II. Pathophysiology
- Cerebral perfusion is autoregulated via arteriole constriction and dilation
- Blood Pressure may exceed autoregulation and allow fluid and blood to leak across the blood brain barrier
- Results in Hypertension-induced cerebral edema and micro-Hemorrhages
-
Blood Pressure thresholds vary based on chronicity of Severe Hypertension
- Chronic Severe Hypertension is compensated and encephalopathy occurs at a higher Blood Pressure
- Acute Severe Hypertension is more likely to cause encephalopathy at a lower Blood Pressure (esp children)
- Underlying conditions (e.g. Vasculitis) or new medications may predispose to rapid onset Severe Hypertension
IV. Signs
- Extremely high Blood Pressure
- Altered Level of Consciousness or confusion
- Lethargy
- Seizures
V. Labs
VI. Imaging
-
MRI Brain
- Abnormal in Reversible Posterior Leukoencephalopathy Syndrome (RPLS, PRES)
- Symmetric white matter edema in posterior Cerebral Hemispheres
VII. Diagnosis
- Clinical diagnosis of exclusion (although RPLS or PRES may be diagnosed on MRI)
- Evaluate for other causes of Altered Level of Consciousness
- Hypertensive Encephalopathy typically improves with Blood Pressure lowering (even 10-20%)
- Other causes of Altered Level of Consciousness and Hypertension (e.g. CVA) do not improve as readily
-
Asymptomatic Hypertension may present with very high Blood Pressure and mild Headache
- However, contrast with Hypertensive Encephalopathy in which patient's appear ill and are confused
VIII. Differential Diagnosis
- See Altered Level of Consiousness
- Cerebrovascular Accident
- Eclampsia
- Encephalitis
- Hepatic Encephalopathy
- Uremic Encephalopathy
- Neuroleptic Malignant Syndrome
- Serotonin Syndrome
- Anticholingeric Syndrome
- Cytotoxic Chemotherapy reaction (esp. Serum Creatinine >1.8 mg/dl and Fluid Overload)
IX. Management
- See Hypertensive Emergency for agents to emergently lower Blood Pressure
- Nicardipine is typically recommended in Hypertensive Encephalopathy
- Lower Blood Pressure by 10-25% within first hour of presentation
- Further lowering as needed
X. Prognosis
- Fully reversible in most cases within days to weeks
- In one study, nearly 25% died and another subset had permanent neurologic dysfunction
XI. References
- Swaminathan and Weingart in Herbert (2020) EM:Rap 20(2): 3-4
- Elliott and Varon (2016) Evaluation and Treatment of Hypertensive Emergencies, UpToDate, accessed 12/28/2016
- Neill (2016) Reversible Posterior Leukoencephalopathy Syndrome, UpToDate, accessed 12/28/2016
- Staykov (2012) J Intensive Care Med 27(1): 11-24 +PMID:21257628 [PubMed]
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Related Studies
| Definition (MSH) | Brain dysfunction or damage resulting from sustained MALIGNANT HYPERTENSION. When BLOOD PRESSURE exceeds the limits of cerebral autoregulation, cerebral blood flow is impaired (BRAIN ISCHEMIA). Clinical manifestations include HEADACHE; NAUSEA; VOMITING; SEIZURES; altered mental status (in some cases progressing to COMA); PAPILLEDEMA; and RETINAL HEMORRHAGE. |
| Concepts | Disease or Syndrome (T047) |
| MSH | D020343 |
| ICD9 | 437.2 |
| ICD10 | I67.4 |
| SnomedCT | 155408008, 195225002, 50490005 |
