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Posterior Reversible
Encephalopathy Syndrome
(PRES)
Ade Wijaya, MD – June 2019
Definition
A disorder of reversible subcortical vasogenic brain oedema in patients with acute
neurological symptoms (eg, seizures, encephalopathy, headache, and visual
disturbances) in the setting of renal failure, blood pressure fluctuations, cytotoxic
drugs, autoimmune disorders, and pre-eclampsia or eclampsia
Fugate JE, Rabinstein AA. Posterior reversible encephalopathy syndrome: clinical and radiological manifestations, pathophysiology, and outstanding questions. The Lancet Neurology. 2015 Sep 1;14(9):914-25.
Cerebral Autoregulation
Mamo, Y. A. (2015). Cerebrovascular effects of vasoactive drugs: in vitro, in vivo and clinical investigations (Doctoral dissertation)
Patophysiology
Fugate JE, Rabinstein AA. Posterior reversible encephalopathy syndrome: clinical and radiological manifestations, pathophysiology, and outstanding questions. The Lancet Neurology. 2015 Sep 1;14(9):914-25.
Clinical Presentation
 Encephalopathy (50–80%)
 Seizure (60–75%)
 Headache (50%)
 Visual disturbances (33%)
 Focal neurological defi cit (10–15%)
 Status epilepticus (5–15%)
Fugate JE, Rabinstein AA. Posterior reversible encephalopathy syndrome: clinical and radiological manifestations, pathophysiology, and outstanding questions. The Lancet Neurology. 2015 Sep 1;14(9):914-25.
Differential Diagnosis
Fugate JE, Rabinstein AA. Posterior reversible encephalopathy syndrome: clinical and radiological manifestations, pathophysiology, and outstanding questions. The Lancet Neurology. 2015 Sep 1;14(9):914-25.
Radiology
Fugate JE, Rabinstein AA. Posterior reversible encephalopathy syndrome: clinical and radiological manifestations, pathophysiology, and outstanding questions. The Lancet Neurology. 2015 Sep 1;14(9):914-25.
Management
 No Spesific treatment
 Treat underlying disease
 Smooth control of hypertension (IV drugs preferred)
Mancia G, Fagard R, Narkiewicz K, et al. 2013 ESH/ESC guidelines for the management of arterial hypertension: the Task Force for the Management of Arterial Hypertension of the European Society of
Hypertension (ESH) and of the European Society of Cardiology (ESC). Eur Heart J 2013; 34: 2159–219
Prognosis
 The prognosis of PRES is usually favourable and most patients wholly recover
 Not always fully reversible; mortality was about 3–6% during a range of follow up times
(generally 1–3 months)
 Severe neurological injury and fatality can be attributed to intracranial haemorrhage,
 Posterior fossa oedema with brainstem compression or acute hydrocephalus, or marked
diffuse cerebral oedema and increased global intracranial pressure
 Persistent neurological sequelae are reported in 10–20% of patients
 Hyperglycaemia and time to control of the causative factor have been also reported to
be independently associated with poor outcomes
 PRES is recurrent in about 5–10% of cases and is more common in patients with
uncontrolled hypertension compared with patients who have other causes (ie,
immunosuppressant therapy)
Fugate JE, Rabinstein AA. Posterior reversible encephalopathy syndrome: clinical and radiological manifestations, pathophysiology, and outstanding questions. The Lancet Neurology. 2015 Sep 1;14(9):914-25.
Fugate JE, Rabinstein AA. Posterior reversible encephalopathy syndrome: clinical and radiological manifestations, pathophysiology, and outstanding questions. The Lancet Neurology. 2015 Sep 1;14(9):914-25.
