Epilepsy in children with subacute sclerosing panencephalitis.
TL;DR: Atypical SSPE presentation can include mainly focal intractable seizures, and no significant influence was found of the history of epilepsy on the type of S SPE progression.
Abstract: 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.
Citations
More filters
TL;DR: A case-control study of medical, nutritional and other risk factors associated with NS among children of Kitgum District, northern Uganda found families with one or more NS Cases had been significantly more dependent on emergency food and, immediately prior to head nodding onset in the child, subsistence on moldy plant materials, specifically moldy maize.
41 citations
TL;DR: Subacute sclerosing panencephalitis is a progressive neurodegenerative disease that usually occurs 7–10 years after measles infection and is preventable by ensuring that an effective vaccine program for measles is made compulsory for all children younger than 5 years in endemic countries.
Abstract: Subacute sclerosing panencephalitis is a progressive neurodegenerative disease. It usually occurs 7–10 years after measles infection. The clinical course is characterized by progressive cognitive decline and behavior changes followed by focal or generalized seizures as well as myoclonus, ataxia, visual disturbance, and later vegetative state, eventually leading to death. It is diagnosed on the basis of Dyken’s criteria. There is no known cure for subacute sclerosing panencephalitis to date, but it is preventable by ensuring that an effective vaccine program for measles is made compulsory for all children younger than 5 years in endemic countries.
38 citations
Cites background from "Epilepsy in children with subacute ..."
...Developed countries such as USA have reported an incidence of 6.5–11 cases per 100,000 acute measles infections.12 European countries like Turkey have reported an incidence of 2.2 cases per million in their population.16 Developing countries like Pakistan have reported an estimated incidence of 10 cases per million in their population.17 The highest rate that has ever been reported is from Papua New Guinea, which is 51 cases per one million during 2007–2009.18 Although there is no gender predisposition, but SSPE has been seen more commonly in boys.19 The risk of SSPE is higher if the onset of measles is at a younger age, in low socioeconomic class, in cases with low parental education and large family size.4 The clinical course is characterized by progressive cognitive decline and behavior changes followed by focal or generalized seizures as well as myoclonus, ataxia, visual disturbance, and later vegetative state.20,21 Patients suffering from SSPE die within few years of initial clinical presentation although there have been rare case reports of spontaneous remission.20 Epilepsy has been reported in one-third of the patients with SSPE.22 Jabbour et al23 have divided the clinical manifestations into four stages....
[...]
...Although the number of countries with measles vaccine 1 coverage of >90% has risen between 2010 and 2015, we are still a long way from a global measles eradication.37 Pediatric Health, Medicine and Therapeutics 2018:9submit your manuscript | www.dovepress.com Dovepress Dovepress 70 Jafri et al Supportive treatment including management of seizures and other complications is the mainstay.11 Divalproate sodium is one of the common antiepileptics employed.38 There are no standard treatment protocols for the treatment of SSPE....
[...]
...Neuroimaging may be helpful but is not characteristic of SSPE....
[...]
...Ketogenic diet has also been tried; it was found to temporarily reduce the myoclonic jerks.40 Steroids and intravenous immunoglobulin are no longer recommended for the treat- ment of SSPE....
[...]
...Acute complications include otitis media, pneumonia, diarrhea, and postinfectious encephalitis.5 Neurologi- cal complications of measles involve post-measles encephalitis, measles inclusion body encephalitis, transverse myelitis, and SSPE.6 The risk of serious complications and death is increased in children younger than 5 years and adults older than 20 years.7 This disease is preventable, and immunization against measles via live attenuated vaccine has been available for more than 45 years.8 SSPE usually occurs 7–10 years after measles infection, but the latency varies from 1 month to 27 years.9 A shorter latency has been reported in intrafamilial cases of SSPE as well as in children who were affected at an earlier ( 2 years) age such that the incidence was 18/100,000 in children younger than 5 years and 1.1/100,000 in those children with measles after 5 years.10,11 It is caused by the cerebral involvement of the Correspondence: Shahnaz H Ibrahim Department of Pediatrics and Child Health, Aga Khan University, Stadium Road, PO Box 3500, Karachi, Pakistan Tel +92 21 3486 4732 Fax +92 21 3493 2095 Email shahnaz.ibrahim@aku.edu Pediatric Health, Medicine and Therapeutics 2018:9submit your manuscript | www.dovepress.com Dovepress Dovepress 68 Jafri et al measles virus, which causes destruction of the neurons....
[...]
TL;DR: This report documents current data relating to the clinical phenotype, epidemiology, and understanding of SSPE, inclusive of preventive interventions, and indicates children residing in areas with poor vaccination coverage and high prevalence of human immunodeficiency virus are at increased risk of developing S SPE.
