Teaching Video NeuroImages: An unusual case of fulminant subacute sclerosing panencephalitis
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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
(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.↵
- Garg RK
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