Abstract
Patients with primary antibody deficiency (PAD) are predisposed to develop meningoencephalitis that is often considered to be enteroviral. However, there is a paucity of literature on this subject, and there are no studies from developing countries. We analyzed our cohort of children with PAD who developed meningoencephalitis. This complication was observed in 11/135 (8.1%) patients with PAD - 4 patients had X-linked agammaglobulinemia (XLA), and 7 had common variable immunodeficiency (CVID). The mean age at onset of neurological illness was 8.6 years (range: 2-28 years). Presenting features included seizures (n=7), neurodevelopmental delay (n=2), regression of milestones (n=1), and acute flaccid paralysis (n=1). Trough IgG levels were found to be low in 9 (81.8%) patients at the time of development of neurological symptoms. Herpes simplex virus (HSV), cytomegalovirus (CMV), and Streptococcus pneumoniae were isolated in 1 patient each. No etiological agent was identified in cerebrospinal fluid of 8 patients. Eight (72.7%) patients had altered signal hyperintensities in gray matter and deep white matter on magnetic resonance imaging (MRI), while 3 patients showed global cerebral atrophy. All patients were treated with high-dose intravenous immunoglobulin (IVIg). Fluoxetine was given to 2 patients. Eight (72.7%) patients in the present series have succumbed, while three have recovered with varying degrees of neurological sequelae. To conclude, meningoencephalitis is an uncommon complication in patients with PAD and is associated with high morbidity and mortality in our setting. Early diagnosis of immune deficiency and initiation of replacement immunoglobulin therapy may prevent the development of neurological complications.
Subcutaneous immunoglobulin loading regimens for previously untreated patients with primary antibody deficiency
