Progressive multifocal leukoencephalopathy (PML) in HIV-infected patients – a case report and review of patients treated at the University Hospital for Infectious Diseases in Zagreb

Abstract

U četrdesetogodišnje bolesnice s postupnim i progresivnim razvojem neuroloških simptoma dijagnosticirana je HIV-bolest uz veoma izraženu imunodeficijenciju. Na temelju neuroloških simptoma (motorni deficiti, kognitivni poremećaji, poremećaji govora, vida, tonusa muskulature, smetnje koordinacije) te slikovnih radioneuroloških tehnika postavljena je klinička dijagnoza progresivne multifokalne leukoencefalopatije (PML). Naknadno je dijagnoza PML potvrđena pozitivnim nalazom reakcije lančane polimeraze (PCR) na JC virus u likvoru. Bolesnica je liječena antiretrovirusnim lijekovima uz ostalu simptomatsku i suportivnu terapiju. Unatoč određenom poboljšanju laboratorijskih parametara stečene imunodeficijencije na provedeno liječenje tijekom pet mjeseci bolest je nezadrživo napredovala do smrtnog ishoda. Do sada je u 20-godišnjem razdoblju u našoj Klinici liječeno ukupno 11 bolesnika s HIV-bolešću u kojih je na temelju kliničko-neuroloških manifestacija i neuroradioloških pretraga postavljena dijagnoza PML. Detalji su prikazani u tablici 2. U spomenutom slučaju po prvi put je u Hrvatskoj etiološka dijagnoza potvrđena dokazom JC virusa u cerebrospinalnom likvoru.HIV-infection with advanced immunodeficiency was diagnosed in a forty-year-old female patient with gradual and progressive development of neurological symptoms. Based on neurological symptoms (cognitive and motor deficits, speech, vision and muscular tonus disorders, coordination disturbances) as well as neuroradiological imaging, progressive multifocal leukoencephalopathy (PML) was diagnosed. Subsequently, the diagnosis of PML was confirmed with positive cerebrospinal fluid (CSF) polymerase chain reaction (PCR) for JC virus. The patient was treated with antiretroviral drugs together with other symptomatic and supportive therapy. Despite somewhat improved laboratory parameters of acquired immunodeficiency after five-month therapy, the disease continued to progress and resulted in fatal outcome. In the last 20 years, altogether 11 HIV-infected patients were diagnosed with PML in our hospital based on clinical/neurological manifestations and neuroradiologic imaging findings. The details are presented in Table 2. In described case, for the first time in Croatia, the etiological diagnosis was confirmed with JC virus detection in the CSF