Persistent and Fatal Central-Nervous-System ECHOvirus Infections in Patients with Agammaglobulinemia

Catherine M. Wilfert, Rebecca H. Buckley, T. Mohanakumar, John F. Griffith, Samuel L. Katz, John K. Whisnant, Peyton A. Eggleston, Marcie Moore, Edward Treadwell, Michael N. Oxman, Fred S. Rosen

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181 Scopus citations


We observed persistent ECHOvirus infection of the central nervous system, as defined by continued presence of isolatable virus in cerebrospinal fluid, in five patients with agammaglobulinemia. The immunologic deficit in each was characterized by absence of surface-immunoglobulin-bearing B lymphocytes and of lymph-node cortical follicles, but normal T-cell function. ECHOviruses 30, 19, 9 and 33 were recovered from cerebrospinal fluid for periods varying from two months to three years. The patients had few signs of acute Central-nervous-system infection. Three of the five patients had a dermatomyositis-like syndrome, with peripheral lymphocytes that reacted with anti-human leukemia-specific primate and rabbit serums in a cytotoxicity assay. These data suggest that intact B-cell function is essential for eradication of ECHOvirus infection of the central nervous system. (N Engl J Med 296:1485–1489, 1977) Children with either infantile X-linked or common variable forms of agammaglobulinemia are extremely susceptible to infections with pyogenic bacteria. Recurrent bacterial disease can be reduced considerably in these patients by administration of human immune serum globulin or normal plasma. Primarily because these patients have intact cell-mediated immunity it has been thought that such agammaglobulinemic states do not ordinarily predispose to an unusual frequency of, or serious complications from, viral infections. However, an increased incidence of paralytic poliomyelitis resulting from either wild-type or live, attenuated vaccine virus has been reported among such patients.1 2 3 The persistent Central-nervous-system ECHOvirus infections occurring in the.

Original languageEnglish (US)
Pages (from-to)1485-1489
Number of pages5
JournalNew England Journal of Medicine
Issue number26
StatePublished - Jun 30 1977

ASJC Scopus subject areas

  • General Medicine


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