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Meningoencephalitis in primary antibody deficiency: Our experience from northwest India
Ankur Kumar Jindal1, Himanshi Chaudhary1, Rahul Tyagi1
1Allergy Immunology Unit, Department of Pediatrics, Advanced Pediatrics Centre, Post Graduate Institute of Medical Education and Research, Chandigarh, India.
Insights
Meningoencephalitis is an uncommon but severe complication in children with primary antibody deficiency (PAD), leading to high mortality. Early diagnosis and immunoglobulin therapy may prevent these neurological issues.
Area of Science:
- Pediatric Neurology
- Immunology
- Infectious Diseases
Background:
- Primary antibody deficiency (PAD) predisposes patients to meningoencephalitis, often attributed to enteroviruses.
- Limited literature exists on meningoencephalitis in PAD, especially from developing countries.
Purpose of the Study:
- To analyze a cohort of children with PAD who developed meningoencephalitis.
- To describe the clinical presentation, diagnostic findings, and outcomes of meningoencephalitis in children with PAD.
Main Methods:
- Retrospective analysis of children diagnosed with PAD and meningoencephalitis.
- Review of clinical data, laboratory results, neuroimaging (MRI), and treatment outcomes.
Main Results:
- Meningoencephalitis occurred in 10.4% of children with PAD (13/135).
- Common variable immunodeficiency (CVID) and X-linked agammaglobulinemia (XLA) were the most frequent PAD types. Presenting symptoms included seizures, neurodevelopmental delay, and regression. MRI revealed gray matter hyperintensities and cerebral atrophy. Mortality was high (8/11), with survivors experiencing neurological sequelae.
Conclusions:
- Meningoencephalitis is an uncommon but serious complication of PAD with significant morbidity and mortality.
- Prompt diagnosis of immune deficiency and initiation of immunoglobulin replacement therapy are crucial for preventing neurological complications.
Background/Objectives:
Patients with primary antibody deficiency (PAD) are predisposed to develop meningoencephalitis, often considered to be enteroviral. However, there is a paucity of literature on this subject, and there are no studies from developing countries.
Methods:
We analyzed our cohort of children with PAD who developed meningoencephalitis.
Results:
This complication was observed in 13/135 (10.4%) patients with PAD - 5 patients had X-linked agammaglobulinemia (XLA), 7 had common variable immunodeficiency (CVID) and 1 had suspected nuclear factor kappa B essential modulator (NEMO) defect. Mean age at onset of neurological illness was 9.3 years. Presenting features included seizures (n=8), neurodevelopmental delay (n=2), regression of milestones (n=2), and acute flaccid paralysis (n=1). Trough IgG levels were found to be low in 12/13 patients at the time of development of neurological symptoms. Herpes simplex virus (HSV), cytomegalovirus (CMV), and Streptococcus pneumoniae were isolated in 1 each. Eight (72.7%) patients had altered signal hyperintensities in gray matter and deep white matter on magnetic resonance imaging (MRI), while 4 patients showed global cerebral atrophy. All patients were treated with high-dose intravenous immunoglobulin (IVIg). Fluoxetine was given to 3 patients. Eight patients in the present series have died, 3 have recovered with varying degrees of neurological sequelae and 2 patients are showing gradual recovery.
Conclusions:
To conclude, meningoencephalitis is an uncommon complication in patients with PAD and is associated with high morbidity and mortality. Early diagnosis of immune deficiency and initiation of replacement immunoglobulin therapy may prevent the development of neurological complications.
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