Abstract: 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.
31 citations
Cites background from "Epilepsy in children with subacute ..."
...Levetiracetam is reported to have mild improvement in myoclonus.(45) Improvement in myoclonus with carbamazepine is also reported....
[...]
TL;DR: Measles and its neurological complications are preventable and must be prevented as mentioned in this paper, and the consequences of inadequate herd immunity to measles should be warned to the public and other clinicians.
Abstract: Owing to vaccine hesitancy, there has been a resurgence of measles infections in developed countries. Practitioners can expect to see an increase in patients with neurologic complications of measles. These devastating disorders include primary measles encephalitis, acute post measles encephalitis, subacute sclerosing panencephalitis (SSPE), and measles inclusion body encephalitis (MIBE). Although there are many unanswered questions regarding the neurologic complications of measles, recent advances have led to better understanding of the mechanism of the spread of measles within the nervous system, particularly the disruption of F protein function, which raises the possibility of treatment with fusion-inhibiting molecules. Measles and its neurological complications are preventable and must be prevented. Neurologists must educate other clinicians and the public regarding the consequences of inadequate herd immunity to measles. More effective treatments for SSPE and MIBE may be available in the near future, but currently these remain lethal diseases.
12 citations
TL;DR: Because imaging studies and analysis of cerebrospinal fluid failed to demonstrate the parasite in the central nervous system (CNS) of NS patients, it was suggested that an immunological process related to O. volvulus could play a role in the origination of NS.
Abstract: Nodding syndrome (NS) is a neurological disorder primarily characterized by paroxysmal head nodding seizures, affecting previously healthy children at the age between 3–18 years (1,2). Growing numbers of patients with NS were documented since the early 2000s in three distinct areas in the southern Sudan (3,4), southern Tanzania (1) and northern Uganda (5,6). In retrospect, NS was also found in western Uganda (7,8) and suspected cases were reported from Liberia (9) and Cameroon (10). In many NS patients, initial head seizure are followed by other seizures types, progressing physical and cognitive deterioration, and death (6,8). To date, the exact causation of NS is not clarified, but epidemiological and case-control studies have shown a consistent correlation between NS and infection with Onchocerca volvulus ( O. volvulus ), a parasite known as the cause of river blindness in large parts of tropical Africa (2,4,11,12). Because imaging studies and analysis of cerebrospinal fluid (CSF) failed to demonstrate the parasite in the central nervous system (CNS) of NS patients (1,4-6,13) it was suggested that an immunological process related to O. volvulus could play a role in the origination of NS (14,15).
3 citations
References
More filters
TL;DR: A combination of oral isoprinosine (Inosiplex) and intraventricular interferon alfa appears to be the best effective treatment for subacute sclerosing panencephalitis.
Abstract: Subacute sclerosing panencephalitis (SSPE) is a progressive neurological disorder of childhood and early adolescence. It is caused by persistent defective measles virus. Brain biopsies or postmortem histopathological examination show evidence of astrogliosis, neuronal loss, degeneration of dendrites, demyelination, neurofibrillary tangles, and infiltration of inflammatory cells. Patients usually have behavioral changes, myoclonus, dementia, visual disturbances, and pyramidal and extrapyramidal signs. The disease has a gradual progressive course leading to death within 1-3 years. The diagnosis is based upon characteristic clinical manifestations, the presence of characteristic periodic EEG discharges, and demonstration of raised antibody titre against measles in the plasma and cerebrospinal fluid. Treatment for SSPE is still undetermined. A combination of oral isoprinosine (Inosiplex) and intraventricular interferon alfa appears to be the best effective treatment. Patients responding to treatment need to receive it life long. Effective immunisation against measles is the only solution presently available to the problem of this dreaded disease.
308 citations
TL;DR: Management of the disease includes seizure control and avoidance of secondary complications associated with the progressive disability, and trials of treatment with interferon, ribavirin, and isoprinosine using different methodologies have reported beneficial results.
Abstract: 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.
173 citations
TL;DR: 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 was conducted in this paper.
Abstract: Background 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. Methods 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. Results 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. Conclusions 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.
154 citations
TL;DR: Irrespective of the incidence of occurrence of SSPE in a community, a clinician should be aware of the wide spectra of EEG findings and provide valuable insight into the structural and clinical correlates of EEG changes in S SPE.
55 citations
TL;DR: An overall review on SSPE and its relationship to measles is given, including a prospectus of its history, considerations as to its etiology, correlation of clinicopathological features, and thoughts on the past and present epidemiology and treatment.
Abstract: 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.
54 citations