Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Case Reports
. 2019 Aug 27:9.
doi: 10.7916/tohm.v0.680. eCollection 2019.

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

Affiliations
Case Reports

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

Antonio Jose Reyes et al. Tremor Other Hyperkinet Mov (N Y). .

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.

Keywords: Subacute sclerosing panencephalitis; dementia; isoprinosine; measles; myoclonic jerks; vaccination..

PubMed Disclaimer

Conflict of interest statement

Funding: None. Conflicts of interest: The authors report no conflicts of interest. Ethics Statement: The patient who appears in the video has provided written informed consent; authorization for the recording and publication of the video was provided.

Figures

Figure 1
Figure 1
Photographs of the MRI scan of the brain of the patient with SSPE. (A) Sagittal magnetic resonance imaging (MRI) T1-weighted image of the brain showing diffuse atrophy. (B) Axial T1-weighted post-IV gadolinium showing mild subcortical enhancement in the right parietal lobe (blue arrows). (C) Axial MRI T2-weighted image of the brain showing asymmetric hyperintensities best noted in the right temporoparietal region (blue arrows). (D) Axial MRI T2-FLAIR-weighted image of the brain showing diffuse hyperintensities in mesial temporal lobes bilaterally (blue arrows). (E) Axial MRI T2-FLAIR-weighted image with hyperintensities best seen in the frontal lobes (blue arrows) bilaterally.

References

    1. Park DW, Boldt HC, Messicotte SJ, et al. . subacute sclerosing panencephalitis manifesting as viral retinitis: clinical and histopathologic findings. Am J Ophthalmol 1997;123:533–542. doi: 10.1016/S0002-93949394(14)70179-5 - DOI - PubMed
    1. Swoveland PT, Johnson KP. Subacute sclerosing panencephalitis and other paramyxovirus infections. In: Mckendall RR, editor. Handbook of clinical neurology. Vol 12 (56). Virus diseases. Amsterdam: North Holland Publishing Company; 1989, pp. 417–437.
    1. Saha V, John TJ, Mukundan P, et al. . High incidence of subacute sclerosing panencephalitis in South India. Epidemiol Infect 1990;104:151–156. doi: 10.1017/S0950268800054637 - DOI - PMC - PubMed
    1. Dyken PR. Subacute sclerosing panencephalitis. Neurol Clin 1985;3:179–195. - PubMed
    1. Elmali AD, Simsekoglu R, Sahin E, Ilki CD, et al. . Senile-onset subacute sclerosing panencephalitis, presenting with peculiar findings. Clin EEG Neurosci 2018;50(4):283–286. doi: 10.1177/1550059418793758 - DOI - PubMed

Publication types