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Published on: April 15, 2014
Chronic enteroviral meningoencephalitis in agammaglobulinemic patients
Abstract:
Patients with agammaglobulinemia are particularly susceptible to chronic enteroviral infections of the central nervous system. Data on 42 patients were obtained by literature review, communications with other physicians, and personal experiences. Thirty-eight patients had congenital immunodeficiencies, most frequently X-linked agammaglobulinemia. Most patients who could be assessed were receiving maintenance therapy with intramuscular gamma-globulin before their enteroviral infection. Seven patients had not been recognized as hypogammaglobulinemic before the onset of infection. The commonest pathogens were echoviruses (37 of 41 cases), especially type 11 (11 cases). Thus far, four patients have had sequential or simultaneous infections with a second enteroviral serotype. Other features of the disease have included weakness, lethargy or coma, headaches, hearing loss, seizures, ataxia, and paresthesias. Some patients have also had nonneurologic manifestations of chronic enteroviral infection, including fever, the dermatomyositis-like syndrome, edema, rashes, and hepatitis. Treatment has consisted primarily of antibody administration, either in intravenous immunoglobulin preparations or in immune plasma. Twelve patients have received intraventricular immunoglobulin through reservoir devices; six of these 12 have improved substantially, as judged by clinical criteria.
Insights
Patients with agammaglobulinemia face severe chronic enteroviral infections of the central nervous system. Prompt antibody therapy, including intraventricular immunoglobulin, shows promise for improving outcomes in these vulnerable individuals.
Area of Science:
- Immunology
- Neurology
- Infectious Diseases
Background:
- Agammaglobulinemia significantly increases susceptibility to chronic central nervous system (CNS) enteroviral infections.
- Congenital immunodeficiencies, particularly X-linked agammaglobulinemia, are common underlying conditions.
- Many patients were already on gamma-globulin therapy, yet still developed enteroviral infections.
Observation:
- Echoviruses, especially type 11, are the most frequent causative agents.
- Neurological symptoms include weakness, lethargy, coma, headaches, hearing loss, seizures, ataxia, and paresthesias.
- Non-neurological manifestations such as fever, dermatomyositis-like syndrome, edema, rashes, and hepatitis can also occur.
Findings:
- Antibody administration, via intravenous immunoglobulin or immune plasma, is the primary treatment.
- Intraventricular immunoglobulin administration through reservoir devices led to substantial clinical improvement in 50% of treated patients (6 out of 12).
- Sequential or simultaneous infections with multiple enteroviral serotypes have been observed.
Implications:
- Highlights the critical need for early diagnosis and aggressive management of enteroviral CNS infections in agammaglobulinemia patients.
- Suggests intraventricular immunoglobulin therapy as a potentially effective strategy for severe or refractory cases.
- Underscores the importance of ongoing research into optimal treatment modalities for these complex infections.
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