Journal Article•
Subacute sclerosing panencephalitis: clinical and demographic characteristics
01 Aug 2014-Jcpsp-journal of The College of Physicians and Surgeons Pakistan (J Coll Physicians Surg Pak)-Vol. 24, Iss: 8, pp 557-560
TL;DR: Measles antibody in the CSF is diagnostic for SSPE and is helpful in early diagnosis and can be prevented by timely immunization against measles.
Abstract: 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.
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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: In vivo and in vitro studies manifesting the means in which IP impacts the host’s immune system are summarized, and positive results from limited studies, suggesting the putative future use of IP in new therapeutic indications are briefly described.
Abstract: Inosine pranobex (IP), commonly known as inosine acedoben dimepranol, isoprinosine and methisoprinol, has been proven to positively impact the host's immune system, by enhancing T-cell lymphocyte proliferation and activity of natural killer cells, increasing levels of pro-inflammatory cytokines, and thereby restoring deficient responses in immunosuppressed patients. At the same time, it has been shown that it can affect viral RNA levels and hence inhibit growth of several viruses. Due to its immunomodulatory and antiviral properties, and its safety profile, it has been widely used since 1971 against viral infections and diseases, among which subacute sclerosis panencephalitis, herpes simplex virus, human papilloma virus, human immunodeficiency virus, influenza and acute respiratory infections, cytomegalovirus and Epstein-Barr virus infections. Following an analysis of almost five decades of scientific literature since its original approval, we here summarize in vivo and in vitro studies manifesting the means in which IP impacts the host's immune system. We also provide a synopsis of therapeutic trials in the majority of which IP was found to have a beneficial effect. Lastly, positive results from limited studies, suggesting the putative future use of IP in new therapeutic indications are briefly described. In order to support use of IP against viral infections apart from those already approved, and to establish its use in clinical practice, further well-designed and executed trials are warranted.Funding: Ewopharma International.
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 "Subacute sclerosing panencephalitis..."
...Divalproate sodium is one of the common antiepileptics employed.(38) There are no standard treatment protocols for the treatment of SSPE....
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TL;DR: It is important to screen children admitted with acute cognitive decline and intractable seizures for SSPE, especially those who were infants during the measles outbreak, especially children who were toddlers during the 2009-2011 outbreak.
Abstract: Between 2009 and 2011, there was an outbreak of measles throughout South Africa (SA). The largest age category infected was children<5 years of age. In 2014, four patients, with a median age of 4 years and 5 months (range 4 years 3 months-4.5 years), three males and one female, presented with subacute sclerosing panencephalitis (SSPE). All were infected with measles during the period of the 2009-2011 outbreak in early infancy, at a time when their immune systems were immature and before they were vaccinated against the measles virus. One patient was immunocompromised, with vertically acquired HIV infection. All the children presented with cognitive and behavioural decline, abnormal movements and medically intractable myoclonic and atonic seizures. Outcome was poor in all and no reversibility was evident with standard therapeutic interventions. Optimal seizure control with carbamazepine is reported in patients with SSPE. Three of our patients who received carbamazepine experienced improved seizure control, but their neuroregression continued. Since submission of this case series, patient 1 (see Table 1) has died, and a further child has presented with the same clinical phenotype as described. On the basis of this clustering of patients in the Western Cape Province, SA, it is important to screen children admitted with acute cognitive decline and intractable seizures for SSPE, especially those who were infants during the measles outbreak.
12 citations
Cites background from "Subacute sclerosing panencephalitis..."
...A study from Karachi in Pakistan reported on 43 patients with similar clinical findings and outcomes.[16] There are several implications for management, which is significantly determined by confirmation of the diagnosis, beyond the importance to the family of having diagnostic closure....
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TL;DR: The management of measles-induced encephalitis mainly revolves around prevention against contracting the disease and providing supportive care if acquired as discussed by the authors, which is the major means of preventing this disease in childhood.
Abstract: Encephalitis, a well-known complication of measles, is inflammation of the brain parenchyma which is mostly due to the viral invasion of neurons. It presents with a variety of symptoms ranging from mild to severe depending on the extent of the damaged neurons. The diagnosis is based on clinical symptoms such as fever, headache, altered level of consciousness, focal neurological deficits, etc. A detailed history and physical examination facilitate the diagnosis. Investigations include blood tests for measles-specific antibodies, CT, MRI, and analysis of the CSF. The management of measles-induced encephalitis mainly revolves around prevention against contracting the disease and providing supportive care if acquired. The administration of the measles vaccine is the major means of preventing this disease in childhood. Two doses are required to achieve sufficient immunity against measles, the first at the age of 12–15 months and the second at 4–6 years of age. Supportive care includes administering acetaminophen for fever, oral rehydrating salt (ORS) for diarrhea and vomiting, antibiotics for otitis media and pneumonia, and using anti-epileptics such as sodium valproate for seizures. Vitamin A can be given to prevent severe effects in children. The specific treatment would depend on the type of encephalitis the patient has developed.
4 citations
References
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TL;DR: Within the limits of expert opinion and with the understanding that new research data may take precedence, the experts' recommendations provide helpful guidance in situations where the medical literature is scant or lacking.
