Clinical, imagiological and etiological spectrum of posterior reversible encephalopathy syndrome

Abstract

Objective: Analyze the cases of posterior reversible encephalopathy syndrome (PRES) admitted in a Neurology Department during an 8-year period. Method: Retrospective observational study in a central hospital in the north of Portugal. Results: 14 patients were identified, mean age 52.3 years. Precipitating factors included: eclampsia, isolated arterial hypertension, spinal trauma and autonomic dysreflexia, Guillain-Barré syndrome, sepsis, sarcoidosis and pulmonary cryptococcosis and drugs. Most patients presented posterior-predominant vasogenic edema lesions, however 64.2% presented frontal lesions and in 42.8% cerebellum was involved. Four patients also had acute ischemic lesions and 1 had hemorrhagic lesions. During follow-up 10 patients recovered fully, 2 recovered partially, 1 suffered a recurrence and 2 died in hospital. Conclusion: PRES has many etiological factors. The terms posterior and reversible should be revised because PRES frequently involves other brain regions and it is not always reversible. PRES patients may develop life-threatening complications and mortality is not negligible.Keywords: posterior reversible encephalopathy syndrome, vasogenic edema, arterial hypertension. RESUMOObjetivo: Análise dos casos de síndrome de encefalopatia posterior reversível (PRES) internados em um Serviço de Neurologia durante oito anos. Método: Estudo restrospectivo observacional num hospital central do norte de Portugal. Resultados: Identificaram-se 14 casos, idade média de 52,3 anos. Os factores precipitantes foram: eclâmpsia, hipertensão arterial isolada, traumatismos vertebro-medulares com disfunção autonómica, síndrome de Guillain-Barré, sépsis, sarcoidose e criptococose pulmonar e fármacos. A maioria dos doentes apresentou lesões edematosas de predomínio posterior, contudo 64,2% apresentaram lesões frontais e 42,8% apresentaram também lesões cerebelosas. Quatro doentes tinham lesões isquémicas agudas e um apresentou lesões hemorrágicas. Durante o seguimento, 10 doentes recuperaram totalmente, 2 recuperaram com sequelas, 1 teve recidiva e 2 faleceram durante o internamento. Conclusão: A PRES apresenta muitos factores precipitantes. As designações posterior e reversível deverão ser reequacionadas dado que a PRES afecta outras zonas do cérebro e nem sempre é reversível, apresentado complicações e mortalidade não negligenciáveis.Palavras-chave: síndrome de encefalopatia posterior reversível, edema vasogénico, hipertensão arterial.

Posterior reversible encephalopathy syndrome in malignant hypertension secondary to focal segmental glomerulosclerosis

“…PRES can occur secondary to many conditions like pre-eclampsia, eclampsia, immunosuppressive agents, systemic lupus erythromatosis, systemic sclerosis, sepsis, tumour lysis syndrome, thrombotic thrombocytic purpura, Guillain-Barre's syndrome, AIDS and acute intermittent porphyria 2 5 7. PRES in association with nephrotic syndrome secondary to FSGS is rare6 and we found only three reported cases of PRES in association with FSGS 6 8 9.…”

“…Its association is well established with a number of clinical conditions and has mostly been reported with eclampsia, malignant hypertension and immunosuppressive agents 5. However, PRES secondary to focal segmental glomerulosclerosis (FSGS) is extremely rare 6.…”

Posterior reversible encephalopathy syndrome

“…The PRES can develop in a wide array of situations including pregnancy, post-partum, sepsis, autoimmune diseases, solid organ transplantations and end stage renal disease patients [3][4]. Classic pattern is the involvement of dominant parietal occipital area [1]. Rarely, it can present with fever and significant pleocytosis CSF that may mimic infectious meningoencephalitis as seen in the present case [5].…”

“…Posterior reversible encephalopathy syndrome is a clinico-radiological entity characterized clinically by headache, confusion, seizures and visual disturbances and radiologically by bilateral white matter abnormalities mostly in posterior regions of brain [1]. The suggested mechanism is disruption of blood-brain barrier due to failure of central nervous system auto-regulation leading to vasogenic edema [2].…”

Posterior Reversible Encephalopathy Syndrome in a Thalassemia Minor Woman after in vitro Fertilization

“…Herein, we report a thalassemia minor patient with twin pregnancy who developed PRES. Most studies indicate that IVF-pregnancies and oocyte donation are associated with more obstetric complications increasing risk of maternal mortality when compared to naturally conceived pregnancies (2)(3)(4)(5).…”