Posterior reversible encephalopathy syndrome (PRES), also known as reversible posterior leukoencephalopathy syndrome (RPLS), is a clinical syndrome consisting of rapid onset headaches, altered levels of consciousness, visual disturbances, and seizures associated with characteristic radiologic findings on MRI including symmetric white matter edema in the posterior cerebral hemispheres, especially the parieto-occipital regions. Occasionally, gray matter is involved. Headaches are typically moderate to severe, nonlocalized, and unresponsive to analgesia. Seizures may be the presenting manifestation and are usually generalized tonic-clonic. Altered consciousness ranges from confusion and mild somnolence to coma.
Physical examination often reveals hypertension and detectable visual perception abnormalities, including hemianopia, cortical blindness, or visual neglect. Reflexes are brisk and Babinski sign is often present. Risk factors for development of RPLS include hypertension, eclampsia, and immunosuppressive therapy, especially cyclosporine, tacrolimus, sirolimus, cisplatin, and rituximab.
Treatment is aimed at the precipitating cause, and prompt treatment is necessary given the potentially reversible nature of the condition. Onset is usually over hours to a day. Most patients recover within 2 weeks, with few patients having residual neurologic deficits.
Emergency: requires immediate attention
Reversible posterior leukoencephalopathy syndrome
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Codes
ICD10CM:
I67.83 – Posterior reversible encephalopathy syndrome
SNOMEDCT:
450886002 – Posterior reversible encephalopathy syndrome
I67.83 – Posterior reversible encephalopathy syndrome
SNOMEDCT:
450886002 – Posterior reversible encephalopathy syndrome
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Diagnostic Pearls
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Differential Diagnosis & Pitfalls
- Stroke
- Intracranial hemorrhage
- Brain tumor or brain metastasis
- Traumatic brain injury
- Subarachnoid hemorrhage
- Status epilepticus
- Cerebral vasculitis
- Metabolic encephalopathy (eg, hepatic encephalopathy, uremic encephalopathy)
- Bacterial meningitis
- Viral meningitis / encephalitis (eg, herpes simplex virus encephalitis)
- Fungal meningitis
- Carcinomatous meningitis
- Progressive multifocal leukoencephalopathy
- Migraine headache
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Drug Reaction Data
Below is a list of drugs with literature evidence indicating an adverse association with this diagnosis. The list is continually updated through ongoing research and new medication approvals. Click on Citations to sort by number of citations or click on Medication to sort the medications alphabetically.
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Last Reviewed:04/09/2018
Last Updated:07/09/2018
Last Updated:07/09/2018