Critical Care: Fellow Case Report Poster - Critical Care III |

A Case of a Rare and Devastating Consequence of Childhood Measles FREE TO VIEW

Anthony Andriotis, MD; Nahreen Ahmed, MD; Anna Nolan, MD
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New York Univeristy School of Medicine, New York, NY

Copyright 2016, American College of Chest Physicians. All Rights Reserved.

Chest. 2016;150(4_S):259A. doi:10.1016/j.chest.2016.08.272
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SESSION TITLE: Fellow Case Report Poster - Critical Care III

SESSION TYPE: Affiliate Case Report Poster

PRESENTED ON: Tuesday, October 25, 2016 at 01:30 PM - 02:30 PM

INTRODUCTION: Subacute sclerosing panencephalitis (SSPE) is a rare, slow, persistent viral infection caused by defective measles virus. It typically occurs in children 6-8 years after primary measles infection and is characterized by progressive neurologic decline resulting in death within 1-3 years. We present a case of a pregnant woman with rapidly progressive SSPE.

CASE PRESENTATION: 26 year old G1P0 female presented at 36 weeks gestation with increasing confusion and changes in her personality and speech for a few weeks. Brain MRI showed extensive T2 flair hyperintensities in the white matter of the left parietal and both occipital lobes. CSF was significant for 5 WBCs, 12% lymphocytes, normal protein and glucose. EEG showed diffuse cerebral dysfunction with intermittent bursts of delta waves. The patient developed increasing lethargy, incomprehensible speech and progressive motor deficits, prompting an urgent C-section. Her serum ANA was positive, but all other autoimmune serologies were negative. A CSF autoimmune panel, paraneoplastic panel, and anti-NMDA receptor antibodies were negative. Since she had measles at the age of 11 months, measles serologies were checked - her serum IgG was positive at 12.98, and serum IgM was negative. CSF rubeola IgG was positive at 1:128 and IgM was 1:1. Therefore, the patient was diagnosed with SSPE. Despite initiation of isoprinosine, her neurologic status progressed to a persistent vegetative state within 3 weeks.

DISCUSSION: While our patient’s serologies, MRI and EEG findings are typical of SSPE, the rapidity of her disease progression was surprising. However, SSPE during pregnancy often presents acutely and fulminantly and is thought to be due to immunological and hormonal alterations of pregnancy. Treatment for SSPE is limited. Studies suggest combination therapy with isoprinosine and intraventricular interferon alfa is the most effective treatment. One small study found that this combination resulted in remission or stabilization of symptoms in 44% of patients versus less than 5% in untreated patients, however it was ineffective in those with rapidly progressive symptoms.

CONCLUSIONS: As vaccination rates decline, we must keep SSPE on our differential for patients who present such as ours did in order to allow for prompt diagnosis and initiation of potentially beneficial treatment.

Reference #1: Garg R K, Subacute sclerosing panencephalitis. Postgrad Med J 2002; 78:916 63-70

DISCLOSURE: The following authors have nothing to disclose: Anthony Andriotis, Nahreen Ahmed, Anna Nolan

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