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.

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.

The behavior pattern of parents of patients with subacute sclerosing panencephalitis concerning alternative medicine.

Isikay S. The behavior pattern of parents of patients with subacute sclerosing panencephalitis concerning alternative medicine. Turk J Pediatr 2017; 59: 288-294. The aim of the study was to examine the attitude of the parents of Subacute Sclerosing Panencephalitis (SSPE) patients regarding alternative treatment methods and compare with those of the parents of epilepsy patients. The study comprised 39 SSPE and 53 epilepsy patients who were under follow-up in Gaziantep Children`s Hospital. A questionnaire designed to inquire about the knowledge (13 questions) and behavior (11 questions) of parents about alternative medicine methods was given to the caregiver of all patients. The ratio of parents using alternative medicine methods was 29/39 (74.4%) in the SSPE group and 8/53 (15.1%) in the epilepsy group. Less than half of the parents of SSPE patients reported talking about it with their doctors. These results show parents facing a chronic debilitating disease frequently seek benefit from alternative methods. Most define this treatment as complementary to the established medical treatment. However, potential and unrecognized adverse events of alternative methods and their interference with regular medical treatment can be of importance, especially because treating physicians are seldom informed about concurrent use.

Measles-induced encephalitis.

Encephalitis is the most frequent neurological complication of measles virus infection. This review examines the pathophysiology of measles infection and the presentations, diagnosis and treatment of the four types of measles-induced encephalitis including primary measles encephalitis, acute post-measles encephalitis, measles inclusion body encephalitis and subacute sclerosing panencephalitis. The early symptoms of encephalitis may be non-specific and can be mistakenly attributed to a systemic infection leading to a delay in diagnosis. This review provides a summary of the symptoms that should cause health care workers to suspect measles-induced encephalitis.

Subacute Sclerosing Panencephelitis in Pregnancy.

Subacute sclerosing panencephalitis (SSPE) is a rare delayed complication of measles virus infection in infancy. We present here 7 month pregnant lady who had SSPE. She delivered low birth-weight baby prematurely which died on 3rd day of delivery. Patient died due to sepsis one month after admission.

Current therapies and future perspectives in subacute sclerosing panencephalitis.

Subacute sclerosing panencephalitis is a progressive neurological disorder of children and young adults caused by a measles virus that became defective by persisting in the host. According to the results of clinical trials, antiviral and/or immunomodulatory therapy can slow the progression of the disease and improve life expectancy in patients. However, its long-term effects and eventual outcome remain debatable due to conflicting results and its lack of effect on the rapidly progressive form of the disease. Possible future therapies for subacute sclerosing panencephalitis are RNAi and antiapoptotic agents, which are currently in the hypothetical and experimental stages of research.

Subacute sclerosing panencephalitis (SSPE) the story of a vanishing disease.

Subacute sclerosing panencephalitis (SSPE), is a devastating "slow virus" brain disease which affects young children who had measles some 6-7 years earlier. Although, the pandemic of SSPE during 1960-1980's was almost eradicated due to mass immunization, the disease is still taking the life of young children in countries where measles immunization is incomplete and in world regions where genetic polymorphism to this particular infection is present. The present review was written for the fortunate young generation of pediatricians and pediatric neurologists who probably have not seen a case of SSPE during their career, and for those who work in counties where the disease has not been eradicated. It is also a reminder that with full coverage of measles immunization this devastating disease can be fully eradicated.

Subacute sclerosing panencephalitis: a case report.

Subacute sclerosing panencephalitis (SSPE) is a progressive neurological disorder of childhood and early adolescence caused by persistent defective measles virus. Clinical manifestations appear many years after the acute measles infection. The incidence of SSPE has substantially declined after the introduction of an effective vaccine. We report a case of a child with SSPE that began with atonia, dysarthria, and intellectual deterioration without the presence of any particular EEG anomalies. We have reported this girl who was affected by this severe affliction in the hope that, because of the rarity of SSPE, it would not go undiagnosed.

Subacute sclerosing panencephalitis.

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.

[Epidemiologic features of subacute sclerosing panencephalitis from clinical data of patients receiving a public aid for treatment].

In 1999, clinical data of 125 patients with subacute sclerosing panencephalitis (SSPE) were obtained by the Research Committee from local prefectural governments. The data were made by physicians treating the patients, and were submitted to the governments when the patients applied for the aid. By analyzing the data, we observed the epidemiologic features of the disease in Japan, and discussed the availability of the data as the source of epidemiologic researches. Of the 125 patients, 66 were males and 59 were females. The distribution of age at onset had a peak in 5-14 years of age with the average of 10.3 years. Among the 109 cases in which the time of infection was obvious, more than 80% suffered from measles before 2 years of age, in agreement with the hypothesis that measles infection in young age is a risk factor of SSPE. The interval between measles and the onset of SSPE was between 5 and 10 years in most cases, with average of 8.8 years, median of 4.3 years, ranging from 2 months to 23.6 years. Because the data contain some problems, we have to observe the epidemiologic features of SSPE in Japan based on multiple data sources including this one, considering their advantages and disadvantages.

Retinitis and dementia in a pregnant girl: an unusual case.

A 14-year-old pregnant Caucasian girl presented with a 1-week history of dementia. She had presented 1 year prior with acute unilateral visual impairment and was noted to have macular degeneration of presumed infective aetiology. This evolved to a pigmentary macular lesion. During the course of the current presentation she developed myoclonic jerks. An electroencephalogram revealed periodic spike and slow wave complexes at 1-2 second intervals. Blood and cerebrospinal fluid examination showed raised anti-measles IgG antibody titres. Intrathecal synthesis of anti-measles virus antibody was demonstrated unequivocally and a diagnosis of subacute sclerosing panencephalitis was made. A healthy male infant was delivered by elective caesarean section at 33 weeks' gestation. She continued to deteriorate clinically despite treatment with intraventricular alpha-interferon. She had not had primary immunization against measles.