Cryptococcal Neoformans and Varicella Zoster Meningitis in a Patient With Selective Innate Immunodeficiency: A Case...

Area of Science:

• Infectious Diseases
• Immunology
• Neurology

Background:

• Opportunistic infections like Cryptococcal neoformans (C. neoformans) and varicella-zoster (VZV) meningitis primarily affect immunocompromised individuals (e.g., HIV, cancer, transplant recipients).
• The predisposition to these infections due to innate immunodeficiencies, rather than acquired ones, remains less understood.
• Idiopathic hypereosinophilic syndrome is a rare condition characterized by persistently high eosinophil counts without a clear cause.

Observation:

• A young male with idiopathic hypereosinophilic syndrome and hypocomplementemia presented with symptoms of C. neoformans and VZV meningitis.
• Clinical presentation included headache, myalgia, joint pain, insomnia, night sweats, fever, and vesicular skin lesions.
• Diagnostic workup confirmed disseminated C. neoformans and VZV meningitis, with evidence of fungemia and VZV skin infection.

Findings:

• The patient exhibited idiopathic hypereosinophilia, high IgE levels, low complement levels, and high rheumatoid factor.
• Immune evaluation revealed HIV-negativity, normal CD4 count, high IgE, and a low CD16/56 level, suggesting potential natural killer (NK) cell dysfunction.
• The patient responded favorably to antiviral (acyclovir), antifungal (amphotericin, flucytosine), and maintenance therapy (fluconazole).

Implications:

• This case underscores that innate immunodeficiencies, such as those potentially linked to hypereosinophilic syndrome and NK cell dysfunction, can predispose individuals to severe opportunistic infections.
• It highlights the importance of thorough immune evaluation in patients presenting with unusual or disseminated infections, even in the absence of typical risk factors.
• The successful treatment outcome emphasizes the efficacy of targeted antimicrobial and supportive care in managing disseminated cryptococcal and VZV meningitis in complex immunological contexts.