Posterior reversible encephalopathy syndrome: Difference between revisions
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==Background== | ==Background== | ||
* | *PRES, a rare syndrome characterized by acute neurological symptoms due to vasogenic edema in the posterior brain | ||
**Somewhat of a misnomer as changes seen on MRI are not limited to the posterior fossa and symptoms are not always reversible | |||
*Somewhat of a misnomer as changes seen on MRI are not limited to the posterior fossa and symptoms are not always reversible | |||
**Renamed reversible posterior leukoencephalopathy syndrome (RPLS) by the American Academy of Neurology | **Renamed reversible posterior leukoencephalopathy syndrome (RPLS) by the American Academy of Neurology | ||
*Risk factors: [[malignant hypertension]], kidney disease, autoimmune disease, immunosuppression, [[eclampsia]] | |||
*Poorly understood entity, but thought to be due to 2 theoretical mechanisms:<ref>Zelaya JE, Al-Khoury L. Posterior Reversible Encephalopathy Syndrome. [Updated 2022 May 1]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2024 Jan-.</ref> | |||
**Hypertension-hyperperfusion: Hypertensive emergency causes vascular extravasation of fluid and vasogenic edema | |||
**Endothelial dysfunction: Autoimmune or cytotoxic etiologies lead to endothelial dysfunction, leading to increased vascular permeability and edema | |||
*Epidemiolgy: Most frequently in middle-aged females, which may be related to underlying disease<ref>Fischer M, Schmutzhard E. Posterior reversible encephalopathy syndrome. J Neurol. 2017 Aug;264(8):1608-1616. doi: 10.1007/s00415-016-8377-8. Epub 2017 Jan 4. PMID: 28054130; PMCID: PMC5533845.</ref> | |||
*Prognosis: While many case reports suggest PRES is benign and fully reversible, the consequences of PRES, including intracerebral hemorrhage or extensive intracerebral edema, can result in residual neurologic deficits | |||
[[File:Posterior reversible encephalopathy syndrome MRI.jpg|thumb|Magnetic resonance image showing multiple cortico-subcortical areas of hyperdense signal involving the occipital and parietal lobes bilaterally and pons in a patient with posterior reversible encephalopathy syndrome]] | [[File:Posterior reversible encephalopathy syndrome MRI.jpg|thumb|Magnetic resonance image showing multiple cortico-subcortical areas of hyperdense signal involving the occipital and parietal lobes bilaterally and pons in a patient with posterior reversible encephalopathy syndrome]] | ||
==Clinical Features<ref>Staykov D. "Posterior reversible encephalopathy syndrome". PMID 21257628</ref>== | ==Clinical Features<ref>Staykov D. "Posterior reversible encephalopathy syndrome". PMID 21257628</ref>== | ||
*[[Seizures]] | *Onset may range from hours to days | ||
*[[Seizures]], including [[status epilepticus]] | |||
*[[Encephalopathy]], ranging from mild [[altered mental status]] to [[coma]] | |||
*[[Acute Vision Loss (Noninflamed)|Visual disturbances]] | |||
*[[Hypertension]] | *[[Hypertension]] | ||
* | **Note that some proportion of patients may not exhibit significant hypertension; of note, a significant proportion of patients also had no known pre-existing hypertension<ref>Fugate JE, Claassen DO, Cloft HJ, Kallmes DF, Kozak OS, Rabinstein AA. Posterior reversible encephalopathy syndrome: associated clinical and radiologic findings. Mayo Clin Proc. 2010 May;85(5):427-32. doi: 10.4065/mcp.2009.0590. PMID: 20435835; PMCID: PMC2861971.</ref> | ||
* | *[[Headache]], [[vomiting]], or other focal neurological deficits | ||
*[[ | |||
