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Hypertensive encephalopathy …
- Updated 01.29.2024
- Released 06.09.1999
- Expires For CME 01.29.2027
Hypertensive encephalopathy
Introduction
Overview
Hypertensive encephalopathy is a syndrome in which altered mental status, headache, vision changes, or seizures accompany elevated blood pressure. Failure of cerebral autoregulation from a sudden elevation of blood pressure results in endothelial injury and vasogenic edema. Radiographically, hypertensive encephalopathy most commonly presents with evidence of posterior-predominant T2-hyperintense lesions without pathologic contrast enhancement. Thus, hypertensive encephalopathy shares many clinical and radiographic features with posterior reversible encephalopathy syndrome (PRES). Although these changes are classically noted in the parietal and occipital lobes, edematous changes can also be found in the deep gray matter, brainstem, or cerebellum or even anteriorly. Clinical symptoms and radiographic findings are most often reversible with prompt blood pressure management.
Key points
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• Hypertensive encephalopathy results from endothelial dysfunction triggered by a sudden and sustained rise in blood pressure; the degree of elevation may be moderate, especially in patients without a history of hypertension. | |
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• Clinically, pathophysiologically, and radiographically, hypertensive encephalopathy shares many features of posterior reversible encephalopathy syndrome (PRES) and may be thought of as a subtype of this syndrome. | |
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• Clinically, manifestations include headache, altered mental status, visual disturbances, and seizures. | |
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• T2-weighted magnetic resonance imaging most commonly reveals posterior and white-matter-predominant vasogenic edema that does not respect the posterior cerebral artery territory boundary; this can help confirm the diagnosis and exclude mimics of hypertensive encephalopathy such as ischemic stroke. |
Historical note and terminology
Volhard was the first to distinguish hypertension-induced neurologic dysfunction from a uremic state and introduced the term “pseudouremia” to refer to hypertensive encephalopathy (55). Ten years later, the term “hypertensive encephalopathy” was introduced by Oppenheimer and Fishberg (42). With the advent of modern neuroimaging techniques in the 1980s, Rail and Perkins published a case series of patients with the clinical syndrome of hypertensive encephalopathy that also demonstrated hypodensity in the posterior white matter on computerized tomography (44). Several years later, Hauser and colleagues demonstrated that magnetic resonance imaging was even more sensitive for demonstrating increased T2 signal in the posterior white matter and occipital lobes (24). These radiographic findings in hypertensive encephalopathy, posterior reversible encephalopathy syndrome, and reversible posterior leukoencephalopathy syndrome supported the concept that the syndrome results from fluid and protein extravasation across the blood-brain barrier, owing to failure of cerebral autoregulation.
In 1996, Hinchey and colleagues published a series of patients who presented with headaches, vomiting, confusion, seizures, cortical blindness, and other visual abnormalities arising from various etiologies--eclampsia, acute hypertension, renal failure, or treatment with certain immunosuppressants. MRI imaging in all cases demonstrated posterior leukoencephalopathy without infarction that resolved after cessation of the purportedly triggering medication, delivery, or control of blood pressure. Hinchey and colleagues named this entity reversible posterior leukoencephalopathy syndrome, which was meant to capture the diverse etiologies resulting in an overlapping radiographic syndrome that also included hypertensive encephalopathy (27).
Their article sparked controversy in the literature, prompting others to point out that such patients were at risk of hemorrhage and ischemia, and, thus, the syndrome was not necessarily “reversible.” Moreover, many reported cases emphasized that the findings are not strictly “posterior”; such authors suggested “hyperperfusion syndrome” as an alternate name (47).
To address some of the inaccuracies conveyed by the name reversible posterior leukoencephalopathy syndrome, the term posterior reversible encephalopathy syndrome (PRES) was later coined in the radiology literature in 2000 (05). PRES has now been described as a result of a plethora of etiologies, but hypertension continues to be chief among them.
Clinical manifestations
Presentation and course
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• Hypertensive encephalopathy commonly presents with headache and altered mental status, ranging from mild confusion to coma. | |
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• Visual disturbances without fundoscopic changes are common. | |
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• MRI most often demonstrates T2-weighted hyperintensities in the posterior white matter bilaterally, representative of vasogenic edema; however, changes localized to the deep gray or anterior white matter may be found and do not exclude hypertensive encephalopathy. | |
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• Cerebral spinal fluid analysis and EEG monitoring may be abnormal, but findings are often nonspecific. |
Hypertension-induced neurologic signs and symptoms can be broadly divided into either focal deficits associated with chronic hypertension (eg, lacunar stroke or hypertensive basal ganglia hemorrhage) or diffuse cerebral dysfunction related to acute hypertension, which is described as “hypertensive encephalopathy.”
