cases (41 males, 16 females) with mean age of 7.45 years were studied. Forty (71.4%) of them were vaccinated with single dose at about 9 months of age, 41% (23/57) had measles infections ≤ 2 years of age. Using the Neurology Disability Index for these patients 10.5% had fulminant, 17.5% had acute, 49.2% subacute and 22.8% had chronic course. Age, gender, age at measles infection, SSPE onset age and nutritional status were poor predictors of clinical course of SSPE. Unvaccinated patients showed significantly more rapid course of disease (p = 0.04). Clinical course of SSPE cannot be predicted at the onset of this catastrophic disorder. Children not immunized against measles had a significant rapid course of disease.

Subacute sclerosing panencephalitis: an update

Subacute sclerosing panencephalitis (SSPE) is a chronic encephalitis occurring after infection with measles virus. The prevalence of the disease varies depending on uptake of measles vaccination, with the virus disproportionally affecting regions with low vaccination rates. The physiopathology of the disease is not fully understood; however, there is evidence that it involves factors that favour humoral over cellular immune response against the virus. As a result, the virus is able to infect the neurons and to survive in a latent form for years. The clinical manifestations occur, on average, 6 years after measles virus infection. The onset of SSPE is insidious, and psychiatric manifestations are prominent. Subsequently, myoclonic seizures usually lead to a final stage of akinetic mutism. The diagnosis is clinical, supported by periodic complexes on electroencephalography, brain imaging suggestive of demyelination, and immunological evidence of measles infection. Management of the disease includes seizure control and avoidance of secondary complications associated with the progressive disability. Trials of treatment with interferon, ribavirin, and isoprinosine using different methodologies have reported beneficial results. However, the disease shows relentless progression; only 5% of individuals with SSPE undergo spontaneous remission, with the remaining 95% dying within 5 years of diagnosis.

Tau proteins in the cerebrospinal fluid of patients with subacute sclerosing panencephalitis

Neurodegenerative diseases characterized by cytoskeletal deformation and neurofibrillary tangles are associated with altered levels of tau and related proteins in cerebrospinal fluid (CSF). Neuronal or glial fibrillary tangles have been shown in 20% of subacute sclerosing panencephalitis (SSPE) patients. We therefore investigated CSF samples from 60 newly diagnosed SSPE and 31 neurological control patients for total tau (t-tau), phosphorylated tau (p-tau), and S100-B levels by ELISA. There was no difference between patient and control groups in t-tau and S100-B levels. p-Tau was lower in the SSPE group ( p = 0.009). Past history of measles infection, measles immunization status, latent period between measles and onset of SSPE, duration of symptoms, frequency of myoclonia, neurological deficit index, stage and progression rate of the disease, CSF glucose levels and cell counts, CSF and serum measles IgG titer, distribution of lesions on brain magnetic resonance imaging were not related to t-tau, p-tau and S100-B levels. Mental status and age were negatively correlated with t-tau, and male gender and EEG abnormalities were associated with higher t-tau levels. The levels of tau proteins in our patients suggest there is no, or only scarce and immature, neurofibrillary tangle formation in SSPE. Autopsy studies showing neurofibrillary tangles might have examined older patients with longer disease and more parenchymal involvement.

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.

Cerebrospinal fluid pressures in subacute sclerosing panencephalitis

Increased intracranial pressure can rarely be the initial symptom in subacute sclerosing panencephalitis (SSPE). We examined cerebrospinal fluid (CSF) pressures and their correlation with clinical features in 58 patients with SSPE. CSF pressure varied between 50 and 500 mmH 2O, mean 210.9 ± 103.7 mmH 2O. Twenty-five (42%) patients had pressures above 200 mmH 2O and 15/58 (25%), above 250 mmH 2O. There was no correlation between CSF pressure and neurological disability, spasticity, or clinical stage. Frequent myoclonia and shorter interval between measles and onset of SSPE were associated with CSF pressure >200 mmH 2O ( p = 0.035). The causes of high pressure in certain SSPE patients is unknown but may include the effect of myoclonic jerks or inflammatory reaction. Because these patients may be unable to express symptoms, increased intracranial pressure should be considered in the presence of irritability or frequent myoclonia.

