Neuropathy in X-linked agammaglobulinemia: A systematic review and meta-analysis

X-linked agammaglobulinemia (XLA) is a type of primary immunodeficiency disorder due to mutations in the Bruton’s tyrosine kinase gene located on the long arm of the X-chromosome, increasing the patients’ susceptibility to both infectious and non-infectious neuropathies. A comprehensive review of the literature was carried out following the preferred reporting items for systematic reviews and meta-analyses guidelines, accompanied by a meta-analysis. The articles where the cases did not involve the nervous system, did not have XLA, and studies not performed on humans and review articles were excluded. The certainty of evidence and risk of selection and measurement bias were determined for each included article. All the cases of XLA with neuropathies were listed along with their detailed information. A total of 50 studies were included, encompassing 82 cases. There were 8 cases of meningitis, 11 cases of encephalitis, and 49 cases of meningoencephalitis, which included cases of infective as well as non-infective etiology, along with other less common neural manifestations such as progressive multifocal leukoencephalopathy, progressive neurodegenerative disease, and Mohr–Tranebjaerg syndrome. We analyzed the laboratory parameters and prepared the forest plots. The most advised and life-saving treatment involves early genetic diagnosis and continuous intravenous immunoglobulin replacement therapy, which can help prevent and manage neuropathies related to XLA.