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Related Experiment Videos

Cryptococcal meningitis in a child with hyperimmunoglobulin E syndrome

B Z Garty1, B Wolach, S Ashkenazi

  • 1Kipper Institute of Pediatric Allergy and Immunology, Department of Pediatrics, Petah Tiqva, Israel.

Pediatric Allergy and Immunology : Official Publication of the European Society of Pediatric Allergy and Immunology
|August 1, 1995
PubMed
Summary

Patients with hyperimmunoglobulin E syndrome face increased risks of fungal infections like cryptococcal meningitis. This case highlights challenges in managing increased intracranial pressure in such infections.

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Area of Science:

  • Infectious Diseases
  • Immunology
  • Neurology

Background:

  • Hyperimmunoglobulin E (hyper-IgE) syndrome is a primary immunodeficiency associated with recurrent sinopulmonary infections and eczema.
  • Individuals with hyper-IgE syndrome have a compromised immune system, increasing susceptibility to opportunistic infections.
  • Cryptococcal meningitis is a serious fungal infection of the central nervous system.

Observation:

  • A 13-year-old male with hyper-IgE syndrome presented with symptoms of increased intracranial pressure, including headache, blurred vision, and papilledema.
  • Cerebrospinal fluid analysis confirmed cryptococcal meningitis.

Findings:

  • Standard treatment with amphotericin B and flucytosine, along with repeated lumbar punctures, failed to adequately reduce intracranial pressure.

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  • A myeloperitoneal shunt was surgically placed to manage refractory intracranial hypertension.
  • The patient required six months of fluconazole maintenance therapy for cryptococcal meningitis.

    • Implications:

    • This case underscores the significant risk of severe opportunistic fungal infections, such as cryptococcal meningitis, in individuals with hyper-IgE syndrome.
    • Management of intracranial pressure in cryptococcal meningitis can be challenging and may necessitate invasive procedures like shunting.
    • Prolonged antifungal therapy is crucial for successful treatment and prevention of relapse in immunocompromised patients.