Cryptococcal meningoencephalitis in a patient with hyper immunoglobulin M (IgM) syndrome: a case report

Cryptococcal meningoencephalitis is an opportunistic infection that predominantly affects immunocompromised patients. Hyper immunoglobulin M syndrome is a primary immunodeficiency syndrome that increases susceptibility to several opportunistic infections. Here, we report a case of cryptococcal meningoencephalitis in the context of hyper immunoglobulin M syndrome, a situation that has been reported very few times and whose management is not clearly defined. We describe our management of this case and the outcome of the patient to help in future similar situations.

The patient is a 19-year-old Caucasian male student diagnosed with X-linked hyper immunoglobulin M syndrome and treated chronically with weekly intravenous immunoglobulin and daily sulfamethoxazole-trimethoprim. He was admitted to the infectious diseases ward because of headache, diplopia and a cerebral-spinal fluid analysis revealing cryptococcal meningoencephalitis. The patient was treated with liposomal amphotericin and flucytosine with a favorable outcome. Maintenance therapy with fluconazole has continued and will be sustained for 6 months following his upcoming bone marrow transplantation.

Monitoring for cryptococcal meningoencephalitis should be considered in patients with primary immunodeficiencies, as clinical manifestations may go unnoticed. In these patients, it is expected that chronic treatment with fluconazole will be the only treatment that will prevent reinfection or reactivation, and therefore should be kept at least until bone marrow transplant, the only curative treatment, is performed. It may, however, lead to intolerable side effects and hepatic toxicity.