Open Access Highly Accessed Case report

CMV-associated encephalitis and antineuronal autoantibodies - a case report

Xinling Xu1, Peter Bergman27*, Thomas Willows3, Charlotte Tammik1, Marie Sund4, Tomas Hökfelt5, Cecilia Söderberg-Naucler1 and Stefania Varani6

Author Affiliations

1 Department of Medicine, Karolinska Institutet, Stockholm, Sweden

2 Department of Laboratory Medicine F68, Karolinska University Hospital and Karolinska Institutet, Huddinge, Stockholm, Sweden

3 Department of Neurology, Karolinska University Hospital Huddinge, Stockholm, Sweden

4 Department of Radiology, Karolinska University Hospital Huddinge, Stockholm, Sweden

5 Department of Neuroscience, Karolinska Institutet, Stockholm, Sweden

6 Department of Hematology and Oncology, Section of Microbiology, University of Bologna, Bologna, Italy

7 Department of Medicine, Center for Infectious Medicine (CIM), Karolinska University Hospital and Karolinska Institutet Huddinge, Stockholm, Sweden

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BMC Neurology 2012, 12:87  doi:10.1186/1471-2377-12-87

Published: 4 September 2012

Abstract

Background

Human cytomegalovirus (CMV) is an ubiquitous pathogen capable of modulating the host immune system. Immune dysfunction is common during CMV infection and includes autoimmune phenomena. Here we focus on a case of primary CMV infection associated with encephalopathy in a patient with a rudimentary spleen. We discuss diagnostic challenges and immunological aspects as well as the hypothesis that CMV may break tolerance and induce potentially encephalitogenic autoantibodies.

Case presentation

A 33-year-old woman was admitted with features of encephalitis, rapidly progressing into a catatonic state. The patient tested negative for presence of herpes simplex virus DNA in cerebrospinal fluid (CSF), and had elevated liver enzymes and hepatomegaly at computed tomography scan (CT) examination. CT scan and magnetic resonance imaging (MRI) showed only a rudimentary spleen. Initially, serum was negative for anti-CMV IgM, but borderline for anti-CMV IgG by enzyme-linked immunosorbent assay. However, a more sensitive assay resulted in a positive specific IgM Western blot profile and low IgG avidity, suggesting primary CMV infection. Further, CMV DNA was retrospectively detected in a CSF sample collected at admission. We also detected antineuronal autoantibodies, which stained GAD-positive neurons in the hippocampus. The patient was treated by a combination of prednisone, intravenous immunoglobulins (IVIg) and antivirals, which resulted in a dramatic amelioration of the patient’s neurological status. One year after admission the patient exhibited a nearly complete recovery with mild deficits in attention and memory.

Conclusions

A possible reason for the critical course of CMV infection could be the lack of a functional spleen in this patient, a condition previously associated with severe CMV infection. Prompt treatment with antiviral drugs, steroids and IVIg was most likely important for the positive outcome in this case and should be considered for similar cases of severe primary CMV infection associated with immunopathological phenomena.

Keywords:
Human cytomegalovirus; Encephalitis; Antineuronal autoantibodies