Patients with hypertensive encephalopathy most commonly present with headache, altered mental status--ranging from mild confusion to coma, nausea, vomiting, visual changes, and seizures. Headache is the most common symptom, occurring in more than 75% of patients with hypertensive encephalopathy (08). Gradual onset of morning headaches accompanied by nausea and vomiting is typical, and symptoms may progress to stupor, coma, and death if left untreated.
Visual disturbances are also common, even in absence of funduscopic abnormalities (29). The presence of cortical visual symptoms remains a helpful diagnostic feature corresponding to occipital lobe involvement. Retinal or optic disc ischemia and papilledema may be present in some patients with visual complaints (03), but more often, the fundi are unremarkable, and the visual symptoms relate to the involvement of the occipital lobe involvement (37; 02).
Typical visual symptoms may range from a nonspecific blurriness with normal acuity to color blindness, visual field defects, impairment of facial recognition (prosopagnosia), parietal lobe dysfunction with visual neglect, cortical blindness, denial of blindness (Anton syndrome), and visual hallucinations. Periodic alternating gaze deviation and nystagmus have also been reported when vasogenic edema involves the cerebellum (33).
The level of blood pressure elevation that will result in hypertensive encephalopathy is variable. However, a rapid rise in blood pressure in a previously normotensive patient is more likely to result in manifestations of hypertensive encephalopathy than a similar elevation in a chronically hypertensive patient; thus, a moderate elevation in blood pressure can be sufficient to produce the syndrome and should raise suspicion in the appropriate clinical context (41).
Acute renal failure leading to hypertension and volume overload as well as medication nonadherence are common precipitants. Use of mineralocorticoids, erythropoietin (10), or MAO-inhibitors, withdrawal of clonidine, and abuse of drugs with phencyclidine and cocaine all have been reported to cause hypertensive encephalopathy (19).
The condition may also be precipitated by Cushing disease, pheochromocytoma, and scleroderma (46; 54). One case report described cortical blindness in a patient with untreated malignant hypertension in the setting of undiagnosed pheochromocytoma (43). There have been two case reports of patients with SARS-CoV-2 infection developing seizures or headaches in the context of hypertension and later found to have posterior predominant white matter changes, which is consistent with the syndrome of hypertensive encephalopathy/PRES. Although the significance of hypertension in these cases is unclear, both patients had elevated blood pressures at the time of symptom onset, and one responded well to verapamil, which has both vasodilatory and blood pressure-lowering properties (12; 17). Another study looking at two case reports of patients with COVID-19 demonstrated that these patients may be susceptible to encephalopathy resulting from hypertension and that this may prolong ventilator time (31). It has been postulated that hypertension and, specifically, blood pressure variability, which is exacerbated by COVID-19’s effect on renin-angiotensin-aldosterone system, results in endothelial dysfunction and hypertensive encephalopathy (57).
Hypertensive encephalopathy may also occur in the pediatric population. In these cases, a secondary cause for hypertension is often discovered. For example, one retrospective study found that elevated blood urea nitrogen (BUN) levels were independently associated with hypertensive encephalopathy, possibly suggesting that kidney injury and resultant BUN elevation make children more susceptible to vascular autoregulation dysfunction (58). Another case report described hypertensive encephalopathy as the presenting symptom of a rare autosomal dominant disorder called homozygous familial hypercholesterolemia, which affects low-density lipoprotein receptor genes (35), whereas another case report described the presenting symptom as pandysautonomia (30).
These cases of severe “brainstem hypertensive encephalopathy” are important to recognize because a decompressive ventriculostomy (01) and cerebellar resection (06) may be lifesaving, although some may respond well to antihypertensive medication (52).
Prognosis and complications
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• Early recognition and correction of hypertension often results in reversal of the encephalopathy; without prompt treatment, permanently damaging ischemia or hemorrhage may result. | |
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• Malignant cerebral edema may develop and warrant treatment with osmotic therapy or ventriculostomy. |
Early recognition of hypertensive encephalopathy followed by prompt lowering of blood pressure will most often result in clinical recovery, usually within hours to several days, but sometimes the recovery is slower. Complete resolution of the MRI signal changes is often delayed, sometimes taking several weeks or months (45).