Summary
 PRES is a clinical syndrome consisting of acute neurological symptoms caused by
endothelial dysfunction
 Usually arises from severe abrupt arterial hypertension or blood pressure fl
uctuations, but can also be caused by direct endothelial injury from
immunosuppressant drugs, autoimmune disorders, or in the setting of eclampsia
 Vasogenic oedema in PRES is usually visualised with brain imaging in characteristic
patterns including the usually described posterior brain regions, arterial watershed
zones, and the superior frontal sulcus pattern
 Usually have favorable prognosis
Posterior Reversible Encephalopathy Syndrome

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Posterior Reversible Encephalopathy Syndrome

  • 2. Definition A disorder of reversible subcortical vasogenic brain oedema in patients with acute neurological symptoms (eg, seizures, encephalopathy, headache, and visual disturbances) in the setting of renal failure, blood pressure fluctuations, cytotoxic drugs, autoimmune disorders, and pre-eclampsia or eclampsia Fugate JE, Rabinstein AA. Posterior reversible encephalopathy syndrome: clinical and radiological manifestations, pathophysiology, and outstanding questions. The Lancet Neurology. 2015 Sep 1;14(9):914-25.
  • 3. Cerebral Autoregulation Mamo, Y. A. (2015). Cerebrovascular effects of vasoactive drugs: in vitro, in vivo and clinical investigations (Doctoral dissertation)
  • 4. Patophysiology Fugate JE, Rabinstein AA. Posterior reversible encephalopathy syndrome: clinical and radiological manifestations, pathophysiology, and outstanding questions. The Lancet Neurology. 2015 Sep 1;14(9):914-25.
  • 5. Clinical Presentation  Encephalopathy (50–80%)  Seizure (60–75%)  Headache (50%)  Visual disturbances (33%)  Focal neurological defi cit (10–15%)  Status epilepticus (5–15%) Fugate JE, Rabinstein AA. Posterior reversible encephalopathy syndrome: clinical and radiological manifestations, pathophysiology, and outstanding questions. The Lancet Neurology. 2015 Sep 1;14(9):914-25.
  • 6. Differential Diagnosis Fugate JE, Rabinstein AA. Posterior reversible encephalopathy syndrome: clinical and radiological manifestations, pathophysiology, and outstanding questions. The Lancet Neurology. 2015 Sep 1;14(9):914-25.
  • 7. Radiology Fugate JE, Rabinstein AA. Posterior reversible encephalopathy syndrome: clinical and radiological manifestations, pathophysiology, and outstanding questions. The Lancet Neurology. 2015 Sep 1;14(9):914-25.
  • 8. Management  No Spesific treatment  Treat underlying disease  Smooth control of hypertension (IV drugs preferred) Mancia G, Fagard R, Narkiewicz K, et al. 2013 ESH/ESC guidelines for the management of arterial hypertension: the Task Force for the Management of Arterial Hypertension of the European Society of Hypertension (ESH) and of the European Society of Cardiology (ESC). Eur Heart J 2013; 34: 2159–219
  • 9. Prognosis  The prognosis of PRES is usually favourable and most patients wholly recover  Not always fully reversible; mortality was about 3–6% during a range of follow up times (generally 1–3 months)  Severe neurological injury and fatality can be attributed to intracranial haemorrhage,  Posterior fossa oedema with brainstem compression or acute hydrocephalus, or marked diffuse cerebral oedema and increased global intracranial pressure  Persistent neurological sequelae are reported in 10–20% of patients  Hyperglycaemia and time to control of the causative factor have been also reported to be independently associated with poor outcomes  PRES is recurrent in about 5–10% of cases and is more common in patients with uncontrolled hypertension compared with patients who have other causes (ie, immunosuppressant therapy) Fugate JE, Rabinstein AA. Posterior reversible encephalopathy syndrome: clinical and radiological manifestations, pathophysiology, and outstanding questions. The Lancet Neurology. 2015 Sep 1;14(9):914-25.
  • 10. Fugate JE, Rabinstein AA. Posterior reversible encephalopathy syndrome: clinical and radiological manifestations, pathophysiology, and outstanding questions. The Lancet Neurology. 2015 Sep 1;14(9):914-25.
  • 11. Summary  PRES is a clinical syndrome consisting of acute neurological symptoms caused by endothelial dysfunction  Usually arises from severe abrupt arterial hypertension or blood pressure fl uctuations, but can also be caused by direct endothelial injury from immunosuppressant drugs, autoimmune disorders, or in the setting of eclampsia  Vasogenic oedema in PRES is usually visualised with brain imaging in characteristic patterns including the usually described posterior brain regions, arterial watershed zones, and the superior frontal sulcus pattern  Usually have favorable prognosis