Patients With Subacute Sclerosing Panencephalitis in Japan: A 2022 Nationwide Survey.

BACKGROUND: In Japan, the incidence of subacute sclerosing panencephalitis (SSPE) has reduced; however, the medical conditions and factors associated with disease progression remain unclear. METHODS: A nationwide survey of SSPE was conducted using a questionnaire in 2022. We conducted a descriptive analysis of the patients with SSPE in 2022 and Cox proportional hazards analyses for disease progression. We compared the patients with SSPE with those in a 2007 survey. RESULTS: A total of 37 surviving patients with SSPE were enrolled [median age: 32 years (range: 16-52 years)]. No new cases have been identified since 2017 in the survey. Jabbour stage IV was the most common stage (66.7%). The hazard ratios (95% confidence intervals) of male sex and age at the time of measles infection (years) were 2.56 (1.13-5.76) and 0.57 (0.34-0.93), respectively. Compared with those in 2007, the proportion of patients in hospitals decreased from 13.7% to 2.7%, whereas that of patients in nursing facilities increased from 17.6% to 29.7%. The proportions of patients prescribed inosine pranobex, interferon and ribavirin at the time of the survey decreased from 96.1% to 79.4%, 74.8% to 14.3% and 25.3% to 0%, respectively. The proportions of patients with gastrostomy, tracheostomy and ventilator use increased from 5.9% to 69.7%, 23.3% to 60.0% and 10.8% to 32.4%, respectively. CONCLUSIONS: Decreased measles cases in Japan reduced new SSPE cases. However, surviving patients in 2022 had advanced disease stages and needed medical care. Male sex and early measles infection were significantly associated with disease progression.

Measles Virus and the Central Nervous System: An Update.

MEASLES VIRUS AND ASSOCIATED CENTRAL NERVOUS SYSTEM: Sequelae Renee Buchanan, Daniel J. Bonthius Seminars in Pediatric Neurology Volume 19, Issue 3, September 2012, Pages 107-114 Worldwide, measles remains one of the most deadly vaccine-preventable diseases. In the United States, enrollment in the public schools requires that each child receives 2 doses of measles-containing vaccine before entry, essentially eliminating this once endemic disease. Recent outbreaks of measles in the United States have been associated with importation of measles virus from other countries and subsequent transmission to intentionally undervaccinated children. The central nervous system complications of measles can occur within days or years of acute infection and are often severe. These include primary measles encephalitis, acute postinfectious measles encephalomyelitis, measles inclusion body encephalitis, and subacute sclerosing panencephalitis. These measles associated central nervous system diseases differ in their pathogenesis and pathologic effects. However, all involve complex brain-virus-immune system interactions, and all can lead to severe and permanent brain injury. Despite better understanding of the clinical presentations and pathogenesis of these illnesses, effective treatments remain elusive.

Fatal subacute sclerosing panencephalitis in an 8-year-old male: a case report.

Subacute sclerosing panencephalitis (SSPE) is a chronic slow progressive neurodegenerative disease that is often associated with measles complications. The disease is characterized by seizures, behavioral changes, motor deficit and eventually death. In this case report we discuss the case of an 8-year-old male who developed SSPE and was presented to our hospital with a history of generalized tonic colonic convulsion followed by gait abnormality, episodes, abnormal behaviors, and cognitive regression. On clinical exploration, the child had a history of measles at 8 months of age and meningitis at 18 months. The electroencephalogram (EEG) investigation showed high amplitude spikes, with focal seizure and slowing, while the magnetic resonance imaging reveal signals synonymous with high fluid-attenuated inversion recovery (FLAIR), both of which are consistent with probable SSPE. The case was managed symptomatically; until his parents decided to take him back home, after which his condition deteriorated, and he sadly died. To the best of our knowledge, this is the first recorded case of SSPE in Mogadishu, Somalia. Hence, the need to further investigation to better understand the incidence of the disease in the population and propose better ways of managing the condition.

A Re-emergence of Subacute Sclerosing Panencephalitis in the United Kingdom.

New pediatric and adult subacute sclerosing panencephalitis cases between 1996 and 2020 were reported based on an established UK registry with no evidence of under-ascertainment using a separate pediatric surveillance system. After 15 years with no pediatric UK-acquired cases, 3 cases arose from 2017 after increased measles. Modeling suggested this was in line with measles notifications, underreporting of laboratory-confirmed measles or increased subacute sclerosing panencephalitis risk.

