Cardiovascular Medicine Book

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Hypertensive Encephalopathy

Aka: Hypertensive Encephalopathy, Posterior Reversible Encephalopathy Syndrome, Posterior Leukoencephalopathy Syndrome, PRES, RPLS
  1. See Also
    1. Hypertensive Emergency
  2. Pathophysiology
    1. Cerebral perfusion is autoregulated via arteriole constriction and dilation
      1. Blood Pressure may exceed autoregulation and allow fluid and blood to leak across the blood brain barrier
      2. Results in Hypertension-induced cerebral edema and micro-Hemorrhages
    2. Blood Pressure thresholds vary based on chronicity of Severe Hypertension
      1. Chronic Severe Hypertension is compensated and encephalopathy occurs at a higher Blood Pressure
      2. Acute Severe Hypertension is more likely to cause encephalopathy at a lower Blood Pressure (esp children)
        1. Underlying conditions (e.g. Vasculitis) or new medications may predispose to rapid onset Severe Hypertension
  3. Symptoms
    1. Headache
    2. Visual changes
    3. Vomiting
    4. Dizziness
  4. Signs
    1. Extremely high Blood Pressure
    2. Altered Level of Consciousness or confusion
    3. Lethargy
    4. Seizures
  5. Labs
    1. See Altered Level of Consciousness
  6. Imaging
    1. MRI Brain
      1. Abnormal in Reversible Posterior Leukoencephalopathy Syndrome (RPLS, PRES)
      2. Symmetric white matter edema in posterior Cerebral Hemispheres
  7. Diagnosis
    1. Clinical diagnosis of exclusion (although RPLS or PRES may be diagnosed on MRI)
      1. Evaluate for other causes of Altered Level of Consciousness
    2. Hypertensive Encephalopathy typically improves with Blood Pressure lowering (even 10-20%)
      1. Other causes of Altered Level of Consciousness and Hypertension (e.g. CVA) do not improve as readily
    3. Asymptomatic Hypertension may present with very high Blood Pressure and mild Headache
      1. However, contrast with Hypertensive Encephalopathy in which patient's appear ill and are confused
  8. Differential Diagnosis
    1. See Altered Level of Consiousness
    2. Cerebrovascular Accident
    3. Eclampsia
    4. Encephalitis
    5. Hepatic Encephalopathy
    6. Uremic Encephalopathy
    7. Neuroleptic Malignant Syndrome
    8. Serotonin Syndrome
    9. Anticholingeric Syndrome
    10. Cytotoxic Chemotherapy reaction (esp. Serum Creatinine >1.8 mg/dl and Fluid Overload)
  9. Management
    1. See Hypertensive Emergency for agents to emergently lower Blood Pressure
    2. Nicardipine is typically recommended in Hypertensive Encephalopathy
    3. Lower Blood Pressure by 10-25% within first hour of presentation
      1. Further lowering as needed
  10. Prognosis
    1. Fully reversible in most cases within days to weeks
    2. In one study, nearly 25% died and another subset had permanent neurologic dysfunction
      1. Covarrubias (2002) Am J Neuroradiol 23(6): 1038-48 +PMID:12063238 [PubMed]
  11. References
    1. Swaminathan and Weingart in Herbert (2020) EM:Rap 20(2): 3-4
    2. Elliott and Varon (2016) Evaluation and Treatment of Hypertensive Emergencies, UpToDate, accessed 12/28/2016
    3. Neill (2016) Reversible Posterior Leukoencephalopathy Syndrome, UpToDate, accessed 12/28/2016
    4. Staykov (2012) J Intensive Care Med 27(1): 11-24 +PMID:21257628 [PubMed]

Hypertensive Encephalopathy (C0151620)

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
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
Derived from the NIH UMLS (Unified Medical Language System)

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
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
Derived from the NIH UMLS (Unified Medical Language System)

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, カギャクセイコウハクシツノウショウショウコウグン
Hungarian Reverzibilis posterior leukoencephalopathia syndroma, Posterior reverzibilis encephalopathia syndroma
Norwegian Reversibel bakre leukoencefalopatisyndrom
Derived from the NIH UMLS (Unified Medical Language System)

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