Prognosis and demographic characteristics of SSPE patients in Istanbul, Turkey

Güler, Serhat; Küçükkoç, Mehmet; İşcan, Akın

doi:10.1016/j.braindev.2014.09.006

Cited by 20 publications

(20 citation statements)

References 18 publications

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“…Between 1975 and 1987, a registry recorded 401 patients in Turkey . Guler and colleagues, in 2015, presented data 64 patients that were seen between 2007 and 2013 . In Papua New Guinea, between 1988 and 1991, 87 confirmed cases were reported .…”

Section: Epidemiologymentioning

confidence: 99%

Subacute sclerosing panencephalitis

Garg

Mahadevan

Malhotra

et al. 2019

Reviews in Medical Virology

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Summary 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.

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Section: Epidemiologymentioning

confidence: 99%

Subacute sclerosing panencephalitis

Garg

Mahadevan

Malhotra

et al. 2019

Reviews in Medical Virology

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“…By remaining in cells, the virus evades the host's immune response. 23,[26][27][28] Periodic myoclonus often leads to difficulty in walking and repeated falls. 23 Clinical course of SSPE SSPE typically begins with behavioural and intellectual disability followed by paroxysmal movements, myoclonic jerks, and/or negative myoclonia (head drops).…”

Section: Reasons For Poor Vaccinationmentioning

confidence: 99%

“…30 TREATMENT AND OUTCOMES SSPE is usually a life-threatening disease with no cure, despite the development of antiviral and immunomodulator drugs. 27 In a survey of 500 patients with SSPE from seven countries, physicians reported that isoprinosine monotherapy and isoprinosine plus ribavirin were the standard treatments, but also intravenous immunoglobulin therapy, intrathecal a-interferon (a-IFN), and amantadine therapy. 27 In a survey of 500 patients with SSPE from seven countries, physicians reported that isoprinosine monotherapy and isoprinosine plus ribavirin were the standard treatments, but also intravenous immunoglobulin therapy, intrathecal a-interferon (a-IFN), and amantadine therapy.…”

Section: Brain Biopsymentioning

confidence: 99%

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

Mekki

Eley

Hardie

et al. 2019

Develop Med Child Neuro

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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.

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“…The incubation period between primary infection and symptom onset is typically 7–10 years. The clinical course is usually protracted, lasting 1–3 years, progressing through four stages beginning with personality change and deterioration in school performance, followed by myoclonic jerks, seizures and dementia; progressing to extrapyramidal symptoms and rigidity; and culminating in coma and death . The EEG typically shows periodic complexes (not present in our case).…”

Section: Discussionmentioning

confidence: 73%