Cryptococcal Meningitis and Post-Infectious Inflammatory Response Syndrome in a Patient With X-Linked Hyper IgM Syndrome: A Case Report and Review of the Literature.

Abstract

The hyper IgM syndromes are a rare group of primary immunodeficiency. The X-linked Hyper IgM syndrome (HIGM), due to a gene defect in CD40L, is the commonest variant; it is characterized by an increased susceptibility to a narrow spectrum of opportunistic infection. A few cases of HIGM patients with Cryptococcal meningoencephalitis (CM) have been described in the literature. Herein we report the case of a young male diagnosed in infancy with HIGM who developed CM complicated by a post-infectious inflammatory response syndrome (PIIRS), despite regular immunoglobulin replacement therapy and appropriate antimicrobial prophylaxis. The patient was admitted because of a headache and CM was diagnosed through detection of Cryptococcus neoformans in the cerebrospinal fluid. Despite the antifungal therapy resulting to negative CSF culture, the patient exhibited persistent headaches and developed diplopia. An analysis of inflammatory cytokines on CSF, as well as the brain MRI, suggested a diagnosis of PIIRS. Therefore, a prolonged corticosteroids therapy was started obtaining a complete resolution of symptoms without any relapse.

• Authors: Lorenza Romani, Peter Richard Williamson, Silvia Di Cesare, Gigliola Di Matteo, Maia De Luca, Rita Carsetti, Lorenzo Figà-Talamanca, Caterina Cancrini, Paolo Rossi, Andrea Finocchi
• Journal: Frontiers in immunology (Front Immunol) Vol. 12 Pg. 708837 ( 2021) ISSN: 1664-3224 [Electronic] Switzerland
• PMID: 34335625 (Publication Type: Case Reports, Journal Article, Research Support, N.I.H., Intramural, Research Support, Non-U.S. Gov't, Review)
• Copyright: Copyright © 2021 Romani, Williamson, Di Cesare, Di Matteo, De Luca, Carsetti, Figà-Talamanca, Cancrini, Rossi and Finocchi.

Chemical References

• Adrenal Cortex Hormones

Topics

• Adrenal Cortex Hormones (therapeutic use)
• Humans
• Hyper-IgM Immunodeficiency Syndrome, Type 1 (complications)
• Male
• Meningitis, Cryptococcal (diagnostic imaging, drug therapy, etiology, immunology)
• Systemic Inflammatory Response Syndrome (diagnostic imaging, drug therapy, etiology, immunology)
• Young Adult