Familial Subacute Sclerosing Panencephalitis Associated With Short Latency

Sharma, Vinod; Gupta, Vineet Bhushan; Eisenhut, Michael

doi:10.1016/j.pediatrneurol.2007.10.013

Cited by 12 publications

(6 citation statements)

References 12 publications

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“…Recently an association between programmed cell death protein 1 (PD-1), a member of the CD28 family, and children with SSPE has been reported [17]. Individuals with acquired immunodeficiency syndrome or children whose mothers have acquired immunodeficiency syndrome might be at higher risk of a fulminant course of SSPE [18], and earlier onset of familial cases of SSPE also has been reported to present with shorter latency period [19]. …”

Section: Discussionmentioning

confidence: 99%

Subacute Sclerosing Panencephalitis in a Toddler: Changing Epidemiological Trends

Aulakh

Tiwari

2013

Case Reports in Pediatrics

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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.

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Section: Discussionmentioning

confidence: 99%

Subacute Sclerosing Panencephalitis in a Toddler: Changing Epidemiological Trends

Aulakh

Tiwari

2013

Case Reports in Pediatrics

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“…All these factors if present increase the risk of SSPE [8]. The occurrence of multiple cases in a single family suggests a genetic predisposition to SSPE [9,10]. Comparison of intrafamilial with sporadic cases of SSPE in a recent study revealed that latency of clinical manifestations in familial SSPE is significantly shorter than that in sporadic cases (median of 6.4 years versus median of 9.7 years) [9].…”

Section: Epidemiologymentioning

confidence: 99%

Subacute sclerosing panencephalitis

2008

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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.

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“…Our children are from a comparatively homogenous Melanesian group but there were two first cousins of similar age diagnosed within a year of one another. Although from a single set of close-living relatives, SSPE has been described previously in sibling and twin pairs implying at least some familial predisposition, but a clear genetic basis for susceptibility has yet to be defined [37], [38], [39], [40], [41]. Single nucleotide polymorphisms in a number of immunity-related genes have been found to be associated with SSPE in Japanese [42], [43] and Turkish [44] patients, but not in other ethnic groups [45].…”

Section: Discussionmentioning

confidence: 99%

Subacute Sclerosing Panencephalitis in Papua New Guinean Children: The Cost of Continuing Inadequate Measles Vaccine Coverage

et al. 2011

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IntroductionSubacute sclerosing panencephalitis (SSPE) is a late, rare and usually fatal complication of measles infection. Although a very high incidence of SSPE in Papua New Guinea (PNG) was first recognized 20 years ago, estimated measles vaccine coverage has remained at ≤70% since and a large measles epidemic occurred in 2002. We report a series of 22 SSPE cases presenting between November 2007 and July 2009 in Madang Province, PNG, including localized clusters with the highest ever reported annual incidence.Methodology/Principal FindingsAs part of a prospective observational study of severe childhood illness at Modilon Hospital, the provincial referral center, children presenting with evidence of meningo-encephalitis were assessed in detail including lumbar puncture in most cases. A diagnosis of SSPE was based on clinical features and presence of measles-specific IgG in cerebrospinal fluid and/or plasma. The estimated annual SSPE incidence in Madang province was 54/million population aged <20 years, but four sub-districts had an incidence >100/million/year. The distribution of year of birth of the 22 children with SSPE closely matched the reported annual measles incidence in PNG, including a peak in 2002.Conclusions/SignificanceSSPE follows measles infections in very young PNG children. Because PNG children have known low seroconversion rates to the first measles vaccine given at 6 months of age, efforts such as supplementary measles immunisation programs should continue in order to reduce the pool of non-immune people surrounding the youngest and most vulnerable members of PNG communities.

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Familial Subacute Sclerosing Panencephalitis Associated With Short Latency

Cited by 12 publications

References 12 publications

Subacute Sclerosing Panencephalitis in a Toddler: Changing Epidemiological Trends

Subacute Sclerosing Panencephalitis in a Toddler: Changing Epidemiological Trends

Subacute sclerosing panencephalitis

Subacute Sclerosing Panencephalitis in Papua New Guinean Children: The Cost of Continuing Inadequate Measles Vaccine Coverage

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