Enteroviral infections in primary immunodeficiency (PID): a survey of morbidity and mortality.

We have explored the natural history of enteroviral infection in patients with primary antibody deficiency by surveying both published and unpublished case reports before the new anti-enteroviral drug, pleconaril, was available. Many different enteroviruses were involved, Echovirus 11 being the most common. The central nervous system was nearly always involved, with evidence of systemic involvement of muscle and/or liver and/or joints in about 40% of patients. Neurological symptoms and signs varied with minor or no changes in the cerebro-spinal fluid. There was high morbidity and mortality; a third of polio cases (usually vaccine related), and nearly half of non-polio infected patients died; about 40% of survivors of the initial illness had long-term neurological symptoms. Prophylactic immunoglobulin therapy did not prevent infection but these patients were on lower doses as compared to current recommendations. Our data provides a useful background for assessing the efficacy of new anti-viral treatment in this condition.