Abstract
Chronic enteroviral meningoencephalitis is a well-recognized complication in patients with X-linked agammaglobulinemia (XLA). The majority of published cases refers to its occurrence in patients on no replacement therapy or on only intramuscular immunoglobulin. The advent of intravenous immunoglobulin (IVIg) in the early 1980s and its widespread use in XLA was thought to have virtually eradicated enteroviral meningoencephalitis in these patients. We describe the development of echovirus meningoencephalitis in an 11-year-old boy on regular IVIg replacement whose serum IgG levels were maintained at between 6 and 8 g/L (NR 6–13 g/L). Treatment with daily high-dose IVIg was commenced, with significant clinical improvement being noted within a few weeks in association with a reduction in blood-brain barrier permeability. The persistence of live virus, however, necessitated the use of intraventricular immunoglobulin. The virus proved resistant to two courses of specific intraventricular immunoglobulin and a 6-week course of oral ribavirin and eventually proved fatal 5 months after presentation. In view of the therapeutic uncertainties we have reviewed the use of immunoglobulin in the treatment of enteroviral meningoencephalitis over the past 6 years.
Similar content being viewed by others
References
McKinney RE, Katz SL, Wilfert CM: Chronic enteroviral meningoencephalitis in agammaglobulinaemic patients. Rev Infect Dis 9:334–356, 1987
McCormick JB, King IJ, Webb PA,et al.: Lassa fever—Effective therapy with ribavirin. N Engl J Med 314:20–26, 1986
Nagington J, Gandy G, Walker J, Gray JJ: Use of normal immunoglobulin in an echovirus 11 outbreak in a special care baby unit. Lancet 2:443–446, 1983
Mease PJ, Ochs HD, Wedgewood RJ: Successful treatment of echovirus meningoencephalitis and myositis-fasciitis with intravenous immunoglobulin therapy in a patient with X-linked agammaglobulinaemia. N Engl J Med 304:1278–1281, 1981
Bernatowska E, Madalinski K: Intravenous immunoglobulin therapy of progressive encephalitis in X-linked hypogamma-globulinaemia. Acta Paediat Scand 76:155–156, 1987
Mease PJ, Ochs HD, Corey L, Dragavon J, Wedgwood RJ: Echovirus encephalitis/myositis in X-linked agammaglobulinaemia. N Engl J Med 313:758, 1985 (letter)
Lau YL, Levinsky RJ, Morgan G, Strobel S: Dual meningoencephalitis with echovirus type 11 and adenovirus in combined (common variable) immunodeficiency. Paediat Infect Dis J 7:873–876, 1988
Kondoh H, Kobayashi K, Sugio Y, Hayashi T: Successful treatment of echovirus meningoencephalitis in X-linked agammaglobulinaemia by intrathecal and intravenous injection of high titre gammaglobulin. Eur J Paediat 146:610–612, 1987
Erlendsson K, Swartz T, Dwyer JM: Successful reversal of echovirus encephalitis in X-linked hypogammaglobulinaemia by intraventricular administration of immunoglobulin. N Engl J Med 312:351–353, 1985
Dwyer JM, Erlendsson K: Intraventricular gammaglobulin for the management of enterovirus encephalitis. Paediat Infect Dis J 7:S30-S33, 1988
Johnson PR, Edwards KM, Wright PF: Failure of intraventricular gammaglobulin to eradicate echovirus encephalitis in a patient with X-linked agammaglobulinaemia. N Engl J Med 313:1546–1547, 1985 (letter)
Gilbert BE, Knight V: Biochemistry and clinical applications of ribavirin. Antimicrob Agents Chemother 30:201–205, 1986
Crumpacker C, Bubley G, Lucey D, Hussey S, Connor J: Ribavirin enters cerebrospinal fluid. Lancet 2:45–46, 1986
Crennan JM, Van Scoy RE, McKenna CH, Smith TF: Echovirus polymyositis in patients with hypogammaglobulinaemia. Am J Med 81:35–42, 1986
Hadfield MG, Seidlin M, Houff SA, Adair CF, Markowitz SM, Straus SE: Echovirus meningomyeloencephalitis with administration of intrathecal immunoglobulin. J Neuropathol Exp Neurol 44:520–529, 1985
Prentice RL, Dalgleish AG, Gatenby PA, Lobbay RH, Wade S, Kappagoda N, Basten A: Central nervous system echovirus infection in Bruton's X-linked hypogammaglobulinaemia. Aust NZ J Med 15:443–445, 1985
Roberton DM, Jack I, Joshi W, Law F, Hosking CS: Failure of intraventricular gammaglobulin and alpha interferon for persistent encephalitis in congenital hypogammaglobulinaemia. Arch Dis Child 63:948–952, 1988
Van Maldergem L, Mascart F, Ureal D, Jauniaux E, Broeckx W, Vainsel M: Echovirus meningoencephalitis in X-linked Hypogammaglobulinaemia. Acta Paediatr Scand 78:325–326, 1989
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Misbah, S.A., Spickett, G.P., Ryba, P.C.J. et al. Chronic enteroviral meningoencephalitis in agammaglobulinemia: Case report and literature review. J Clin Immunol 12, 266–270 (1992). https://doi.org/10.1007/BF00918150
Accepted:
Issue Date:
DOI: https://doi.org/10.1007/BF00918150