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Volume 9, Number 10—October 2003

Letter

West Nile Virus Meningitis in Patient with Common Variable Immunodeficiency

Suggested citation for this article

To the Editor: Infection by West Nile virus (WNV) was first recognized in the Western Hemisphere in 1999 in New York (1). Subsequently, this mosquito-borne flavivirus has spread westward and has emerged as an important cause of infectious meningoencephalitis in the United States (2). In September 2002, during a WNV epidemic in Michigan (2), a 38-year-old woman with common variable immunodeficiency (CVID) sought treatment at the University of Michigan Hospital with acute WNV-associated meningitis. Although persons with CVID are at increased risk for enteroviral meningoencephalitis, a greater susceptibility to arthropod-borne flavivirus infections has not been reported.

The patient had a history of recurrent sino-pulmonary infections and gastrointestinal giardiasis and salmonellosis; at 33 years of age, she was diagnosed with CVID that has been subsequently treated with intravenous immunoglobulin (IVIG) every 3 weeks. She was in her usual state of health until 5 days before admission, when she noted the abrupt onset of severe headache, followed by temperatures up to 39.4°C, progressive photophobia, nausea, vomiting, and a transient papular rash on her trunk and extremities.

On arrival, the patient reported marked photophobia. Physical examination showed a temperature of 40.6°C, heart rate 80 beats per minute, and blood pressure 122/70 mmHg. She had cervical tenderness to palpation and active range of motion with minimal rigidity. Small, diffuse, nontender lymphadenopathy was noted in the cervical region. No focal or global deficits were found on neurologic exam. Results of the remainder of the physical examination was unremarkable. Initial laboratory values included a peripheral leukocyte count of 3.5 K/mm3 (normal: 4.0–10.0 K/ mm3; 42% neutrophils, 52% lymphocytes, and 7% monocytes), which was unchanged from the patient’s baseline leukopenia. Her serum IgG level was 1,081 mg/dL (620–1,520 mg/dL).

Cerebrospinal fluid (CSF) sampling indicated the following: erythrocytes 3/mm3, leukocytes 77/mm3 (41% neutrophils, 51% lymphocytes, 7% histiocytes), glucose 50 mg/dL (50–70 mg/dL), and protein 75 mg/dL (15–45 mg/dL). Results of routine Gram stain, bacterial and fungal cultures, polymerase chain reaction testing for herpes simplex virus and Cryptococcus neoformans antigen were negative. Assay results of the patient’s CSF for WNV by IgM-capture enzyme-linked immunosorbent assay, performed by the Michigan Department of Community Health, were positive.

The patient was initially treated with parenteral ampicillin, ceftazidime, and acyclovir, which were discontinued within 48 hours. Her symptoms improved with routine medical support, and she was discharged on hospital day 5. We were notified of the positive CSF IgM for WNV approximately 2 weeks after the patient was discharged, at which time her symptoms had completely resolved. At a follow-up visit 3 weeks after her hospitalization, the patient had no residual symptoms of meningitis.

Patients with agammaglobulinemia, either common variable or X-linked, are known to be susceptible to recurrent infections (3). Bacterial infections are the best described; however, chronic enteroviral meningoencephalitis is also associated with deficiencies in B-cell immunity (46). Although the role of immunoglobulins in host defense against WNV infection is not completely understood, evidence suggests that humoral immunity protects against WNV infection and severe disease (79).

WNV is a single-stranded RNA virus of the family Flaviviridae. Its genome is processed to eight proteins, including the envelope (E) glycoprotein, the matrix protein, the nucleocapsid protein, and five nonstructural proteins (7). E-glycoprotein antibodies develop during human WNV infection (8), and passive immunization of mice with E-glycoprotein antiserum protects against WNV infection and death (7,8). In addition, IVIG therapy was associated with recovery from WNV meningoencephalitis of an immunosuppressed 70-year-old woman (9). Although our patient had normal levels of serum IgG at the time of illness, viral meningitis may occur in agammaglobulinemic patients despite regular IVIG therapy (10).

This case demonstrates the need to consider WNV in patients with CVID. Our patient recovered promptly, without evidence of neurologic sequelae, despite her underlying immunodeficiency. More experience is needed to provide a better understanding of the relationship between CVID and WNV.

Augusto M. Alonto*, David M. Aronoff*, and Preeti N. Malani*Comments to Author 
Author affiliations: *University of Michigan Health System, Ann Arbor, Michigan, USA

References

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  2. Centers for Disease Control and Prevention. Provisional surveillance summary of the West Nile virus epidemic—United States, January–November 2002. MMWR Morb Mortal Wkly Rep. 2002;51:112933.PubMed
  3. Sneller MC, Strober W, Eisenstein E, Jaffe JS, Cunningam-Rundles C. New insights into common variable immunodeficiency. Ann Intern Med. 1993;118:72030.PubMed
  4. Wilfert CM, Buckley RH, Mohanakumar T, Griffith JF, Katz SL, Whisnant JK, Persistent and fatal central nervous system echovirus infections in patients with agammaglobulinemia. N Engl J Med. 1977;296:14859. DOIPubMed
  5. McKinney RE, Katz SL, Wilfert CM. Chronic enteroviral meningoencephalitis in agammaglobulinemic patients. Rev Infect Dis. 1987;9:33456.PubMed
  6. Oneil KM, Pallansch MA, Winkelstein JA, Lock TM, Modlin JF. Chronic group A coxsackievirus infection in agammaglobulinemia: demonstration of genomic variation of serotypically identical isolates persistently excreted by the same patient. J Infect Dis. 1988;157:1836.PubMed
  7. Wang T, Anderson JF, Magnarelli LA, Bushmich S, Wong S, Koski RA, West Nile virus envelope protein: role in diagnosis and immunity. Ann N Y Acad Sci. 2001;951:3257. DOIPubMed
  8. Wang T, Anderson JF, Magnarelli LA, Wong SJ, Koski RA, Fikrig E. Immunization of mice against West Nile virus with recombinant envelope protein. J Immunol. 2001;167:52737.PubMed
  9. Shimoni Z, Niven MJ, Pitlick S, Bulvik S. Treatment of West Nile virus encephalitis with intravenous immunoglobulin. Emerg Infect Dis. 2001;7:759. DOIPubMed
  10. Misbah SA, Spickett GP, Ryba PC, Hockaday JM, Kroll JS, Sherwood C, Chronic enteroviral meningoencephalitis in agammaglobulinemia: a case report and literature review. J Clin Immunol. 1992;12:26670. DOIPubMed

Suggested citation for this article: Alonto AM, Aronoff DM, Malani PN. West Nile virus meningitis in patient with common variable immunodeficiency. Emerg Infect Dis [serial online] 2003 Oct [date cited]. Available from: URL: http://wwwnc.cdc.gov/eid/article/9/10/03-0195.htm

DOI: 10.3201/eid0910.030195

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Table of Contents – Volume 9, Number 10—October 2003

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Preeti N. Malani; Division of Infectious Diseases, University of Michigan Health System, 1500 E. Medical Center Drive, Room 3116, Ann Arbor, MI 48109-0378, USA; fax: (734) 769-7039





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