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Persistent and fatal central-nervous-system ECHOvirus infections in patients with agammaglobulinemia
Abstract:
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.
Insights
Persistent ECHOvirus infection of the central nervous system occurred in patients with agammaglobulinemia, lacking B lymphocytes. This suggests B-cell function is crucial for clearing ECHOvirus from the CNS.
Area of Science:
- Virology
- Immunology
- Neurology
Background:
- Agammaglobulinemia is a primary immunodeficiency characterized by a severe lack of B lymphocytes and antibodies.
- Enteric Cytopathogenic Human Orphan (ECHO) viruses can cause central nervous system (CNS) infections.
Observation:
- Persistent ECHOvirus infection of the CNS was observed in five patients with agammaglobulinemia.
- These patients lacked surface-immunoglobulin-bearing B lymphocytes but had normal T-cell function.
- ECHOviruses were isolated from cerebrospinal fluid for extended periods (months to years).
Findings:
- Patients exhibited minimal acute CNS infection symptoms.
- Three patients developed a dermatomyositis-like syndrome.
- Peripheral lymphocytes showed reactivity in cytotoxicity assays.
Implications:
- Intact B-cell function appears essential for the eradication of ECHOvirus CNS infections.
- This highlights the critical role of humoral immunity in controlling viral infections of the nervous system.
- Understanding this link may inform therapeutic strategies for immunodeficient patients.