Delayed recognition and treatment of the clinical syndrome, on the other hand, may lead to permanent neurologic deficits or even death. Complications include cerebral hemorrhage and infarction. Overzealous lowering of blood pressure during the acute phase may also result in permanent neurologic deficit due to ischemic stroke, especially in border zone territories (18). One study showed an increasing rate of hypertensive encephalopathy in a French cohort from 2014 through 2022; hypertensive encephalopathy was associated with many comorbid conditions, which ultimately conveyed a poorer prognosis (22).
Rarely, the vasogenic edema that arises from endothelial cell dysfunction may be extreme enough to result in intracranial pressure crisis, which may progress to herniation if not immediately addressed (20). As such, transorbital sonography for noninvasive measurement of the optic nerve sheath diameter has been suggested as a way to noninvasively identify raised intracranial pressure (32).
In extreme cases, obstructive hydrocephalus may occur due to brainstem edema, causing compression of the cerebral aqueduct or fourth ventricle. Emergent ventriculostomy may be necessary (01) although in one case, the hydrocephalus resolved with aggressive blood pressure control (40).
Clinical vignette
A 38-year-old woman with systemic lupus erythematosus had a several-year history of hypertension. Two weeks after she was discharged from the hospital, after successful treatment of peritonitis related to her dialysis, she was readmitted, complaining of several days of headaches, nausea, unsteadiness of gait, and intermittent visual blurriness. On the morning of the admission, she had a seizure. Examination revealed elevated blood pressure of 200/140 mm Hg. She was edematous, confused, and drowsy. Her neck was supple, and her visual fields were full. Her fundi were remarkable for bilateral disc swelling and arteriolar narrowing without frank hemorrhages. An MRI revealed bilateral occipital high-intensity signal changes near the occipital pole on T2-weighted and FLAIR sequences. On further questioning, she stated that she had not been taking her antihypertensive medications due to financial hardship since her discharge from the hospital. Brief treatment with intravenous nitroprusside, followed by reinstitution of her oral blood pressure medications, resulted in rapid clinical improvement to her baseline, and she was discharged on the fourth hospital day.
Biological basis
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• The pathogenesis of hypertensive encephalopathy is incompletely understood but is likely the result of failure of cerebral autoregulation and endothelial injury. | |
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• Preferential involvement of the posterior circulation may be due to the decreased sympathetic innervation of the vertebrobasilar system. |
Etiology and pathogenesis
Failure to clearly separate the pathologic changes of chronic hypertension and acute hypertensive encephalopathy has delayed understanding of the pathogenesis of both. In addition, only few cases of acute hypertensive encephalopathy undergo brain biopsy as a diagnostic investigation, so the range of pathologic changes accompanying the disorder cannot be studied comprehensively.
In general, the pathophysiology of hypertensive encephalopathy can be regarded as failure of cerebral autoregulation from sudden elevation of blood pressure that results in endothelial injury and vasogenic edema. Persistent damage to small vessels leads to vessel stenosis and decreased vessel reactivity; in turn, there is a decrease in hemodynamic reserve with impaired autoregulation (51). Normally, cerebral perfusion is maintained despite fluctuations in systemic blood pressure by adjustment of arteriolar caliber. Acute hypertensive encephalopathy represents a failure of cerebral blood flow regulation beyond the autoregulatory range (50).
Using a mouse model developed for studying hypertension and vascular dementia, researchers have found MRI evidence of T2-hyperintense lesions in the cortical watershed and posterior white matter that resemble the changes humans have with hypertensive encephalopathy. Histopathology of these animals demonstrates severe blood-brain barrier disruption, white matter vacuolization, and microbleeds that are predominantly posterior (25). Additionally, the hematologic data demonstrate thrombocytopenia and is suggestive of thrombotic microangiopathy. TTP-HUS and thrombotic microangiopathy have been observed to concurrently occur in patients with hypertensive encephalopathy; however, it is uncertain if thrombotic microangiopathy plays a role in the pathogenesis of hypertensive encephalopathy or if these are merely two coincidental consequences of malignant hypertension (11).
Preferential posterior cerebral involvement in this syndrome may be due, in part, to decreased sympathetic innervation in the vertebrobasilar artery territory in contrast to more robust sympathetic innervation in the carotid system (14). More effective vasoconstriction of the carotid arterial territory results in reduction of distal perfusion pressure during malignant hypertension, whereas decreased sympathetic innervation in the vertebrobasilar distribution allows for higher capillary pressure that may overwhelm the autoregulatory limit. Distal border zone or watershed regions may be more vulnerable in hypertensive encephalopathy, but the reason is unclear (23).