==Differential Diagnosis<ref name="Garg">Garg RK (January 2001). "Posterior leukoencephalopathy syndrome". Postgrad Med J 77 (903): 24–8. doi:10.1136/pmj.77.903.24. PMC 1741870. PMID 11123390</ref>== | ==Differential Diagnosis<ref name="Garg">Garg RK (January 2001). "Posterior leukoencephalopathy syndrome". Postgrad Med J 77 (903): 24–8. doi:10.1136/pmj.77.903.24. PMC 1741870. PMID 11123390</ref>== | ||
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==Evaluation== | ==Evaluation== | ||
*[[CT head]] to rule out other etiologies | |||
*[[brain MRI|MRI]] often shows symmetrical cerebral edema, showing as hyperintensities on T2-weighted image in the posterior circulation, most commonly in the parietal-occipital areas<ref name="Garg"/> | |||
**However, any brain region can be involved, including the frontal and temporal lobes | |||
*Focus on [[Altered Mental Status#Work-Up|altered mental status workup]], with PRES as diagnosis of exclusion | *Focus on [[Altered Mental Status#Work-Up|altered mental status workup]], with PRES as diagnosis of exclusion | ||
*[[ | *Consider [[lumbar puncture]] if there is a concern for [[meningitis]] or [[encephalitis]] | ||
*May require EEG for detection of status epilepticus | |||
==Management== | ==Management== | ||
*[[Hypertensive Emergency#Treatment|Control Blood Pressure]] | *Treat the underlying etiology | ||
*Discontinue immunosuppressants | *[[Hypertensive Emergency#Treatment|Control Blood Pressure]], considering gradual reduction to avoid sudden hypoperfusion | ||
*Discontinue immunosuppressants or cytotoxic medications | |||
*Standard seizure management, if seizures are present | |||
*In cases related to [[Preeclampsia]] or [[HELLP syndrome]], consider early OB/GYN consultation for delivery<ref>Parasher A, Jhamb R. Posterior reversible encephalopathy syndrome (PRES): presentation, diagnosis and treatment. Postgrad Med J. 2020 Oct;96(1140):623-628. doi: 10.1136/postgradmedj-2020-137706. Epub 2020 May 28. PMID: 32467104.</ref> | |||
==Disposition== | ==Disposition== | ||
*Admit | *Admit | ||
**Consider ICU for blood pressure titration, obtunded state, status epilepticus, intracranial hemorrhage, or other serious sequelae | |||
==See Also== | ==See Also== | ||
Latest revision as of 16:23, 9 February 2024
Background
- PRES, a rare syndrome characterized by acute neurological symptoms due to vasogenic edema in the posterior brain
- Somewhat of a misnomer as changes seen on MRI are not limited to the posterior fossa and symptoms are not always reversible
- Renamed reversible posterior leukoencephalopathy syndrome (RPLS) by the American Academy of Neurology
- Risk factors: malignant hypertension, kidney disease, autoimmune disease, immunosuppression, eclampsia
- Poorly understood entity, but thought to be due to 2 theoretical mechanisms:[1]
- Hypertension-hyperperfusion: Hypertensive emergency causes vascular extravasation of fluid and vasogenic edema
- Endothelial dysfunction: Autoimmune or cytotoxic etiologies lead to endothelial dysfunction, leading to increased vascular permeability and edema
- Epidemiolgy: Most frequently in middle-aged females, which may be related to underlying disease[2]
- Prognosis: While many case reports suggest PRES is benign and fully reversible, the consequences of PRES, including intracerebral hemorrhage or extensive intracerebral edema, can result in residual neurologic deficits
Clinical Features[3]
- Onset may range from hours to days
- Seizures, including status epilepticus
- Encephalopathy, ranging from mild altered mental status to coma
- Visual disturbances
- Hypertension