Abstract: Background. Childhood epilepsies are a heterogeneous group of conditions that differ in diagnostic criteria and management and have dramatically different outcomes. Despite increasing data on treatment of epilepsy, research findings on childhood epilepsy are more limited and many clinical questions remain unanswered, so that clinicians must often rely on clinical judgment. In such clinical situations, expert opinion can be especially helpful. Methods. A survey on pediatric epilepsy and seizures (33 questions and approximately 650 treatment options) was sent to 57 European physicians specializing in pediatric epilepsy, 42 (74%) of whom completed it. In some questions, the experts were asked to recommend overall treatment approaches for specific syndromes (the order in which they would use certain strategies). Most of the questions asked the experts to rate options using a modified version of the RAND 9-point scale for medical appropriateness. Consensus was defined as a non-random distribution of scores by chi-square test, with ratings used to assign a categorical rank (first line/usually appropriate, second line/equivocal, and third line/usually not appropriate) to each option. Results. Valproate was treatment of choice for symptomatic myoclonic and generalized tonic-clonic seizures. For initial monotherapy for complex partial seizures, carbamazepine and oxcarbazepine were treatments of choice, with valproate also first line. As initial therapy for infantile spasms caused by tuberous sclerosis, viagabatrin was treatment of choice. As initial therapy for infantile spasms that are symptomatic in etiology, vigabatrin was also treatment of choice, with adrenocorticotropic hormone (ACTH) and prednisone other first-line options. As initial therapy for Lennox-Gastaut syndrome, valproate was treatment of choice. For acute treatment of a prolonged febrile seizure or cluster of seizures, rectal diazepam was treatment of choice. Valproate was treatment of choice as preventive therapy for febrile seizures. For benign childhood epilepsy with centro-temporal spikes, valproate was treatment of choice. For childhood and juvenile absence epilepsy, valproate was treatment of choice, with lamotrigine another first-line option (ethosuximide was another first-line option for childhood absence epilepsy). For juvenile myoclonic epilepsy in adolescent males, valproate was treatment of choice, with lamotrigine another first-line option; for juvenile myoclonic epilepsy in adolescent females, lamotrigine was treatment of choice, with valproate another firstline option. As initial therapy for neonatal status epilepticus, intravenous (IV) phenobarbital was treatment of choice. As initial therapy for all types of pediatric status epilepticus, IV diazepam was treatment of choice. For generalized tonic-clonic status epilepticus, rectal diazepam and IV lorazepam were also treatments of choice; for complex partial status epilepticus, IV lorazepam was another first-line option. Conclusion. The expert panel reached consensus on many treatment options. Within the limits of expert opinion and with the understanding that new research data may take precedence, the experts’ recommendations provide helpful guidance in situations where the medical literature is scant or lacking. The information in this report should be evaluated in conjunction with evidence-based findings.
252 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: There is no effective treatment to completely cure SSPE, but Oral isoprinosine and intrathecal or intraventricular alpha-interferon may prolong survival to some extent.
Abstract: Subacute sclerosing panencephalitis (SSPE) is a subacute encephalopathy of childhood and young adolescence. Infrequently, SSPE can occur in adults and pregnant women. It is caused by an aberrant measles virus, known as the SSPE virus. SSPE virus differs from wild-type measles viruses in the form of several mutations affecting the viral genome. The matrix gene is most commonly affected by these mutations. The characteristic clinical manifestations of SSPE include behavioral changes, cognitive decline, myoclonic jerks, seizures, abnormalities in vision, bilateral pyramidal signs and coma. Ocular changes may occur in up to 50% of patients. The most characteristic ophthalmological lesion is necrotizing retinitis. Cortical blindness can be the early feature of SSPE. The diagnosis of SSPE is often difficult in the early stages. In a typical case diagnosis is based on clinical, electroencephalographic, and cerebrospinal fluid findings. At present, there is no effective treatment to completely cure SSPE. Oral isoprinosine and intrathecal or intraventricular alpha-interferon may prolong survival to some extent. Immunization against measles is currently the most effective strategy against SSPE.
120 citations
TL;DR: It is concluded that there seems to be no correlation between the clinical stages and either the duration from the onset of subacute sclerosing panencephalitis or the MRI findings.
Abstract: We studied 36 patients (24 males, 12 females), all of whom had definite subacute sclerosing panencephalitis with typical periodic complexes in their electroencephalograms and increased titers of measles antibody in serum and cerebrospinal fluid. Their clinical and laboratory findings on admission were reviewed retrospectively. The age at onset of symptoms varied from 4 to 23 years. The average age at onset of disease was 13.1 +/- 4.18 years. The mean of the duration from the infection to the onset of subacute sclerosing panencephalitis was 9 years. Unusual symptoms, especially in the early periods of disease, included hemiparesis (7 patients), headache (3), generalized tonic-clonic seizures (6), absence seizure (1), nausea (3), and vomiting (3). Twenty-six cranial magnetic resonance imaging (MRI) and 12 computed tomography examinations were performed. Nine patients had normal MRI. In the early stages, lesions usually involved parieto-occipital corticosubcortical regions asymmetrically. In time, symmetric periventricular white-matter changes became more prominent. In addition to the common clinical findings in cases of subacute sclerosing panencephalitis reported in the literature, there were some different clinical features of the disease. Eventually, we concluded that there seems to be no correlation between the clinical stages and either the duration from the onset of subacute sclerosing panencephalitis or the MRI findings.
75 citations