Selda Hançerli Törün, Turkey
İstanbul univercity Pediatric ınfectious diseasePresenter of 1 Presentation
SUCCESSFUL TREATMENT OF PROGRESSİVE MULTIFOCAL LEUKOENCEPHALOPATHY WITH CIDOFOVIR
Abstract
Background and Aims
Progressive multifocal leukoencephalopathy(PML) is a demyelinating disease caused by John Cunningham(JC) virus reactivation in the central nervous system. Especially in the presence of primary or secondary immunodeficiency, it may manifest itself with various neurological findings.
Methods
A 13-year-old male patient with a diagnosis of atopic dermatitis for nine years; admitted with recurrent wound on nose. There was first-degree consanguineous marriage between his parents. Valaciclovir treatment and excision was performed for 4 times. However, alpha interferon treatment was given because of persistent herpesvirus infections and the lesions scared. Genetic analysis performed at University Medical Center in Freiburg revealed heterozygous missense mutation in TRAF3IP2 and TYK2 regions and he was diagnosed with DOC 8 deficiency. In the first year of the diagnosis, soft tissue lesion started on his scrotum. The excision sample was found compatible with squamous cell carcinoma. The patient was admitted with a generalized tonic clonic seizure, who received intravenous immunoglobulin supplementation with three-week intervals. No pathology was detected in the serebrospinal fluid sample except JC virus copy(23600 copies/ml). Radiological evaluation was consistent with PML. Sidofovir and maravirok treatment was started with the literature review.
Results
After 28 doses of sidofovir, the patient had radiological and neurological regression and sidofovir was discontinued. He transferred to the bone marrow transplantation unit.
Conclusions
JC virus, which is latent in the body, should be considered in any neurological finding, especially in patients with cellular immune suppression. Although there is no proven treatment, it is possible to prolong survival based on the examples in the literature.