Subacute Sclerosing Panencephalitis in Children: The Archetype of Non-Vaccination.

Subacute sclerosing panencephalitis (SSPE) is a late complication of measles virus infection that occurs in previously healthy children. This disease has no specific cure and is associated with a high degree of disability and mortality. In recent years, there has been an increase in its incidence in relation to a reduction in vaccination adherence, accentuated by the COVID-19 pandemic. In this article, we take stock of the current evidence on SSPE and report our personal clinical experience. We emphasise that, to date, the only effective protection strategy against this disease is vaccination against the measles virus.

Spectrum of Movement Disorders Among Children With Subacute Sclerosing Panencephalitis: A Cross-Sectional Study.

Background: Subacute sclerosing panencephalitis is a progressive devastating condition due to persistence of mutant measles virus, affecting children and adolescents, characterised by myoclonus, seizures, and neuropsychiatric issues. Movement disorders apart from myoclonus are reportedly uncommon. We aimed to describe frequency and proportion of movement disorders among children with subacute sclerosing panencephalitis, hypothesizing that these occur more frequently than previously reported. Methods: In this cross-sectional study, we enrolled children with subacute sclerosing panencephalitis between 1 month and 18 years of age who fulfilled the diagnosis of subacute sclerosing panencephalitis as per modified Dyken criteria, and examined them for movement disorders. We also assessed their clinical profile and disease severity via Jabbour staging and modified Rankin Scale score. We compared demographic, clinical, and laboratory features of children with and without movement disorders. Results: We enrolled 50 children (36 males; 72%) (age range 1.5-14 years). Of these, 28 (56%) had movement disorders. Among movement disorders, the most frequent was myoclonus (92%), followed by ataxia (9; 18%), chorea-athetosis (7; 14%), dystonia (6; 12%), tremor (4; 8%), repetitive behavior (4; 8%), and parkinsonism (3; 6%). Movement disorders were the presenting feature of subacute sclerosing panencephalitis among 7 children. There were no significant differences in clinical or laboratory features among children with and without movement disorders. Conclusions: Movement disorders were frequent in subacute sclerosing panencephalitis. Hyperkinetic disorders were dominant. Dystonia and chorea-athetosis occurred more commonly among nonmyoclonus movement disorders. Movement disorders may manifest even in earlier stages of subacute sclerosing panencephalitis and may be the heralding feature. Recognition of these features is important to plan management and reduce morbidity.

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

BACKGROUND: Subacute Sclerosing Panencephalitis (SSPE) is a fatal progressive neurological disorder following measles infection. METHODS: 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. FINDINGS: 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. INTERPRETATION: 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.

Measles Sclerosing Subacute PanEncephalitis (SSPE), an intriguing and ever-present disease: Data, assumptions and new perspectives.

BACKGROUND: Subacute sclerosing panencephalitis (SSPE) is a rare, non-treatable and fatal neurological complication of measles, still present due to the return of the epidemic linked to the loosening of vaccination policies. Its mechanism remains unexplained. OBJECTIVE: The main objective was to investigate explanatory variables relating to the risk of developing SSPE and its pathophysiology. METHODS: Literature analysis was focused on different varieties of SSPE: perinatal forms, short-incubation forms similar to acute measles inclusion body encephalitis (MIBE), rapidly evolving forms, forms occurring in the immunosuppressed, adult forms, and family forms. In addition, several studies on the parameters of innate immunity and interferon responses of patients were analyzed. RESULTS: Two main data were highlighted: a relationship between the so-called fulminant forms and the prescription of corticosteroids was established. In familial SSPE, two groups were individualized according to the duration of the latency period, prompting an analysis of patient exomes. CONCLUSION: Treatment with corticosteroids should be banned. Knowledge of the genes involved and epigenetics should be useful for understanding the pathophysiology of SSPE and other late-onset neurological infections with RNA viruses.

High risk of subacute sclerosing panencephalitis following measles outbreaks in Georgia.