| English | HYPERTENSIVE ENCEPHALOPATHY, ENCEPHALOPATHY HYPERTENSIVE, Hypertensive Encephalopathy, HYPERTENSIVE ENCEPH, ENCEPH HYPERTENSIVE, hypertensive encephalopathy, hypertensive encephalopathy (diagnosis), Encephalopathy hypertensive, Encephalopathy, Hypertensive, Hypertens encephalopathy, Hypertensive Encephalopathy [Disease/Finding], encephalopathy hypertensive, Hypertensive encephalopathy, Hypertensive encephalopathy (disorder), encephalopathy; hypertensive |
| Dutch | encefalopathie hypertensief, encefalopathie; hypertensief, hypertensieve encefalopathie, Encefalopathie, hypertensieve, Hypertensieve encefalopathie |
| German | Enzephalopathie, hypertensiv, ENZEPHALOPATHIE HYPERTONIEBEDINGT, Hypertensionsenzephalopathie, Hypertensive Enzephalopathie, Enzephalopathie, hypertensive |
| Swedish | Blodtrycksutlöst encefalopati |
| Japanese | ｺｳｹﾂｱﾂｾｲﾉｳｼｮｳ, 高血圧性脳症, 高血圧性脳障害, 脳疾患-高血圧性, 脳症-高血圧性, 脳障害-高血圧性 |
| Czech | hypertenzní encefalopatie, Hypertenzní akutní encefalopatie, Hypertenzní encefalopatie |
| Finnish | Hypertensiivinen aivosairaus |
| Russian | ENTSEFALOPATIIA GIPERTENZIVNAIA, GIPERTENZIVNAIA ENTSEFALOPATIIA, ENTSEFALOPATIIA GIPERTONICHESKAIA, GIPERTONICHESKAIA ENTSEFALOPATIIA, ГИПЕРТЕНЗИВНАЯ ЭНЦЕФАЛОПАТИЯ, ГИПЕРТОНИЧЕСКАЯ ЭНЦЕФАЛОПАТИЯ, ЭНЦЕФАЛОПАТИЯ ГИПЕРТЕНЗИВНАЯ, ЭНЦЕФАЛОПАТИЯ ГИПЕРТОНИЧЕСКАЯ |
| Portuguese | ENCEFALOPATIA HIPERTENSIVA, Encefalopatia hipertensiva, Encefalopatia Hipertensiva |
| Spanish | ENCEFALOPATIA HIPERTENSIVA, encefalopatía hipertensiva (trastorno), encefalopatía hipertensiva, Encefalopatía hipertensiva, Encefalopatía Hipertensiva |
| French | ENCEPHALOPATHIE HYPERTENSIVE, Encéphalopathie hypertensive |
| Korean | 고혈압성 뇌병증 |
| Polish | Encefalopatia nadciśnieniowa |
| Hungarian | Hypertensiv encephalopathia, Encephalopathia, hypertensiv |
| Norwegian | Hypertensjonsencefalopati, Hypertensiv encefalopati |
| Italian | Encefalopatia ipertensiva |
Ontology: Posterior Leukoencephalopathy Syndrome (C0878576)
| Definition (MSH) | A condition that is characterized by HEADACHE; SEIZURES; and visual loss with edema in the posterior aspects of the CEREBRAL HEMISPHERES, such as the BRAIN STEM. Generally, lesions involve the white matter (nerve fibers) but occasionally the grey matter (nerve cell bodies). |
| Concepts | Disease or Syndrome (T047) |
| MSH | D054038 |
| English | Posterior Leukoencephalopathy Syndrome, Leukoencephalopathy Syndrome, Posterior, Syndrome, Posterior Leukoencephalopathy, Posterior Leukoencephalopathy Syndrome [Disease/Finding], Syndromes, Posterior Leukoencephalopathy, Leukoencephalopathy Syndromes, Posterior |
| Portuguese | Síndrome da Leucoencefalopatia Posterior |
| Spanish | Síndrome de Leucoencefalopatía Posterior |
| Finnish | Posteriorinen leukoenkefalopatia -oireyhtymä |
| French | Syndrome de leucoencéphalopathie postérieure, Leuco-encéphalopathie postérieure, Leucoencéphalopathie postérieure |
| German | Posteriores Leukenzephalopathie-Syndrom, Leukenzephalopathie-Syndrom, Posteriores, Posteriores Leukencephalopathie-Syndrom, Leukencephalopathie-Syndrom, Posteriores |
