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Case Report: Focal Myoclonus with a Striatal Lesion as a Presentation of Subacute Sclerosing Panencephalitis.

ABSTRACT: Adult-onset subacute sclerosing panencephalitis (SSPE) is rare, and focal myoclonus as a presenting feature poses a diagnostic dilemma. We report an adult SSPE patient with unusual clinical and radiological features. A 20-year-old girl had jerky neck movement 9 months earlier, which progressed to left hemimyoclonus in 2 months and generalized frequent myoclonus and fall at 4 months. By 6 months, she was bedbound. On examination, her Mini-Mental State Examination score was 10, and patchy retinitis was observed around the macula. Magnetic resonance imaging revealed corpus striatal involvement and electroencephalography showed periodic discharges. Measles cerebrospinal fluid/serum immunoglobulin G index was 3.3 (normal < 1.3), confirming the diagnosis of SSPE. SSPE should also be considered in adults having focal myoclonus with corpus striatal lesion. EEG is helpful in the diagnosis.

SUBMITTER: Kalita J

PROVIDER: S-EPMC9209915 | biostudies-literature | 2022 May

REPOSITORIES: biostudies-literature

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Case Report: Focal Myoclonus with a Striatal Lesion as a Presentation of Subacute Sclerosing Panencephalitis.

Kalita Jayantee J Chaudhary Sarvesh K SK Kumar Bishwanath B Jadhav Mahesh M

The American journal of tropical medicine and hygiene 20220509

Adult-onset subacute sclerosing panencephalitis (SSPE) is rare, and focal myoclonus as a presenting feature poses a diagnostic dilemma. We report an adult SSPE patient with unusual clinical and radiological features. A 20-year-old girl had jerky neck movement 9 months earlier, which progressed to left hemimyoclonus in 2 months and generalized frequent myoclonus and fall at 4 months. By 6 months, she was bedbound. On examination, her Mini-Mental State Examination score was 10, and patchy retiniti ...[more]

PMID: 35533695

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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

Case Report: Focal Myoclonus with a Striatal Lesion as a Presentation of Subacute Sclerosing Panencephalitis.

Publications

Case Report: Focal Myoclonus with a Striatal Lesion as a Presentation of Subacute Sclerosing Panencephalitis.

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