OBJECTIVE: To describe cases and estimate subacute sclerosing panencephalitis (SSPE) risk following large-sale measles outbreaks in Georgia. A rare, fatal late complication of measles, SSPE is often overlooked in assessments focused on the acute illness. Georgia had 8377 and 11,495 reported measles cases during the 2004-2005 and 2013-2015 outbreaks, respectively, but SSPE burden has not been assessed. METHODS: SSPE cases diagnosed during 2008-2017 were identified from hospitalization registries in major neurological departments likely to admit SSPE patients. Information on reported measles cases and deaths was obtained from the national measles surveillance system and published reports. The risk of SSPE (number of measles cases per one SSPE case) was calculated for cases associated with the 2004-2005 outbreak. Crude estimates were adjusted to account for potential under-reporting of measles, using 50%, 25% and 10% estimates of completeness of reporting. RESULTS: Sixteen SSPE cases diagnosed during 2008-2017 were identified. Eleven (92%) of 12 SSPE cases with a known history of measles had infection at ≤2 years and one (8%) at 3 years of age. Crude estimate of SSPE risk for the 2004-2005 outbreak was 1:1396. Adjusted estimates were 1:2792, 1:1:5584 and 1:13 960, assuming 50%, 25% and 10% completeness of reporting measles cases, respectively. CONCLUSIONS: The review demonstrated substantial risk of SSPE in Georgia, supporting recent data suggesting that risk of SSPE following measles infection is higher than previously thought. To prevent SSPE in Georgia, very high timely immunization coverage for measles should be achieved among children, and immunity gap among adults should be closed.

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.

Subacute sclerosing panencephalitis: clinical phenotype, epidemiology, and preventive interventions.

Subacute sclerosing panencephalitis (SSPE) is a preventable condition reported in 6.5 to 11 per 100 000 cases of measles, and highest in children who contracted measles infection when they were less than 5 years of age. Children residing in areas with poor vaccination coverage and high prevalence of human immunodeficiency virus are at increased risk of developing SSPE. SSPE is life-threatening in most affected children. This report documents current data relating to the clinical phenotype, epidemiology, and understanding of SSPE, inclusive of preventive interventions. While improvements in disease progression with immunomodulation may occur, overall there is no cure. Most therapies focus on supportive needs. Seizures and abnormal movements may respond to carbamazepine. Many countries advocate policies to enhance vaccination coverage. Effective preventive health care programmes, assurance of parental perceptions, and crisis support for unprecedented events obstructing effective primary health care are needed. Until measles is eradicated worldwide, children in all regions remain at risk. WHAT THIS PAPER ADDS: Measles contracted under 5 years of age has highest risk of developing subacute sclerosing panencephalitis (SSPE). Children with, or exposed to, human immunodeficiency virus infection, who contract measles may be at increased risk of SSPE.

PANENCEFALITIS ESCLEROSANTE SUBAGUDA: FENOTIPO CLÍNICO, EPIDEMIOLOGÍA E INTERVENCIONES PREVENTIVAS: La panencefalitis esclerosante subaguda (SSPE, por sus siglas en inglés) es una afección prevenible notificada en 6,5 a 11 por cada 100 000 casos de sarampión, y es más alta en los niños que contrajeron una infección de sarampión cuando tenían menos de 5 años de edad. Los niños que residen en áreas con una cobertura de vacunación deficiente y una alta prevalencia del virus de inmunodeficiencia humana tienen un mayor riesgo de desarrollar SSPE. La SSPE es potencialmente mortal en la mayoría de los niños afectados. Este informe documenta los datos actuales relacionados con el fenotipo clínico, la epidemiología y la comprensión del SSPE, incluidas las intervenciones preventivas. Si bien pueden producirse mejoras en la progresión de la enfermedad con la inmunomodulación, en general no hay cura. La mayoría de las terapias se centran en las necesidades de apoyo. Las convulsiones y los movimientos anormales pueden responder a la carbamazepina. Muchos países abogan por políticas para mejorar la cobertura de vacunación. Se necesitan programas de atención médica preventiva eficaces, seguridad de las percepciones de los padres y apoyo a la crisis para eventos sin precedentes que obstruyan la atención primaria de salud efectiva. Hasta que se erradique el sarampión en todo el mundo, los niños en todas las regiones siguen en riesgo.

