Title Sindrom posteriorne reverzibilne encefalopatije
Title (english) Posterior reversible encephalopathy syndrome
Author Kim Bogdan Veljković
Mentor Zdravka Poljaković-Skurić (mentor)
Committee member Srđana Telarović (predsjednik povjerenstva)
Committee member Mario Habek (član povjerenstva)
Committee member Zdravka Poljaković-Skurić (član povjerenstva)
Granter University of Zagreb School of Medicine (Department of Neurology) Zagreb
Defense date and country 2022-07-15, Croatia
Scientific / art field, discipline and subdiscipline BIOMEDICINE AND HEALTHCARE Clinical Medical Sciences Neurology
Abstract Sindrom posteriorne reverzibilne encefalopatije (PRES) je kliničko-radiološki sindrom koji je karakteriziran nespecifičnim neurološkim simptomima i karakterističnom radiološkom slikom simetričnog vazogenog edema koji zahvaća bijelu tvar u posteriornim hemisferama velikog mozga. Uzroci PRES-a su mnogobrojni. Međutim, najčešća stanja koja se povezuju su umjerena do teška hipertenzija, preeklampsija i eklampsija, terapija imunosupresivnim i citotoksičnim lijekovima, autoimune bolesti, transplantacija koštane srži, matičnih stanica ili solidnih organa, infekcija s razvojem sepse i šoka te akutne ili kronične bolesti bubrega. Točan patofiziološki mehanizam nastanka PRES-a nije u potpunosti razjašnjen. U načelu je prihvaćeno da se u pozadini procesa nalazi disfunkcija krvno-moždane barijere koja rezultira nastankom vazogenog edema. Postoji nekoliko teorija o patofiziologiji PRES-a. Jedna od njih je teorija o hipertenziji i cerebralnoj hiperperfuziji koja smatra da je primarni uzrok vazogenog edema gubitak autoregulacije u području stražnje cirkulacije moždanog krvožilnog sustava zbog velikih i naglih porasta krvnog tlaka. Druga glavna teorija je teorija o endotelnoj disfunkciji koja smatra da PRES nastaje uslijed oštećenja endotela cirkulirajućim toksinima što dovodi do povećane propusnosti krvno-moždane barijere i razvoja vazogenog edema. Klinička slika je nespecifična te je obilježena glavoboljom i oslabljenom vidnom oštrinom te u težim slučajevima gubitkom vida, epileptičkim napadajima, promijenjenim mentalnim statusom i poremećajem stanja svijesti. U dijagnostičkoj obradi PRES-a najznačajniji je MR mozga u T2 i FLAIR sekvenci koji pruža najbolji prikaz znakova vazogenog edema te pomaže u diferencijalnoj dijagnostici PRES-a od ostalih stanja koja svojom kliničkom slikom i patofiziološkim značajkama nalikuju na PRES. PRES se na radiološkim snimkama može prezentirati na atipičan način, bilo da se radi o zahvaćanju bazalnih ganglija, moždanog debla ili malog mozga ili razvoju komplikacija kao što su cerebralno krvarenje ili citotoksični edem. PRES je obično reverzibilna bolest, a liječenje je usmjereno na uklanjanje uzroka ako je moguće. Prognoza je dobra ako se uzrok otkrije i ukloni, te u roku od nekoliko tjedana dolazi do poboljšanja kliničke slike i regresije promjena na MR-i mozga.
Abstract (english) Posterior reversible encephalopathy syndrome (PRES) is a clinical and radiographic syndrome characterized by nonspecific neurological symptoms and characteristic imaging findings of symmetrical posterior-predominant cerebral white matter vasogenic edema. There are numerous causes of PRES. However, the most common conditions associated with it are moderate to severe hypertension, preeclampsia and eclampsia, the use of immunosuppressant and cytotoxic drugs, autoimmune disorders, bone marrow, stem cell or solid organ transplantation, infection with sepsis and shock, and acute or chronic kidney disease. The precise pathophysiological mechanism behind PRES has yet to be fully clarified. However, the generally accepted mechanism is dysfunction of the blood-brain barrier resulting in vasogenic edema. There are several proposed theories for the pathophysiology of PRES. One of them is the hypertensive and cerebral hyperperfusion theory which proposes that the primary cause of vasogenic edema is the loss of autoregulation in the posterior circulatory area of the cerebral vascular system due to large and sudden increases in blood pressure. The second major theory is the endothelial dysfunction theory, which proposes endothelial injury caused by circulating toxins as the primary cause of PRES, which leads to the increased permeability of the blood-brain barrier and the development of vasogenic edema. Clinical presentation is nonspecific and ranges from headache and impaired visual acuity to visual loss, epileptic seizures, altered mental status and altered levels of consciousness in more severe cases. T2-weighted/FLAIR sequences on MRI play a fundamental role in the diagnosis of PRES and are most useful in detecting vasogenic edema and in differentiating PRES from other medical conditions that share some clinical and pathophysiological features. PRES may occur on imaging in an atypical fashion with the involvement of basal ganglia, brainstem, or cerebellum, or it may be complicated by the presence of cerebral hemorrhage or cytotoxic edema. PRES is usually reversible, and the treatment is aimed at eliminating the cause if possible. Prognosis is good if the cause is recognized and removed, and most patients show clinical improvement and regression of lesions on follow up brain MRI within a few weeks.
Keywords
PRES
vazogeni edem
MR mozga u T2 i FLAIR sekvenci
Keywords (english)
PRES
vasogenic edema
T2-weighted/FLAIR sequences on MRI
Language croatian
URN:NBN urn:nbn:hr:105:695773
Study programme Title: Medicine Study programme type: university Study level: integrated undergraduate and graduate Academic / professional title: doktor/doktorica medicine (doktor/doktorica medicine)
Type of resource Text
File origin Born digital
Access conditions Open access
Terms of use
Created on 2023-03-20 09:40:49