Periodic Jaw-Opening Myoclonus in Subacute Sclerosing Panencephalitis.
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ABSTRACT:
SUBMITTER: Garg D
PROVIDER: S-EPMC10236020 | biostudies-literature | 2023 May
REPOSITORIES: biostudies-literature
Publications
Periodic Jaw-Opening Myoclonus in Subacute Sclerosing Panencephalitis.
Garg Divyani D Kumar Ashna A Sharma Suvasini S
Journal of movement disorders 20230426 2
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Project description:We present a case of subacute sclerosing panencephalitis that developed in a previously healthy 29-year-old pregnant woman who had returned from a trip to rural India shortly before the onset of symptoms. She was admitted to hospital at 27 weeks' gestation with a history of cognitive decline and difficulty completing simple tasks. She had no clinical signs of infection. The working diagnosis was autoimmune encephalitis, although extensive investigations did not lead to a final classifying diagnosis. The patient became comatose and developed hypertension, and an emergency caesarean section was done at 31 weeks to deliver the child, who seemed healthy. The patient died about 6 weeks after the onset of symptoms. The patient was found to have had subacute sclerosing panencephalitis at autopsy. In this Grand Round, we review the clinical features and treatment of subacute sclerosing panencephalitis, and the epidemiological and public health aspects of the case.
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Project description: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.
| S-EPMC9209915 | biostudies-literature
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.
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Project description:We report two cases of subacute sclerosing panencephalitis (SSPE) which is a rare, late neurological sequel, of measles infection acquired in childhood. The diagnosis was reached by adopting criteria defined by Dyken. Past history of measles was present in both the cases and they showed high titres of measles specific antibody in CSF and serum by quantitative ELISA.
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Project description:Subacute sclerosing panencephalitis (SSPE) is still a common disease in India which is characterized by a progressive mental decline, myoclonus, periodic encephalographic abnormalities, and raised anti-measles antibody titter in the cerebrospinal fluid. Acute fulminant SSPE is characterized by a rapid course of disease culminating in death, within 6 months. We report of a 10-year-old boy, who came with a 14-day history of continuous involuntary jerky movements of the left half of the body, including the head. There was a highly increased anti-measles IgG antibody titer, both in the cerebrospinal fluid and serum. We conclude that acute rapidly progressive SSPE can present as acute encephalitis syndrome.
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Project description:Subacute sclerosing panencephalitis (SSPE) occurs in some individuals after measles infection, following a symptom-free period of several years. It resembles chronic traumatic encephalopathy (CTE), which happens after repetitive head impacts or exposure to blast waves, following a symptom-free period. As in CTE, the neurofibrillary changes of SSPE are concentrated in superficial cortical layers. Here we used electron cryo-microscopy (cryo-EM) of tau filaments from two cases of SSPE to show that the tau folds of SSPE and CTE are identical. Two types of filaments were each made of two identical protofilaments with an extra density in the β-helix region. Like in CTE, the vast majority of tau filaments were Type I, with a minority of Type II filaments. These findings suggest that the CTE tau fold can be caused by different environmental insults, which may be linked by inflammatory changes.
| S-EPMC10161654 | biostudies-literature
Project description:Subacute sclerosing panencephalitis (SSPE) is a neurodegenerative disorder because of the persistence of mutated measles virus in the central nervous system. Till date, no curative therapy has been established for SSPE. Multiple drugs have been tried to modify the disease process but have shown mild to moderate benefit at best. It is also challenging to attribute the relative success of some strategies described in single case reports because of the known phenomenon of spontaneous improvement in 5% of patients with SSPE. Critical gaps in understanding the pathophysiological processes involved exist. Current therapies such as interferon alfa require invasive strategies for administration by the intraventricular or intrathecal route, with varying dosage regimens. Oral therapies such as isoprinosine and ribavirin are expensive and not readily available in resource-constrained settings. Most of the evidence so far favors the use of combinational regimens. In this viewpoint, we critically summarize the current evidence on disease-modifying strategies in the context of our region.
| S-EPMC10081548 | biostudies-literature
Project description:In posterior reversible encephalopathy syndrome, brain magnetic resonance imaging (MRI) reveals bilateral occipital, parietal, and subcortical white matter hyperintensities on T2/fluid-attenuated inversion recovery (FLAIR) sequences. After treatment, imaging abnormalities are usually reversible. Eclampsia is the most frequent cause of posterior reversible encephalopathy syndrome in pregnancy. We report a 24-year-old woman, who presented to our clinic 4 weeks after normal vaginal delivery with bilateral vision loss. Loss of vision was first noticed in the 20th week of pregnancy. Even after delivery, her vision did not improve. In the postpartum period, she started having periodic myoclonic jerks. Electroencephalography demonstrated periodic generalized discharges. A brain MRI performed in the 20th week of the antepartum period showed bilateral parieto-occipital cortical white matter T2/FLAIR hyperintensities. A follow-up brain MRI, 5 months later, revealed marked reversal of white matter signal changes. Cerebrospinal fluid examination revealed raised anti-measles antibody titer, confirming the diagnosis of subacute sclerosing panencephalitis. In conclusion, in a patient with subacute sclerosing panencephalitis (SSPE) during the postpartum period, cortical vision loss and parieto-occipital white matter T2/FLAIR hyperintensities can simulate eclampsia. Inadvertent treatment with magnesium sulfate is likely if the diagnosis is missed.
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