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November 20, 2012; 79 (21) Article

Atypical manifestations and poor outcome of herpes simplex encephalitis in the immunocompromised

Ik L. Tan, Justin C. McArthur, Arun Venkatesan, Avindra Nath
First published November 7, 2012, DOI: https://doi.org/10.1212/WNL.0b013e3182752ceb
Ik L. Tan
From the Department of Neurology (I.L.T., J.C.M., A.V.), Johns Hopkins School of Medicine, Baltimore; and Section of Infections of the Nervous System (A.N.), National Institute of Neurological Disorders and Stroke, NIH, Bethesda, MD.
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Justin C. McArthur
From the Department of Neurology (I.L.T., J.C.M., A.V.), Johns Hopkins School of Medicine, Baltimore; and Section of Infections of the Nervous System (A.N.), National Institute of Neurological Disorders and Stroke, NIH, Bethesda, MD.
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Arun Venkatesan
From the Department of Neurology (I.L.T., J.C.M., A.V.), Johns Hopkins School of Medicine, Baltimore; and Section of Infections of the Nervous System (A.N.), National Institute of Neurological Disorders and Stroke, NIH, Bethesda, MD.
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Avindra Nath
From the Department of Neurology (I.L.T., J.C.M., A.V.), Johns Hopkins School of Medicine, Baltimore; and Section of Infections of the Nervous System (A.N.), National Institute of Neurological Disorders and Stroke, NIH, Bethesda, MD.
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Citation
Atypical manifestations and poor outcome of herpes simplex encephalitis in the immunocompromised
Ik L. Tan, Justin C. McArthur, Arun Venkatesan, Avindra Nath
Neurology Nov 2012, 79 (21) 2125-2132; DOI: 10.1212/WNL.0b013e3182752ceb

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ABSTRACT

Objective: To characterize clinical features, neuroimaging, and outcomes of herpes simplex encephalitis (HSE) in immunocompromised individuals.

Methods: We performed a retrospective case control review of patients diagnosed with HSE. Adult patients were dichotomized into immunocompromised (n = 14) and immunocompetent groups (n = 15).

Results: Fewer immunocompromised patients presented with prodromal symptoms and focal deficits. While the majority of CSF profiles in the immunocompromised patients were mononuclear cells predominant, 3 had polymorphonuclear predominance and another 3 had normal profiles. MRI showed widespread cortical involvement, with brainstem or cerebellar involvement in some. Two immunocompromised patients had recurrent HSE. The immunosuppressed state was associated with a decrease in Karnofsky Performance Status Scale (KPSS) score of 23.1 (p = 0.018). Every 1-day delay in initiation of acyclovir was associated with a decrease in KPSS of 10.2 (p = 0.002), and every 10 cell/mm3 increase of CSF leukocytosis was associated with an increase in KPSS of 0.7 (p = 0.009). Mortality rate was 6 times higher in the immunocompromised patients.

Conclusions: Immunocompromised states may predispose to HSE with atypical clinical and neuroradiologic features. Immunocompromised individuals with HSE have significantly worse outcomes and mortality. Early diagnosis and treatment is associated with improved outcome. The findings are particularly important in light of the increasing use of potent immunosuppressive and immunomodulatory therapies.

GLOSSARY

HSE=
herpes simplex encephalitis;
HSV=
herpes simplex virus;
JHH=
Johns Hopkins Hospital;
KPSS=
Karnofsky Performance Status Scale;
MS=
multiple sclerosis

Footnotes

  • Study funding: Supported in part by NIH grants 1P30MH075673, UL1RR025005, R01NS056884, 1P30MH075673, and NS44807 (J.C.M.), National Institute of Neurological Disorders and Stroke intramural funds, and the Steg family.

  • Go to Neurology.org for full disclosures. Disclosures deemed relevant by the authors, if any, are provided at the end of this article.

  • Supplemental data at www.neurology.org

  • Received April 13, 2012.
  • Accepted July 23, 2012.
  • © 2012 American Academy of Neurology
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Letters: Rapid online correspondence

  • Response to Dr. Tenser
    • Avindra Nath, Chief, Section of Infections of the Nervous System, NINDS, NIHnatha@ninds.nih.gov
    Submitted April 03, 2013
  • Acyclovir Resistant HSV in Immunocompromised Patients
    • Richard B. Tenser, Physician, Penn State University College of Medicinertenser@psu.edu
    • Richard B. Tenser, Hershey, PA
    Submitted December 21, 2012
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