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.
Data availability
Not applicable.
Code availability
Not applicable.
References
Vignes S, Bellanger J (2018) Lymphangiectasies intestinales primitives (maladie de Waldmann). Rev Méd Interne 39(7):580–585
Cole SL, Ledford DK, Lockey RF, Daas A, Kooper J (2007) Primary gastrointestinal lymphangiectasia presenting as cryptococcal meningitis. Ann Allergy Asthma Immunol Off Publ Am Coll Allergy Asthma Immunol 98(5):490–492
Anderson DJ, Schmidt C, Goodman J, Pomeroy C (1992) Cryptococcal disease presenting as cellulitis. Clin Infect Dis Off Publ Infect Dis Soc Am 14(3):666–672
Heresbach D, Raoul JL, Genetet N, Noret P, Siproudhis L, Ramée MP et al (1994) Immunological study in primary intestinal lymphangiectasia. Digestion 55(1):59–64
Vignes S, Carcelain G (2009) Increased surface receptor Fas (CD95) levels on CD4+ lymphocytes in patients with primary intestinal lymphangiectasia. Scand J Gastroenterol 44(2):252–256
Sanges S, Germain N, Vignes S, Séguy D, Stabler S, Etienne N et al (2022) Protein-losing enteropathy as a complication and/or differential diagnosis of common variable immunodeficiency. J Clin Immunol 42(7):1461–1472
Chen Y, Shi ZW, Strickland AB, Shi M (2022) Cryptococcus neoformans infection in the central nervous system: the battle between host and pathogen. J Fungi Basel Switz 8(10):1069
Munck A, Sosa Valencia G, Faure C et al (2002) Suivi de long cours des lymphangiectasies intestinales primitives de l’enfant. A propos de six cas [Long-term followup of primary intestinal lymphangiectasia in the child. Six case reports]. Arch Pediatr 9:388–391
Beardsley J, Sorrell TC, Chen SCA (2019) Central nervous system cryptococcal infections in non-HIV infected patients. J Fungi Basel Switz 5(3):71
Molloy SF, Kanyama C, Heyderman RS, Loyse A, Kouanfack C, Chanda D et al (2018) Antifungal combinations for treatment of cryptococcal meningitis in Africa. N Engl J Med 378(11):1004–1017
Perfect JR, Dismukes WE, Dromer F, Goldman DL, Graybill JR, Hamill RJ et al (2010) Clinical practice guidelines for the management of cryptococcal disease: 2010 update by the infectious diseases society of America. Clin Infect Dis Off Publ Infect Dis Soc Am 50(3):291–322
Dierselhuis MP, Boelens JJ, Versteegh FGA, Weemaes C, Wulffraat NM (2007) Recurrent and opportunistic infections in children with primary intestinal lymphangiectasia. J Pediatr Gastroenterol Nutr 44(3):382–385
Fugate JE, Lyons JL, Thakur KT, Smith BR, Hedley-Whyte ET, Mateen FJ (2014) Infectious causes of stroke. Lancet Infect Dis 14(9):869–880
Carod Artal FJ (2016) Clinical management of infectious cerebral vasculitides. Expert Rev Neurother 16(2):205–221
Rosario M, Song X, McCullough LD (2012) An unusual case of stroke. Neurologist 18(4):229–232
Mishra AK, Arvind VH, Muliyil D, Kuriakose CK, George AA, Karuppusami R et al (2018) Cerebrovascular injury in cryptococcal meningitis. Int J Stroke Off J Int Stroke Soc 13(1):57–65
Lan SH, Chang WN, Lu CH, Lui CC, Chang HW (2001) Cerebral infarction in chronic meningitis: a comparison of tuberculous meningitis and cryptococcal meningitis. QJM Mon J Assoc Phys 94(5):247–253
Acknowledgements
We acknowledge Marie Desnos-Ollivier and Françoise Dromer (National Reference Center for invasive mycoses and Antifungals; Pasteur Institute, Paris, France) for the identification and MICs (in the context of the RESSIF surveillance network) from the patient’s isolate.
Author information
Authors and Affiliations
Corresponding authors
Ethics declarations
Ethics approval
Not applicable.
Consent to participate and consent for publication
The patient gave his informed, written consent about the writing and publication of this case report and for the use of the radiographic images.
Competing interests
The authors declare no competing interests.
Additional information
Publisher's Note
Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.
Rights and permissions
About this article
Cite this article
Mathurin, M., Devatine, S., Kopp-Derouet, A. et al. Cryptococcal meningitis and cerebral vasculitis in a patient with primary intestinal lymphangiectasia: a case report. Eur J Clin Microbiol Infect Dis 42, 1263–1267 (2023). https://doi.org/10.1007/s10096-023-04657-y
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s10096-023-04657-y