Epidemiology
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• Accurate prevalence estimates of hypertensive encephalopathy are limited by variable clinical symptoms and poorly defined diagnostic criteria. |
The exact prevalence and incidence of hypertensive encephalopathy is unknown. Data from 1981 suggest that less than 1% of an estimated 60 million Americans with hypertension will have a hypertensive crisis (21). An even smaller portion of this group will have hypertensive encephalopathy. Data from a hospital in Ontario that tracked all visits to the emergency department for hypertension from 2002 to 2012 (total number of more than 200,000 visits) found that only 7.8% of visits resulted in hospitalization, and of those, less than 1% were ultimately diagnosed with hypertensive encephalopathy (38). In contrast, other studies suggest a higher incidence. An accurate estimate remains difficult due to poorly defined diagnostic criteria, variable and often limited clinical manifestations, arbitrarily defined upper limits of blood pressure, and lack of neuroradiologic documentation. Although hypertensive encephalopathy may occur at any age, it is more common in the second to fourth decades of life, in part due to the higher prevalence of eclampsia and nephritis.
Prevention
The best means of preventing hypertension encephalopathy is blood pressure control, usually with antihypertensive medications. For patients on an immunosuppressant treatment known to cause PRES or RPLS, blood pressure should also be closely tracked as these patients often develop clinical and radiographic changes of posterior reversible leukoencephalopathy in the context of concomitant hypertension (27).
Differential diagnosis
Confusing conditions
The most common symptoms of hypertensive encephalopathy—headache, nausea, and visual complaints—are nonspecific; thus, the differential diagnosis for this presentation initially can and should be broad. In patients with acute onset of headache and hypertension, subarachnoid hemorrhage or intraparenchymal hemorrhage must be considered. Patients with a more subacute presentation should be evaluated for venous sinus thrombosis or idiopathic intracranial hypertension.
Funduscopic abnormalities and proteinuria should be sought, but these may not be present. Gradual symptom onset over several days accompanied by significant blood pressure elevation and rapid improvement after lowering blood pressure are important in supporting hypertensive encephalopathy. Frequently, however, the lack of pathognomonic clinical features and absence of diagnostic laboratory or radiologic abnormalities make hypertensive encephalopathy a diagnosis of exclusion.
An MRI appearance of predominantly posterior cerebral white matter lesions with mild or absent hypertension should prompt investigation of the other etiologies of posterior reversible encephalopathy, such as eclampsia or exposure to immunomodulatory or chemotherapeutic agents, such as cyclosporine, methotrexate, interferon alpha, cisplatin, tacrolimus, and vascular endothelial growth factor inhibitor bevacizumab (16).
Depending on the clinical context, the differential also includes posterior cerebral artery territory infarction, venous infarcts, intracranial tumors, osmotic myelinolysis, vasculitis, and such infectious or inflammatory disorders as progressive multifocal leukoencephalopathy, multiple sclerosis, or acute disseminated encephalomyelitis.
Associated or underlying disorders
By definition, hypertension must be present to make a diagnosis of hypertensive encephalopathy. Common precipitants of an acute hypertensive crisis include acute or chronic renal failure with volume overload, medications that may induce hypertension (eg, MAO-inhibitors, vasopressors, clonidine withdrawal), or drugs of abuse (eg, cocaine, methamphetamines).
As discussed above, posterior reversible encephalopathy syndrome and eclampsia likely share a common pathophysiology of failed cerebral autoregulation and endothelial dysfunction.
Diagnostic workup
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• MRI is the most sensitive diagnostic modality for hypertensive encephalopathy. | |
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• CSF studies and EEG may be abnormal but are often nondiagnostic and should primarily be used to rule out other causes of leukoencephalopathy. |
Routine blood tests, urine analysis, and urine toxin screens will help to assess renal function and exclude exogenous toxins that may be associated with hypertensive encephalopathy.
Imaging studies with CT or MRI scans are particularly helpful in suspected cases, often revealing bilateral, posterior cortical, and white matter lesions that extend beyond a posterior cerebral artery territory. A T2-weighted or fluid-attenuated inversion-recovery MRI scan is particularly helpful in disclosing the full extent of the signal abnormality (05). The relative absence of mass effect or pathologic contrast enhancement helps to differentiate hypertensive encephalopathy from a tumor or an abscess. Bilateral posterior cerebral artery distribution infarction can be excluded by the frequent sparing of calcarine and paramedian occipital lobe structures in hypertensive encephalopathy. T2 changes found most often represent vasogenic edema without ischemia or infarct, as evidenced by the absence of diffusion restriction on MRI (48).
