Options in the Treatment of Subacute Sclerosing Panencephalitis: Implications for Low Resource Areas (original) (raw)
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The Journal of infectious diseases, 2005
The most severe sequela of measles virus infection is subacute sclerosing panencephalitis (SSPE), a fatal disease of the central nervous system that generally develops 7-10 years after infection. From 1989 through 1991, a resurgence of measles occurred in the United States, with 55,622 cases of measles reported. The purpose of the present study was to identify cases of SSPE that were associated with the resurgence of measles and to calculate the risk of developing SSPE. Brain tissue samples obtained from 11 patients with a presumptive diagnosis of SSPE were tested for the presence of measles virus RNA. Measles virus genotypes were determined by reverse-transcription polymerase chain reaction (RT-PCR) and by analysis of the sequences of the PCR products. A search of the literature was conducted to identify reports of cases of SSPE in persons residing in the United States who had measles during 1989-1991. The measles virus sequences derived from brain tissue samples obtained from 11 p...
A Multinational Survey on Actual Diagnostics and Treatment of Subacute Sclerosing Panencephalitis
Neuropediatrics, 2015
Subacute sclerosing panencephalitis (SSPE) is a chronic infection of the central nervous system caused by the measles virus (MV). Its prevalence remains high in resource poor countries and is likely to increase in the Northern Europe as vaccination rates decrease. Clinical knowledge of this devastating condition, however, is limited. We therefore conducted this multinational survey summarizing experience obtained from more than 500 patients treated by 24 physicians in seven countries. SSPE should be considered in all patients presenting with otherwise unexplained acquired neurological symptoms. In most patients, the diagnosis will be established by the combination of typical clinical symptoms (characteristic repetitive myoclonic jerks), a strong intrathecal synthesis of antibodies to MV and typical electroencephalogram findings (Radermecker complexes). Whereas the therapeutic use of different antiviral (amantadine, ribavirin) and immunomodulatory drugs (isoprinosine, interferons) an...
Changing Epidemiological Features of Subacute Sclerosing Panencephalitis
Infection, 2001
Background: Subacute sclerosing panencephalitis (SSPE) is a chronic central nervous (CNS) system infection caused by measles virus. Because changing immunization practices affect the epidemiology of measles and consequently SSPE, we examined the epidemiological data of our SSPE registry. Materials and Methods: Age of onset, age at onset of measles, duration of latent period and immunization status were examined in cases recorded at the SSPE Registry Center in Turkey between 1975 and 1999. Results: Age of onset diminished from 13 years before 1994 to 7.6 years after 1995; age at onset of measles declined from 29 months to 20 months and the latent interval from 9.9 years to 5.9 years. Age at onset of measles and immunization status did not directly affect the duration of the latent period. Conclusion: Although its incidence has decreased in Turkey, SSPE has been seen at younger ages in recent years. This change cannot be attributed solely to younger age at onset of measles. Factors affecting the duration of the latent period should be investigated further.
IP Indian Journal of Anatomy and Surgery of Head, Neck and Brain
Subacute sclerosing panencephalitis (SSPE) is characterized by gradual deterioration of cognitive motor and cognitive functions. SSPE more often occurs in individuals who had a history of measles viral infection. We present a case of 8 year old male child who was presented with generalized progressive weakness, recurrent falls to one side, multiple episodes of seizure, urinary incontinence. On liaison it was understood that he had a history of measles at the age of 3 years. Examination showed elevated serum measles antibody titer, electro-encephalogram (EEG) analysis revealed stereotyped polymorphic discharge seen periodically, suggestive of SSPE. He was treated with IVIG and other symptomatic treatment. However he had recurrent episodes of vomiting after IVIG administration, Ribavirin administration caused multiple oral lesions. Upon discharge he was given Syr. Valproate, Oral Steroid (Prednisolone) tapering therapy, THP 2 mg BD, PPI and Laxatives. Parents were advised to maintain ...
Subacute Sclerosing Panencephalitis in Children: The Archetype of Non-Vaccination
Viruses, 2022
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.
Clinical Infectious Diseases
See the Editorial Commentary by Rota et al on pages 233-4.) 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.
Subacute sclerosing panencephalitis: Changes in phenotype during the last decade
Medicinska istraživanja, 2023
Introduction: Subacute sclerosing panencephalitis (SSPE) is a rare, progressive, neurodegenerative disease with poor outcome. Anti-measles vaccination contributed to a decreasing number of SSPE patients, but not to its eradication. The aim of our study is to evaluate the course of the disease in our SSPE patients with a focus on vaccinated children. The main goal is considering possibilities for improving prevention of the disease. Methods: A retrospective study included the patients with SSPE treated in the period from December 2010 to December 2020 at the Pediatric Clinic of the Institute. The inclusion criteria were the patients with the diagnosis of SSPE based on clinical presentation, neuroimaging, electroencephalography and positive IgG anti-measles antibodies, both in serum and CSF. Results: Five children with fulminant course of SSPE were included. All these patients were suffering from measles at an early age. Three of them had been vaccinated against measles and two had not. All of them had previously been healthy, immune-competent children, with normal general development. The course was extremely fulminant with lethal outcome within three months since the initial symptoms in four cases. Progressive motor and cognitive decline, behavior changes, movement disorders, myoclonic jerks and seizures were dominant in clinical presentation. Conclusion: Despite vaccination, SSPE has not been eradicated. An increasing number of vaccinated immune-competent children with fulminant form of SSPE and history of measles infection at an early age were treated at our Clinic. As a measure for improving prevention, we suggest considering weaning of vaccine-derived immunity, and re-vaccination of girls at reproductive period.
Diagnostic Approaches and Management of Subacute Sclerosing Panencephalitis in Children
Berkala Kedokteran
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.
Subacute Sclerosing Panencephalitis in a Toddler: Changing Epidemiological Trends
Case Reports in Pediatrics, 2013
Subacute sclerosing panencephalitis (SSPE) is a devastating “slow virus” brain disease resulting from persistent measles virus infection of neurons. The age at presentation is usually 8 to 11 years with onset usually occurring 2–10 years after measles infection. We report a 2-and-half-year-old boy who presented with progressively increasing myoclonic jerks and subtle cognitive decline. He was diagnosed as a case of SSPE based on clinical features, typical electroencephalographic finding, and elevated cerebrospinal fluid/serum measles antibody titers. He had measles 4 months prior to onset of symptoms. This case along with review of recently published reports suggests progressively decreasing latency period between measles infection and onset of symptoms observed in cases with SSPE. Clinical implication would mean investigating for SSPE even in infants or toddlers with compatible clinical features and recent history of measles infection.
European Journal of Pediatrics, 2002
We report an 18-month-old girl with rapidly progressive subacute sclerosing panencephalitis, whose non immunised mother had measles at the time of delivery. The patient presented with repetitive episodes of myoclonic jerks of the head and arms, followed by a drop of head and trunk with frequent falls. EEG, CSF studies and MRI confirmed the diagnosis. Despite therapy with isoprinosine and valproate, seizure activity continued and she became vegetative within 2 months, with severe spasticity and swallowing difficulties, and died at the age of 28 months. Early age of onset and rapid progression were most likely related to haematogenous in utero acquisition of the measles virus prior to delivery, as well as immaturity of neuronal and immune systems. Conclusion: this case emphasises the importance of a high measles vaccine coverage in the population in order to prevent the risk of disease in general and, in particular, gestational measles.