| Russian | ZADNEI LEIKOENTSEFALOPATII SINDROM, LEIKOENTSEFALOPATII ZADNEI SINDROM, ЛЕЙКОЭНЦЕФАЛОПАТИИ ЗАДНЕЙ СИНДРОМ, ЗАДНЕЙ ЛЕЙКОЭНЦЕФАЛОПАТИИ СИНДРОМ |
| Swedish | Bakre leukoencefalopatisyndrom |
| Czech | syndrom zadní leukoencefalopatie |
| Italian | Sindrome leucoencefalopatica posteriore |
| Polish | Zespół odwracalnej leukoencefalopatii tylnej, Zespół leukoencefalopatii tylnej |
| Japanese | 後白質脳症症候群 |
| Norwegian | Bakre leukoencefalopatisyndrom |
Ontology: Posterior reversible encephalopathy syndrome (C3160858)
| Definition (NCI) | An acute or subacute reversible condition characterized by headaches, mental status changes, visual disturbances, and seizures associated with imaging findings of posterior leukoencephalopathy. It has been observed in association with hypertensive encephalopathy, eclampsia, and immunosuppressive and cytotoxic drug treatment. |
| Definition (NCI_CTCAE) | A disorder characterized by headaches, mental status changes, visual disturbances, and seizures associated with imaging findings of posterior leukoencephalopathy. It has been observed in association with hypertensive encephalopathy, eclampsia, and immunosuppressive and cytotoxic drug treatment. It is an acute or subacute reversible condition. |
| Concepts | Disease or Syndrome (T047) |
| MSH | D054038 |
| ICD10 | I67.83 |
| SnomedCT | 450886002 |
| English | Reversible Posterior Leukoencephalopathy Syndrome, RPLE, PRES, Posterior reversible encephalopathy syndrome, Reversible posterior leukoencephalopathy syndrome, Posterior reversible encephalopathy syndrome (disorder), posterior reversible encephalopathy syndrome (diagnosis), Posterior Reversible Encephalopathy Syndrome |
| French | Leucoencéphalopathie postérieure réversible, LEPR, SLPR, Leuco-encéphalopathie postérieure réversible, Syndrome de leucoencéphalopathie postérieure réversible, Syndrome d'encéphalie postérieure réversible |
| German | Reversibles posteriores Leukencephalopathie-Syndrom, Reversibles posteriores Leukenzephalopathie-Syndrom, reversible posteriore Leukoenzephalopathie, Posteriores reversibles Enzephalopathiesyndrom |
| Czech | syndrom zadní reverzibilní leukoencefalopatie, Posterior reversible encephalopathy syndrome, Reverzibilní syndrom okcipitální leukoencefalopatie, PRES syndrom, syndrom posteriorní reverzibilní encefalopatie |
| Italian | Sindrome leucoencefalopatica posteriore reversibile, Sindrome della leucoencefalopatia posteriore reversibile, Sindrome da encefalopatia posteriore reversibile |
| Spanish | Síndrome de leucoencefalopatía reversible posterior, Síndrome de encefalopatía posterior reversible, síndrome de encefalopatía posterior reversible (trastorno), síndrome de encefalopatía posterior reversible, síndrome de leucoencefalopatía posterior reversible |
| Portuguese | Síndrome de encefalopatia posterior reversível, Síndrome de leucoencefalopatia reversível posterior |
| Dutch | reversibele posterior leukoencefalopathie-syndroom, posterieure reversibele encefalopathie syndroom |
| Japanese | 可逆性後白質脳症症候群, ｶｷﾞｬｸｾｲｺｳﾊｸｼﾂﾉｳｼｮｳｼｮｳｺｳｸﾞﾝ |
| Hungarian | Reverzibilis posterior leukoencephalopathia syndroma, Posterior reverzibilis encephalopathia syndroma |
| Norwegian | Reversibel bakre leukoencefalopatisyndrom |