Although posterior lesions are the most commonly identified distribution, anterior lesions have also been reported and confirmed by biopsy, demonstrating white matter necrosis and micro-hemorrhage with no evidence of vasculitis (26). MRI may also reveal a central variant pattern with preferential involvement of brainstem, thalami, and periventricular white matter (07; 49).
Spinal fluid examination in patients with hypertensive encephalopathy is nondiagnostic. It is often obtained in non-classic cases to rule out alternative diseases such as encephalitis, malignancy, and vasculitis. Despite the frequent appearance of cerebral swelling and edema in patients with hypertensive encephalopathy, Datar and colleagues demonstrated that among patients diagnosed with posterior reversible leukoencephalopathy (84% of whom were hypertensive), the median opening pressure was 17 mmHg (normal is less than 20 mmHg). Mild elevation in the CSF protein was seen in most cases, with a median total protein level of 58 mg/dL (normal is less than 35 mg/dL). On univariate analysis, vasogenic edema involving the thalamus, cerebellum, brainstem, and basal ganglia was associated with higher CSF protein level. In addition, there was a direct correlation between CSF protein level elevation and the extent of vasogenic edema on brain MRI scan (09). Lymphocytic pleocytosis is unusual, and neutrophilic pleocytosis is distinctly rare (39). Its presence should suggest a separate underlying infectious or inflammatory disorder.
EEG recordings in patients with hypertensive encephalopathy and reversible cerebral vasoconstriction syndrome or posterior reversible leukoencephalopathy are frequently abnormal, but the findings are nondiagnostic, ranging from slowing of the background activity to epileptiform transients in up to 80% of patients (53).
Management
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• Hypertensive encephalopathy necessitates prompt hypertension control to the patient’s normal baseline over several hours. |
Hypertensive encephalopathy represents one of the true hypertensive emergencies, the others being progressive myocardial ischemia, aortic dissection, and renal insufficiency. Prompt but gradual lowering of the mean arterial pressure by approximately 25% (or diastolic to 110 to 120 mmHg) over several minutes to a couple of hours is warranted in most hypertensive emergencies (15). Parenteral antihypertensive agents should be first-line as oral agents cannot be titrated in a time-urgent fashion. In chronic, poorly controlled patients, higher diastolic levels should be targeted. Further lowering of the blood pressure to normal levels is a reasonable approach over ensuing weeks for long-term management. Careful blood pressure monitoring is necessary because a treatment-induced stroke may occur (34).
Patients should be evaluated for signs of increased intracranial pressure with noninvasive modalities like transorbital ultrasonography, transcranial dopplers, or pupillometry. If there is a concern for severe intracranial hypertension, invasive intracranial pressure monitoring may be needed to guide treatment.
Outcomes
With blood pressure management, most patients with hypertensive encephalopathy will recover with accompanying normalization of the MRI findings over days to weeks. However, delay in treatment may result in hemorrhage or, less commonly, ischemia. Although blood pressure control is necessary, as a result of the pathologic cerebral vasoregulation and pressure-dependent cerebral perfusion, abrupt blood pressure control may result in ischemia, especially in border-zone areas (34).
Special considerations
Pregnancy
Although eclampsia has long been recognized to cause a similar clinical syndrome as hypertensive encephalopathy, it was not until 1935 that Volhard suggested “eclamptic uremia” might have the same underlying pathophysiology as hypertensive encephalopathy (56). Advanced neuroimaging confirmed the similarities in the radiographic findings: mainly posterior-predominant vasogenic areas of vasogenic edema, which are reversible with the treatment of eclampsia.
Thus, hypertensive encephalopathy and the encephalopathy of (pre)eclampsia likely share a common pathogenesis of endothelial dysfunction and failure of cerebral vasculature autoregulation. Pathologic studies support this shared mechanism of disease and have demonstrated cerebral edema and petechial hemorrhages, especially in the parieto-occipital and occipital lobes. Microscopically, these petechiae are ring hemorrhages around capillaries and precapillaries occluded by fibrinoid material (13).
