Meningoencephalitis in primary antibody deficiency: Our experience from northwest India

• Published in: Journal of neuroimmunology Vol. 371; p. 577952
• Main Authors: Jindal, Ankur Kumar, Chaudhary, Himanshi, Tyagi, Rahul, Rawat, Amit, Suri, Deepti, Patra, Pratap Kumar, Arora, Kanika, Chawla, Sanchi, Vyas, Sameer, Arora, Munish, Aggarwal, Ridhima, Basu, Suprit, Bansal, Reema, Sachdeva, Man Updesh Singh, Gupta, Anju, Pandiarajan, Vignesh, Sankhyan, Naveen, Suthar, Renu, Sahu, Jitendra, Singh, Mini, Mani, Reeta, Sharma, Rajni, Saka, Ruchi, Imai, Kohsuke, Ohara, Osamu, Nonoyama, Shigeaki, Hammarström, Lennart, Chan, Koon Wing, Lau, Yu Lung, Singh, Surjit
• Format: Journal Article
• Language: English
• Published: Elsevier B.V 15.10.2022
• Subjects: Common Variable Immunodeficiency; Enterovirus; Intravenous immunoglobulin; Medicin och hälsovetenskap; Meningoencephalitis; Primary antibody deficiency; X-linked agammaglobulinemia; Common Variable Immunodeficiency; PADs; MRI; Primary antibody deficiency; EEG; Intravenous immunoglobulin; IVIg; Enterovirus; HSV; X-linked agammaglobulinemia; XLA; IEI; CMV; Meningoencephalitis; CVID; CSF; PCR
• ISSN: 0165-5728; 1872-8421; 1872-8421
• DOI: 10.1016/j.jneuroim.2022.577952

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. We analyzed our cohort of children with PAD who developed meningoencephalitis. 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. 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. •Patients with primary antibody deficiencies (PADs) are predisposed to develop a spectrum of neurological complications including meningoencephalitis.•There is paucity of data on this aspect from large patient cohorts, especially from developing countries.•We report the largest, single center experience of a cohort of patients with PADs who developed meningoencephalitis from India.•Early diagnosis of PADs and initiation of immunoglobulin may prevent occurrence of neurological illness in patients with PADs.