- Note that some proportion of patients may not exhibit significant hypertension; of note, a significant proportion of patients also had no known pre-existing hypertension[4]
- Headache, vomiting, or other focal neurological deficits
Differential Diagnosis[5]
Seizure
- Epileptic seizure
- First-time seizure
- Seizure with known seizure disorder
- Status epilepticus
- Temporal lobe epilepsy
- Non-compliance with anti-epileptic medications
- Hyponatremia
- INH toxicity
- Non-epileptic seizure
- Meningitis
- Encephalitis
- Brain abscess
- Intracranial hemorrhage
- Alcohol withdrawal
- Benzodiazepine withdrawal
- Barbiturate withdrawal
- Baclofen withdrawal
- Metabolic abnormalities: hyponatremia, hypernatremia, hypocalcemia, hypomagnesemia, hypoglycemia, hyperglycemia, hepatic failure, uremia
- Eclampsia
- Neurocysticercosis
- Posterior reversible encephalopathy syndrome
- Impact seizure (head trauma)
- Acute hydrocephalus
- Arteriovenous malformation
- Seizure with VP shunt
- Toxic ingestion (amphetamines, anticholinergics, cocaine, INH, organophosphates, TCA, salicylates, lithium, phenothiazines, bupropion, camphor, clozapine, cyclosporine, fluoroquinolones, imipenem, lead, lidocaine, metronidazole, synthetic cannabinoids, theophylline, Starfruit)
- Psychogenic nonepileptic seizure (pseudoseizure)
- Intracranial mass
- Syncope
- Hyperventilation syndrome
- Migraine headache
- Movement disorders
- Narcolepsy/cataplexy
- Post-hypoxic myoclonus (Status myoclonicus)
Evaluation
- CT head to rule out other etiologies
- MRI often shows symmetrical cerebral edema, showing as hyperintensities on T2-weighted image in the posterior circulation, most commonly in the parietal-occipital areas[5]
- However, any brain region can be involved, including the frontal and temporal lobes
- Focus on altered mental status workup, with PRES as diagnosis of exclusion
- Consider lumbar puncture if there is a concern for meningitis or encephalitis
- May require EEG for detection of status epilepticus
Management
- Treat the underlying etiology
- Control Blood Pressure, considering gradual reduction to avoid sudden hypoperfusion
- Discontinue immunosuppressants or cytotoxic medications
- Standard seizure management, if seizures are present
- In cases related to Preeclampsia or HELLP syndrome, consider early OB/GYN consultation for delivery[6]
Disposition
- Admit
- Consider ICU for blood pressure titration, obtunded state, status epilepticus, intracranial hemorrhage, or other serious sequelae
See Also
References
- ↑ Zelaya JE, Al-Khoury L. Posterior Reversible Encephalopathy Syndrome. [Updated 2022 May 1]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2024 Jan-.
- ↑ Fischer M, Schmutzhard E. Posterior reversible encephalopathy syndrome. J Neurol. 2017 Aug;264(8):1608-1616. doi: 10.1007/s00415-016-8377-8. Epub 2017 Jan 4. PMID: 28054130; PMCID: PMC5533845.
- ↑ Staykov D. "Posterior reversible encephalopathy syndrome". PMID 21257628
- ↑ Fugate JE, Claassen DO, Cloft HJ, Kallmes DF, Kozak OS, Rabinstein AA. Posterior reversible encephalopathy syndrome: associated clinical and radiologic findings. Mayo Clin Proc. 2010 May;85(5):427-32. doi: 10.4065/mcp.2009.0590. PMID: 20435835; PMCID: PMC2861971.
- ↑ 5.0 5.1 Garg RK (January 2001). "Posterior leukoencephalopathy syndrome". Postgrad Med J 77 (903): 24–8. doi:10.1136/pmj.77.903.24. PMC 1741870. PMID 11123390
- ↑ Parasher A, Jhamb R. Posterior reversible encephalopathy syndrome (PRES): presentation, diagnosis and treatment. Postgrad Med J. 2020 Oct;96(1140):623-628. doi: 10.1136/postgradmedj-2020-137706. Epub 2020 May 28. PMID: 32467104.