Like posterior reversible leukoencephalopathy, there are primarily two theories that might explain the encephalopathy of eclampsia. In the first, circulating endogenous cytokines damage renovascular networks, resulting in the abrupt onset of hypertension. This theory posits that the change in blood pressure and perfusion pressure drives pathology (04). Alternatively, endogenic cytotoxins caused by eclampsia may drive endothelial dysfunction and disrupt vascular integrity triggering fluid extravasation and edema formation (36). As described above, it is hypothesized antiangiogenic factors such as sFlt-1 (soluble vascular endothelial growth factor receptor 1) and sEng (soluble endoglin) trap circulating vascular endothelial growth factor and transforming growth factor Beta resulting in endothelial dysfunction and vasogenic edema in multiple organ systems, including the brain (28).
Treatment is aimed at prompt lowering of blood pressure and expedited delivery of the fetus to prevent seizures and further neurologic complications. If treatment is not delayed, clinical and radiographic changes are most often reversible.
Media
References
- 01
- Adamson DC, Dimitrov DF, Bronec PR. Upward transtentorial herniation, hydrocephalus, and cerebellar edema in hypertensive encephalopathy: Case report and literature review. Neurologist 2005;11(3):171-5. PMID 15860139
- 02
- Bakshi R, Bates VE, Mechtler LL, Kinkel PR, Kinkel WR. Occipital lobe seizures as the major clinical manifestation of reversible posterior leukoencephalopathy syndrome: magnetic resonance imaging findings. Epilepsia 1998;39:295-9. PMID 9578048
- 03
- Beck RW, Gamel JW, Willcourt RJ, Berman G. Acute ischemic optic neuropathy in severe preeclampsia. Am J Ophthalmol 1980;90:342-6. PMID 7425050
- 04
- Belfort MA, Varner MW, Dizon-Townson DS, Grunewald C, Nisell H. Cerebral perfusion pressure, and not cerebral blood flow, may be the critical determinant of intracranial injury in preeclampsia: a new hypothesis. Am J Obstet Gynecol 2002;187:626-34. PMID 12237639
- 05
- Casey SO, Sampaio RC, Michel E, Truwit CL. Posterior reversible encephalopathy syndrome: utility of fluid-attenuated inversion recovery MR imaging in the detection of cortical and subcortical lesions. AJNR Am J Neuroradiology 2000:21(7):1199-206. PMID 10954269
- 06
- Chang GY. Hypertensive brainstem encephalopathy requiring cerebellar resection. Neurosurg Q 2004;14:179-80.
- 07
- Chang GY, Keane JR. Hypertensive brainstem encephalopathy: three cases presenting with severe brainstem edema. Neurology 1999;53:652-4. PMID 10449143
- 08
- Clarke E, Murphy EA. Neurological manifestations of malignant hypertension. Br Med J 1956;2:1319-26. PMID 13374330
- 09
- Datar S, Singh TD, Fugate JE, Mandreskar J, Rabinstein AA, Hocker S. Albuminocytologic dissociation in posterior reversible encephalopathy syndrome. Mayo Clin Proc 2015;90(10):1366-71. PMID 26349950
- 10
- Delanty N, Vaughan C, Frucht S, Stubgen P. Erythropoietin-associated hypertensive posterior leukoencephalopathy. Neurology 1997;49:686-9. PMID 9305323
- 11
- Deguchi I, Uchino A, Suzuki H, Tanahashi N. Malignant hypertension with reversible brainstem hypertensive encephalopathy and thrombotic microangiopathy. J Stroke Cerebrovasc Dis 2012;21(8):915.e17-20. PMID 22418002
- 12
- Djellaoui A, Seddik L, Cleret De Langavant L, Cattan S, Bachoud-Levi AC, Hosseini H. Posterior reversible encephalopathy syndrome associated with SARS-CoV-2 infection. J Neurol Neurosurg Psychiatry 2020. [Epub ahead of print] PMID 32718920
- 13
- Donaldson JO. The brain in eclampsia. Hypertens Pregnancy 1994;13:115-33.