PANENCEFALITE ESCLEROSANTE SUB-AGUDA: FENÓTIPO CLÍNICO, EPIDEMIOLOGIA, E INTERVENÇÕES PREVENTIVAS: A panencefalite esclerosante sub-aguda (PEES) é uma condição prevenível reportada em 6,5 to 11 por 100.000 casos de sarampo, e é mais alta em crianças que contraíram infecção por sarampo com menos de 5 anos de idade. Crianças que residem em áreas com pobre cobertura vacinal e alta prevalência de vírus da imunodeficiência humana apresentam maior risco de desenvolver PEES. A PEES representa risco de vida para as crianças mais afetadas. Este relato documenta dados atuais relacionados a fenótipo clínico, epidemiologia, e compreensão da PEES, incluindo intervenções preventivas. Enquanto melhoras na progressão da doença com a imunomodulação podem ocorrer, em geral não há cura. A maior parte das terapias foca em necessidades de suporte. Convulsões e movimentos anormais podem responder a carbamazepina. Muitos países defendem políticas para melhorar a cobertura vacinal. Programas efetivos de cuidado preventivo em saúde, reforço das percepções parentais, e suporte de crise para eventos sem precedentes obstruindo o cuidado primário efetivo são necessários. Até que o sarampo esteja erradicado em todo o mundo, crianças de todas as regiões permanecem em risco.

Subacute sclerosing panencephalitis mortality, United States, 1979-2016: Vaccine-induced declines in SSPE deaths.

Subacute sclerosing panencephalitis (SSPE) is a neurodegenerative disease caused by measles virus. We estimate SSPE age-specific mortality in the United States, 1979-2016. The general decline in SSPE mortality reflects that of measles. Shifts, over time, in SSPE mortality by age echo changes in the age distribution of measles in the 1970s and in the 1989-91 outbreak. The current epidemiological situation is that autochthonous SSPE will disappear in the United States, assuming measles vaccination rates remain high.

Subacute Sclerosing Panencephalitis: The Devastating Measles Complication That Might Be More Common Than Previously Estimated.

BACKGROUND: Subacute sclerosing panencephalitis (SSPE) is a fatal complication of measles. We reviewed California cases from 1998-2015 to understand risk factors for SPPE and estimate incidence. METHODS: SSPE cases had clinically compatible symptoms and measles antibody detection in cerebrospinal fluid (CSF) or medical record documentation of SSPE. Cases were identified though a state death certificate search, Centers for Disease Control and Prevention reports, or investigations for undiagnosed neurologic disease. Measles detection in CSF was performed by serology at the California Department of Public Health or at clinical laboratories. RESULTS: Seventeen SSPE cases were identified. Males outnumbered females 2.4:1. Twelve (71%) cases had a history of measles-like illness; all 12 had illness prior to 15 months of age. Eight (67%) children were exposed to measles in California. SSPE was diagnosed at a median age of 12 years (3-35 years), with a latency period of 9.5 years (2.5-34 years). Among measles cases reported to CDPH during 1988-1991, the incidence of SSPE was 1:1367 for children <5 years, and 1:609 for children <12 months at time of measles disease. CONCLUSIONS: SSPE cases in California occurred at a high rate among unvaccinated children, particularly those infected during infancy. Protection of unvaccinated infants requires avoidance of travel to endemic areas, or early vaccination prior to travel at age 6-11 months. Clinicians should be aware of SSPE in patients with compatible symptoms, even in older patients with no specific history of measles infection. SSPE demonstrates the high human cost of "natural" measles immunity.

Prognosis and demographic characteristics of SSPE patients in Istanbul, Turkey.

AIM: SSPE is a rare progressive, invariably fatal long-term complication of measles infection. In this study, we assessed the demographic and prognostic characteristics of 64 consecutive SSPE patients diagnosed at a tertiary center. METHODS: The study had a retrospective design; data were obtained from patient records. RESULTS: The study includes 64 patients diagnosed with SSPE. There was history of consanguineous marriage in 27 (42.2%) patients. The average patient lifespan was 3.8years (45days-12years). The average patient age at diagnosis was 12.3 (range, 5-17)years. A statistically significant correlation was found between the age at diagnosis and lifespan (p=0.002). A statistically significant correlation was found between the incubation period and patient lifespan (p<0.001). No significant correlation was found between duration in the intensive care unit and lifespan (p=0.122). Routine physical therapy had no significant impact on the average lifespan (p=0.619). No significant difference was found between the vaccination dose and lifespan (p=0.651). CONCLUSIONS: High frequency of parental consanguinity in SSPE patients need to be evaluated as there might a genetic influence. Physical therapy and supportive treatments seems to have no affect on lifespan in SSPE patients. The age at diagnosis and incubation period might have an affect on prognosis and lifespan.