Full‐Length Sequence Analysis of Subacute Sclerosing Panencephalitis (SSPE) Virus, a Mutant of Measles Virus, Isolated from Brain Tissues of a Patient Shortly after Onset of SSPE

Subacute sclerosing panencephalitis (SSPE) virus, a measles virus (MeV) mutant, was isolated from brain tissues of a patient shortly after the clinical onset, and the entire viral genome was sequenced. The virus, named SSPE-Kobe-1, formed syncytia on B95a and Vero/SLAM cells without producing cell-free infectious virus particles, which is characteristic of SSPE virus. Phylogenetic analysis classified SSPE-Kobe-1 into genotype D3. When compared with an MeV field isolate of the same genotype (Ich-B strain), SSPE-Kobe-1 exhibited mutation rates of 0.8-1.6% at the nucleotide level in each of the proteincoding regions of the viral genome. It is noteworthy that the mutation rate of the M gene (1.2%) of SSPE-Kobe-1 was considerably lower than for other SSPE virus strains reported so far, but that the majority of the mutations (75%) were the uridine-to-cytidine biased hypermutation characteristic of the SSPE virus M gene. At the amino acid level, the viral proteins, such as N, P, C, V, M, F, H and L proteins, had point-mutations on 3, 7, 1, 4, 3, 9, 8 and 14 residues, respectively, compared with the Ich-B strain. In addition, the F and H proteins had mutated C-termini due to single-point mutations near or at the stop codons. Two of the three mutations in the M protein were Leu-to-Pro mutations, which are likely to affect the conformation and, therefore, the function of the protein. Because of the relatively small number of mutations, SSPE-Kobe-1 would be a useful tool to study genetic evolution of SSPE virus.

The epidemiology of subacute sclerosing panencephalitis in England and Wales 1990–2002

Aim: To assess the impact of measles/mumps/rubella (MMR) vaccine on the epidemiology of subacute sclerosing panencephalitis (SSPE) in England and Wales. Methods: Cases of SSPE resident in England and Wales...

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.

Clinical presentation of subacute sclerosing panencephalitis in Papua New Guinea.

Eighty-three children presented at Goroka Base Hospital in the Eastern Highlands Province (EHP) of Papua New Guinea over a period of 3 years and 9 months between February 1997 and November 2000 were confirmed to have subacute sclerosing panencephalitis (SSPE). Confirmation of the diagnosis was based on the demonstration of high titres of measles antibodies in the cerebrospinal fluid and/or serum in association with clinical features supportive of SSPE, including characteristic electroencephalographic changes and amplification of measles virus genome by reverse transcriptase polymerase chain reaction in some cases. The mean cerebrospinal fluid and serum enzyme immunoassay antibody levels among the SSPE patients were 38 250 and 860 580, respectively. The mean age of onset of SSPE was 7.9 +/- 2.6 years and ranged between 2 and 14 years. The overall male to female ratio was 1.2:1 and 1.4:1 for EHP.

Substantial spontaneous long-term remission in subacute sclerosing panencephalitis (SSPE)

We report a 25-year-old female who developed clinical and electroencephalographic (EEG) features of subacute sclerosing panencephalitis (SSPE) at the age of 17 years. After almost 17 months of progressive neurological deterioration to a level where she was bedridden and incapable of self-care (Risk and Haddad stage 3a), she experienced a substantial spontaneous clinical and EEG remission (stage 4c) which she has maintained for the last 8 years. The measles antibody titer in the cerebrospinal fluid (CSF), however, progressively increased during follow-up. There are only very few patients with well documented diagnosis of SSPE who have maintained such a prolonged remission. The age at onset of SSPE of ≥12 years, disappearance of periodic complexes and a tendency for normalization of the background activity in the EEG, and a progressive increase in the measles antibody titer in the CSF appears to predict a favourable outcome of SSPE.