- 14
- Edvinsson L, Owman C, Sjoberg NO. Autonomic nerves, mast cells, and amine receptors in human brain vessels. A histochemical and pharmacologic study. Brain Res 1976;115(3):377-93. PMID 184880
- 15
- Garcia JY Jr, Vidt DG. Current management of hypertensive emergencies. Drugs 1987;34:263-78. PMID 3304968
- 16
- Glusker P, Recht L, Lane B. Reversible posterior leukoencephalopathy syndrome and bevacizumab. N Engl J Med 2006;354:980-1. PMID 16510760
- 17
- Gómez-Enjuto S, Hernando Requejo V, Lapena-Motilva J, et al. Verapamil as treatment for refractory status epilepticus secondary to PRES syndrome on a SARS-Cov-2 infected patient. Seizure 2020;80:157-8. PMID 32574838
- 18
- Graham DI. Ischaemic brain damage of cerebral perfusion type after treatment of severe hypertension. Br Med J 1975;4:739. PMID 1212586
- 19
- Grewal R, Miller BL. Cocaine induced hypertensive encephalopathy. Acta Neurol (Napoli) 1991;13:279-81. PMID 1927636
- 20
- Griswold WR, Viney J, Mendoza SA, James HE. Intracranial pressure monitoring in severe hypertensive encephalopathy. Crit Care Med 1981;9(8):573-6. PMID 6790226
- 21
- Gudbrandsson T. Malignant hypertension. A clinical follow-up study with special reference to renal and cardiovascular function and immunogenetic factors. Acta Med Scand Suppl 1981;650:1-62. PMID 6948498
- 22
- Halimi JM, de Fréminville JB, Gatault P, et al. Characteristics and prognosis of patients with hypertensive encephalopathy: a French nationwide cohort study. Hypertension 2023;80(8):1716-27. PMID 37283073
- 23
- Hatashita S, Hoff JT, Ishii S. Focal brain edema associated with acute arterial hypertension. J Neurosurg 1986;64:643-9. PMID 3950747
- 24
- Hauser RA, Lacey DM, Knight MR. Hypertensive encephalopathy. Magnetic resonance imaging demonstration of reversible cortical and white matter lesions. Arch Neurol 1988;45(10):1078-83. PMID 3178528
- 25
- Herisson F, Zhou I, Mawet J, et al. Posterior reversible encephalopathy syndrome in stroke-prone spontaneously hypertensive rats on high-salt diet. J Cereb Blood Flow Metab 2019;39(7):1232-46. PMID 29350576
- 26
- Hernandez-Duran S, Barrantes-Freer A, Rohde V, von der Brelie C. Posterior reversible encephalopathy syndrome presenting in the anterior circulation with malignant intracranial hypertension requiring surgical decompression: a case report and literature review. Acta Neurochir (Wien) 2017;159(7):1321-4. PMID 28516363
- 27
- Hinchey J, Chaves C, Appignani B, et al. A reversible posterior leukoencephalopathy syndrome. N Engl J Med 1996;334:494-500.** PMID 8559202
- 28
- Hod T, Cerdeira AS, Karumanchi SA. Molecular mechanisms of preeclampsia. Cold Spring Harb Perspect Med 2015;5(10) pii: a023473. PMID 26292986
- 29
- Jellinek EH, Painter M, Prineas J, Russell RR. Hypertensive encephalopathy with cortical disorders of vision. Q J Med 1964;130:239-56. PMID 14152973
- 30
- Kamate M, Basavanagowda T. Pan-dysautonomia manifesting as hypertensive encephalopathy in children. Indian J Pediatr 2023;90(9):942. PMID 37222848
- 31
- Kishfy L, Casasola M, Banankhah P, et al. Posterior reversible encephalopathy syndrome (PRES) as a neurological association in severe Covid-19. J Neurol Sci 2020;414:116943. PMID 32474362
- 32
- Lochner P, Lesmeister M, Nardone R, Orioli A, Siniscalchi A, Naldi A. Posterior reversible encephalopathy syndrome: role of transorbital ultrasound. Neurol Sci 2021;42(3):1171-4. PMID 329591184
- 33
- Mackay D, Garcia RZ, Galetta S, Prasad S. Periodic alternating gaze deviation and nystagmus in posterior reversible encephalopathy syndrome. Neurol Clin Pract 2014;4(6):482-5. PMID 29443162
- 34
- Mak W, Chan KH, Cheung RTF, Ho SL. Hypertensive encephalopathy: BP lowering complicated by posterior circulation ischemic stroke. Neurology 2004;63:1131-2. PMID 15452320
- 35
- Maldar SB, Pinto CJ. Homozygous familial hypercholesterolaemia in patient presenting with hypertensive encephalopathy. BMJ Case Rep 2022;15(10):e250265. PMID 36316049
- 36
- Marra A, Vargas M, Striano P, Del Guercio L, Buonanno P, Servillo G. Posterior reversible encephalopathy syndrome: the endothelial hypotheses. Med Hypotheses 2014;82(5):619-22. PMID 24613735
- 37
- Marra TR, Shah M, Mikus MA. Transient cortical blindness due to hypertensive encephalopathy: magnetic resonance imaging correlation. J Clin Neuroophthalmol 1993;13:35-7. PMID 8501259
- 38
- Masood S, Austin PC, Atzema CL. A population-based analysis of outcomes in patients with a primary diagnosis of hypertension in the emergency department. Ann Emerg Med 2016;68(3):258-67.e5. PMID 27395439
- 39
- McDonald CK, Waters ML, Griffin FM Jr. Case report: neutrophilic CSF pleocytosis in hypertensive encephalopathy. Am J Med Sci 1993;306:167-8. PMID 8128978
- 40
- Moughal S, Ahmad S, Saleem N, Derham C. Hypertensive posterior reversible encephalopathy causing obstructive hydrocephalus. Surg Neurol Int 2023;14:94. PMID 37025546
- 41
- Obeid T, Awada A. Posterior leukoencephalopathy without severe hypertension: utility of diffusion-weighted MRI. Neurology 1999;53(6):1372-3. PMID 10522914
- 42
- Oppenheimer BS, Fishberg AM. Hypertensive encephalopathy. Arch Intern Med 1928;41:264-78.