Pancreatitis autoinmune: un dilema quirúrgico / Autoimmune pancreatitis: a surgical dilemma

La pancreatitis autoinmune (PAI) es una enfermedad fibroinflamatoria benigna del páncreas, se manifiesta frecuentemente como ictericia obstructiva asociada a masa pancreática o lesión obstructiva de la vía biliar y presenta una respuesta excelente a corticoides. Aunque no existen estudios a nivel mundial que definan su epidemiología, la PAI se considera una entidad poco frecuente, con una prevalencia estimada del 2% de los pacientes con pancreatitis crónica. Su frecuente presentación clínica y radiológica en forma de masa pancreática e ictericia similar al cáncer de páncreas y la falta de elementos diagnósticos específicos son causa de un elevado porcentaje de resecciones quirúrgicas pancreáticas por una enfermedad benigna que responde a tratamiento médico. En esta revisión detallamos los acuerdos actuales para el diagnóstico, clasificación y tratamiento de la PAI, enfatizando en las series quirúrgicas y en estrategias para mejorar el diagnóstico diferencial con el cáncer de páncreas y evitar así resecciones pancreáticas innecesarias

Autoimmune pancreatitis (AIP) is defined as a particular form of pancreatitis that often manifests as obstructive jaundice associated with a pancreatic mass or an obstructive bile duct lesion, and that has an excellent response to corticosteroid treatment. The prevalence of AIP worldwide is unknown, and it is considered as a rare entity. The clinical and radiological presentation of AIP can mimic bilio-pancreatic cancer, presenting difficulties for diagnosis and obliging the surgeon to balance decision-making between the potential risk presented by the misdiagnosis of a deadly disease against the desire to avoid unnecessary major surgery for a disease that responds effectively to corticosteroid treatment. In this review we detail the current and critical points for the diagnosis, classification and treatment for AIP, with a special emphasis on surgical series and the methods to differentiate between this pathology and bilio-pancreatic cancer

The upsurge of SSPE--a reflection of national measles immunization status in Pakistan.

Subacute sclerosing panencephalitis (SSPE) is a rare disorder in the developed world. However, an upsurge has been seen lately in our part of the world owing to inadequate measles immunization coverage. At the midst of our struggle against polio, we are struggling with the war against other vaccine-preventable childhood illnesses like measles. The increasing numbers of SSPE that we reported over the past half decade suggest an underlying periodic measles epidemic in Pakistan. In addition, children are now presenting with SSPE in early childhood, warranting a relook, reinforcement and strengthening of primary immunization and mandatory two-dose measles vaccination for all children nationwide. Previously undertaken Measles Supplementary Immunization Activity were a failure in terms of providing the expected cover against measles in young children. Intensive surveillance and establishment of SSPE registers at the district level is essential for eradication of this easily preventable disorder. Unless timely efforts are made to achieve global immunization, SSPE is bound to add to the national disability burden.

Subacute sclerosing panencephalitis: clinical and demographic characteristics.

OBJECTIVE: To determine the clinical and demographic characteristics of children diagnosed with Subacute sclerosing panencephalitis (SSPE). STUDY DESIGN: Case series. PLACE AND DURATION OF STUDY: The Aga Khan University Hospital, Karachi, from January 2000 to June 2012. METHODOLOGY: A retrospective analysis was done, regarding medical charts of 43 children under the age of 16 years with a discharge diagnosis of SSPE. Demographic and clinical characteristics were recorded. RESULTS were expressed as percentages. RESULTS: Most of the 43 patients were male (72%). The average age at presentation was 8.7 years with average duration of symptoms being 100.6 days. History of measles was present in 17 patients (39.5%). All children had seizures at presentation and 65% had cognitive impairment. Most patients required poly therapy for control of seizures. Sodium valproate was the most commonly used anti-epileptic agent; Isoprinosine was tried in 22 (51%) patients. CSF for antimeasles antibodies was positive in approximately 86% of the 40 (93%) children. EEG showed burst suppression pattern in 36 (83.7%) cases. Forty-two patients (97.6%) were discharged home in a vegetative state. CONCLUSION: SSPE is progressive neurodegenerative disorder. It can be prevented by timely immunization against measles. Measles antibody in the CSF is diagnostic for SSPE and is helpful in early diagnosis. Most patients experience a gradual but progressive decline in motor and cognitive functions.

Epilepsy in children with subacute sclerosing panencephalitis.

INTRODUCTION: 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. OBJECTIVE: 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. METHODS: 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). RESULTS: 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. CONCLUSION: 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.