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Subacute Sclerosing Panencephalitis Causing Rapidly Progressive Dementia and Myoclonic Jerks in a Sexagenarian Woman.

ABSTRACT: Background:Subacute sclerosing panencephalitis (SSPE) is a disease of childhood and adolescence, but can affect adults. Rapidly progressive cognitive decline, seizures including myoclonic jerks, spasticity, ataxia, visual disturbances, and incontinence are typical manifestations. Case report:A 62-year-old woman who presented with rapidly progressive dementia and myoclonus was diagnosed with SSPE. There was resolution of the movement disorder with clonazepam and valproic acid treatment and some amelioration of cognitive decline after 3 months of therapy with interferon alfa and isoprinosine. Discussion:With the recent rise in measles cases worldwide, any increased incidence of SSPE would require vigilance for early interventions.

SUBMITTER: Reyes AJ

PROVIDER: S-EPMC6777289 | biostudies-literature | 2019

REPOSITORIES: biostudies-literature

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Subacute Sclerosing Panencephalitis Causing Rapidly Progressive Dementia and Myoclonic Jerks in a Sexagenarian Woman.

Reyes Antonio Jose AJ Ramcharan Kanterpersad K Perot Sean S Giddings Stanley Lawrence SL Rampersad Fidel F Gobin Reanna R

Tremor and other hyperkinetic movements (New York, N.Y.) 20190827

<h4>Background</h4>Subacute sclerosing panencephalitis (SSPE) is a disease of childhood and adolescence, but can affect adults. Rapidly progressive cognitive decline, seizures including myoclonic jerks, spasticity, ataxia, visual disturbances, and incontinence are typical manifestations.<h4>Case report</h4>A 62-year-old woman who presented with rapidly progressive dementia and myoclonus was diagnosed with SSPE. There was resolution of the movement disorder with clonazepam and valproic acid treat ...[more]

PMID: 31660255

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Project description:Subacute sclerosing panencephalitis (SSPE) is a progressive, lethal complication of measles caused by particular mutants of measles virus (MeV) that persist in the brain despite high levels of neutralizing antibodies. We addressed the hypothesis that antigenic drift is involved in the pathogenetic mechanism of SSPE by analyzing antigenic alterations in the MeV envelope hemagglutinin protein (MeV-H) found in patients with SSPE in relation to major circulating MeV genotypes. To this aim, we obtained cDNA for the MeV-H gene from tissue taken at brain autopsy from 3 deceased persons with SSPE who had short (3-4 months, SMa79), average (3.5 years, SMa84), and long (18 years, SMa94) disease courses. Recombinant MeVs with a substituted MeV-H gene were generated by a reverse genetic system. Virus neutralization assays with a panel of anti-MeV-H murine monoclonal antibodies (mAbs) or vaccine-immunized mouse anti-MeV-H polyclonal sera were performed to determine the antigenic relatedness. Functional and receptor-binding analysis of the SSPE MeV-H showed activity in a SLAM/nectin-4-dependent manner. Similar to our panel of wild-type viruses, our SSPE viruses showed an altered antigenic profile. Genotypes A, G3, and F (SSPE case SMa79) were the exception, with an intact antigenic structure. Genotypes D7 and F (SSPE SMa79) showed enhanced neutralization by mAbs targeting antigenic site IIa. Genotypes H1 and the recently reported D4.2 were the most antigenically altered genotypes. Epitope mapping of neutralizing mAbs BH015 and BH130 reveal a new antigenic site on MeV-H, which we designated Φ for its intermediate position between previously defined antigenic sites Ia and Ib. We conclude that SSPE-causing viruses show similar antigenic properties to currently circulating MeV genotypes. The absence of a direct correlation between antigenic changes and predisposition of a certain genotype to cause SSPE does not lend support to the proposed antigenic drift as a pathogenetic mechanism in SSPE.

Dataset Information

Subacute Sclerosing Panencephalitis Causing Rapidly Progressive Dementia and Myoclonic Jerks in a Sexagenarian Woman.

Publications

Subacute Sclerosing Panencephalitis Causing Rapidly Progressive Dementia and Myoclonic Jerks in a Sexagenarian Woman.

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