Posterior reversible encephalopathy syndrome
Posterior reversible encephalopathy syndrome (PRES) is acute headache, seizures, altered mental status, and visual disturbance with vasogenic edema predominantly in the parietal and occipital lobes, caused by failure of cerebral autoregulation in hypertensive emergency, rapid blood pressure fluctuation, preeclampsia or eclampsia, or cytotoxic and immunosuppressive drugs such as cyclosporine. MRI establishes the diagnosis because CT is often unrevealing; treatment is gradual blood pressure reduction of about 10%-25%, antiseizure therapy, magnesium in pregnancy, and removal of the trigger, and most patients recover within 2 weeks.
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What this article covers
- Overview
Definition: posterior reversible encephalopathy syndrome (PRES), also called reversible posterior leukoencephalopathy syndrome, combines acute neurologic symptoms (headache, seizures, altered mental…
- Epidemiology
Eclampsia almost always shows PRES-type changes: in the vast majority of patients with eclampsia, brain imaging shows PRES-type parieto-occipital white matter edema.
- Risk factors
Hypertensive emergency or rapid blood pressure fluctuation: the most frequent precipitant, especially with poorly controlled chronic hypertension, chronic kidney disease, cocaine or amphetamine use…
- Pathophysiology
Autoregulatory failure: severe or rapidly fluctuating blood pressure overwhelms cerebral autoregulation, causing brain hyperperfusion.
- Clinical features
Defining syndrome: acute or subacute headache, altered consciousness, visual disturbance, and seizures in a patient with a hypertensive crisis, preeclampsia, or a cytotoxic drug.
- Differential diagnosis
Subarachnoid hemorrhage: thunderclap headache with blood on noncontrast CT, or xanthochromia on lumbar puncture if CT is negative.
- Diagnosis
Establishing the diagnosis: no formal criteria set or severity grading exists; PRES is established by the combination of an acute neurologic syndrome (headache, seizures, altered mental status…
- Management
First, stop seizures if present: lorazepam 0.1 mg/kg IV (maximum 4 mg per dose), repeated once after 10-15 minutes if seizures continue then levetiracetam 60 mg/kg IV once (maximum 4500 mg) as a…
- Complications
Status epilepticus: from untreated or recurrent seizures.
- Prognosis
Most patients recover within 2 weeks once blood pressure is controlled and the trigger is removed.
- Prevention
Control chronic hypertension and treat blood pressure of 180/120 mm Hg or higher with end-organ symptoms as an emergency.
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Related articles
- Hypertensive emergency
Hypertensive emergency is severe hypertension (usually above 180/120 mmHg) with acute target-organ damage such as encephalopathy, stroke, acute heart failure, myocardial ischemia, aortic dissection, or acute kidney injury. It requires controlled intravenous blood pressure reduction, with the target and pace determined by the specific end-organ involved.
- Preeclampsia
New-onset hypertension after 20 weeks of gestation with proteinuria or end-organ dysfunction, driven by abnormal placental spiral artery remodeling and widespread endothelial dysfunction. Eclampsia is preeclampsia complicated by seizures; magnesium sulfate treats and prevents seizures, and prompt delivery is the only definitive cure for both.
- Reversible cerebral vasoconstriction syndrome
Reversible cerebral vasoconstriction syndrome (RCVS) is recurrent thunderclap headache with multifocal constriction of intracranial arteries that normalizes within 3 months, usually triggered by vasoactive drugs or the puerperium, and it is the second most frequent cause of thunderclap headache after subarachnoid hemorrhage. Diagnosis follows noncontrast CT, lumbar puncture, and MRA or CTA; treatment is removal of the trigger, blood pressure control, and verapamil, with glucocorticoids and triptans avoided.
- Cerebral venous sinus thrombosis
Thrombosis of the dural venous sinuses or cortical veins that impairs cerebral venous drainage and CSF absorption, producing headache, papilledema, focal deficits, or seizures; MRI/MRV or CT venography establishes the diagnosis, and anticoagulation is first-line even when hemorrhagic venous infarction is present.
- Subarachnoid hemorrhage
Subarachnoid hemorrhage is sudden bleeding into the subarachnoid space, most often from a ruptured saccular (berry) aneurysm, presenting as the worst headache of a patient's life. Diagnosis is noncontrast head CT, then lumbar puncture if CT is negative, then catheter angiography to define the aneurysm; management is early aneurysm securement, blood pressure control, and nimodipine to reduce delayed cerebral ischemia from vasospasm.
- Intracerebral hemorrhage
Spontaneous bleeding into brain parenchyma, most often from chronic hypertension affecting deep penetrating vessels or from cerebral amyloid angiopathy in lobar locations, diagnosed by noncontrast CT and managed with acute blood pressure lowering, reversal of anticoagulation, and surgical evacuation for select cerebellar or deteriorating hemorrhages.
- Status epilepticus
Status epilepticus is a generalized tonic-clonic seizure lasting more than 5 minutes, or repeated seizures without return to baseline, and is a neurologic emergency. Treatment is a timed escalation: IV benzodiazepine first, then an IV antiepileptic drug, then anesthetic coma with continuous EEG if convulsions persist.
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A first or provoked seizure requires a focused history to distinguish it from syncope or a nonepileptic event, urgent glucose and electrolyte testing, brain MRI, and outpatient EEG; an unprovoked seizure with normal imaging and EEG usually does not require antiepileptic drug therapy, since epilepsy is defined only after a second unprovoked seizure or a high-risk first one.
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