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Published on: September 8, 2023
Cryptococcal meningitis and cerebral vasculitis in a patient with primary intestinal lymphangiectasia: a case report
Martin Mathurin1, Sandra Devatine2, Aude Kopp-Derouet2
1Department of Infectious Diseases, Hôpital Lariboisière, Saint-Louis-Lariboisière-Fernand Widal Hospitals, AP-HP, 2 Rue Ambroise Paré, 75010, Paris, France. martin.mathurin@aphp.fr.
Abstract:
Primary intestinal lymphangiectasia (Waldmann's disease) is a rare exudative enteropathy without precisely assessed infectious risk. We report the case of a 49-year-old male patient with meningitis and cerebral vasculitis due to Cryptococcus neoformans complicating Waldmann's disease diagnosed 12 years ago. The treatment combined liposomal amphotericin B, 3 mg/kg daily plus flucytosine 25 mg/kg/6 h, both intravenously during 15 days, then fluconazole 800 mg daily during 8 weeks, and finally 200 mg daily indefinitely. Dexamethasone 0.4 mg/kg daily during the first week was gradually decreased over 2 months. The outcome was good, and the patient is still followed 3 years later without any recurrence.
Insights
Primary intestinal lymphangiectasia, a rare condition, can increase cryptococcal infection risk. This case highlights successful treatment of cryptococcal meningitis and vasculitis in a patient with Waldmann's disease.
Area of Science:
- Gastroenterology
- Infectious Diseases
- Neurology
Background:
- Primary intestinal lymphangiectasia, or Waldmann's disease, is a rare exudative enteropathy.
- The infectious risks associated with this condition are not well-defined.
Observation:
- A 49-year-old male with a 12-year history of Waldmann's disease presented with meningitis and cerebral vasculitis.
- The patient was diagnosed with Cryptococcus neoformans infection.
Findings:
- Treatment involved a combination of liposomal amphotericin B, flucytosine, and fluconazole for cryptococcal infection.
- Corticosteroid therapy (dexamethasone) was tapered over two months.
Implications:
- This case underscores the potential for serious opportunistic infections in patients with Waldmann's disease.
- Effective management strategies for cryptococcal infections in this patient population can lead to favorable outcomes.

