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March 12, 2019; 92 (11) Resident & Fellow Section

Teaching Video NeuroImages: An unusual case of fulminant subacute sclerosing panencephalitis

Imran Rizvi, Ravindra Kumar Garg, Amita Jain, Hardeep Singh Malhotra, Neeraj Kumar, Ravi Uniyal
First published March 11, 2019, DOI: https://doi.org/10.1212/WNL.0000000000007103
Imran Rizvi
From the Department of Neurology, King George's Medical University, Lucknow, India.
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Ravindra Kumar Garg
From the Department of Neurology, King George's Medical University, Lucknow, India.
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Amita Jain
From the Department of Neurology, King George's Medical University, Lucknow, India.
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Hardeep Singh Malhotra
From the Department of Neurology, King George's Medical University, Lucknow, India.
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Neeraj Kumar
From the Department of Neurology, King George's Medical University, Lucknow, India.
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Ravi Uniyal
From the Department of Neurology, King George's Medical University, Lucknow, India.
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Teaching Video NeuroImages: An unusual case of fulminant subacute sclerosing panencephalitis
Imran Rizvi, Ravindra Kumar Garg, Amita Jain, Hardeep Singh Malhotra, Neeraj Kumar, Ravi Uniyal
Neurology Mar 2019, 92 (11) e1270; DOI: 10.1212/WNL.0000000000007103

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A 15-year-old girl had abnormal behavior of 3 weeks duration. There was frequent eye blinking (eyelid myoclonus) and periodic movements of right lower limb (video 1). The patient had measles at 1 year of age. MRI brain showed bilateral frontal hyperintensities (figure). CSF was normal and negative for herpes simplex, varicella-zoster, Japanese encephalitis, dengue, and Epstein-Barr viruses and NMDA receptor encephalitis. CSF demonstrated increased antimeasles antibodies. We made the diagnosis of subacute sclerosing panencephalitis (SSPE).1 She became akinetic and mute within 3 months. SSPE is characterized by cognitive decline, periodic myoclonus, and periodic electroencephalographic changes.1 Typically patients with SSPE survive for 1–3 years, Our patient had a very rapid course.1

Video 1

Frequent eye blinking suggestive of myoclonia and periodic stereotyped involuntary movements of right lower limb.Download Supplementary Video 1 via http://dx.doi.org/10.1212/007103_Video_1

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Figure Imaging

(A) Fluid-attenuated inversion recovery, (B) T2, and (C) diffusion-weighted images show hyperintensities involving bilateral frontal lobes.

Author contributions

Imran Rizvi, Ravindra Kumar Garg: concept, drafting of manuscript, revision, analysis. Amita Jain: concept, drafting of manuscript, revision. Hardeep Singh Malhotra, Neeraj Kumar, Ravi Uniyal: drafting of manuscript, revision, analysis.

Study funding

No targeted funding reported.

Disclosure

I. Rizvi reports no disclosures relevant to the manuscript. R. Garg receives regular honorarium for writing clinical summaries for MedLink Neurology, MedLink Corporation, San Diego, CA. A. Jain, H. Singh Malhotra, N. Kumar, and R. Uniyal report no disclosures relevant to the manuscript. Go to Neurology.org/N for full disclosures.

Footnotes

  • Teaching slides links.lww.com/WNL/A835

  • © 2019 American Academy of Neurology

Reference

  1. 1.↵
    1. Garg RK
    . Subacute sclerosing panencephalitis. Postgrad Med J 2002;78:63–70.
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