- 43
- Patel SP, Jarbath M, Saravis L, et al. Pheochromocytoma manifesting as cortical blindness secondary to PRES with associated TMA: a case report and literature review. BMC Endocr Disord 2022;22(1):205. PMID 35971105
- 44
- Rail DL, Perkin GD. Computerized tomographic appearance of hypertensive encephalopathy. Arch Neurol 1980;37(5):310-1. PMID 7387454
- 45
- Roth C, Ferbert A. The posterior reversible encephalopathy syndrome: what’s certain, what’s new. Pract Neurol 2011;11(3):136-44. PMID 21551107
- 46
- Sauza-Sosa JC, Zenteno-Langle R, Zamora-Medina MDC. Hypertensive emergency in a woman with systemic sclerosis. High Blood Press Cardiovasc Prev 2020;27(6):597-9. PMID 33006010
- 47
- Schwartz RB. A reversible posterior leukoencephalopathy syndrome. N Engl J Med 1996;334(26):1743. PMID 8637524
- 48
- Schwartz RB, Mulkern RV, Gudbjartsson H, Jolesz F. Diffusion-weighted MR imaging in hypertensive encephalopathy: clues to pathogenesis. AJNR Am J Neuroradiol 1998;19(5):859-62. PMID 9613500
- 49
- Srinivasan KG, Balasubramanian P, Mayilvaganan KR, Kannan UN, Bilal M. Central variant of posterior reversible encephalopathy syndrome – a rare case report. J Clin Diagn Res 2017;11(4):TD01-TD02. PMID 2871231
- 50
- Strandgaard S, Paulson OB. Cerebral autoregulation. Stroke 1984;15:413-6. PMID 6374982
- 51
- Svyrydova N. Improving diagnostic approaches to cerebrovascular disorders in patients with hypertensive encephalopathy. J Educ Health Promot 2022;11:269. PMID 36325209
- 52
- Tan YY, Tan K. Hypertensive brainstem encephalopathy: a diagnosis often overlooked. Clin Med (Lond) 2019;19(6):511-3. PMID 31732595
- 53
- Thomas SV, Somanathan N, Radhakumari R. Interictal EEG changes in eclampsia. Electoencephalogr Clin Neurophysiol 1995;94(4):271-5. PMID 7537199
- 54
- Vaidya PN, Baysal B, Finnigan NA. Scleroderma and renal crisis. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing, 2021. PMID 29494031
- 55
- Volhard F. In: Mohr and Staehelin’s Handbuch der inneren Medizin. Vol 3. Berlin: Springer, 1918:1342.
- 56
- Volhard F. Clinical aspects of Bright’s disease. In: Berglund H, Medes G, Huber GL, et al, editors. The kidney in health and disease. Philadelphia: Lea and Febiger, 1935:665-88.
- 57
- Wijeratne T, Wijeratne C, Karimi L, Sales C, Crewther SG. Case report: posterior reversible leukoencephalopathy syndrome (PRES) as a biologically predictable neurological association in severe COVID-19. First reported case from Australia and review of internationally published cases. Front Neurol 2021;11:600544. PMID 33584499
- 58
- Yang Z, Huang Y, Qin Y, Pang Y. Clinical characteristics and factors associated with hypertension in 205 hospitalized children: a single-center study in southwest China. Front Ped 2021;9:620158. PMID 33898356
Contributors
All contributors' financial relationships have been reviewed and mitigated to ensure that this and every other article is free from commercial bias.
Authors
-
Jimmy Suh MD
Dr. Suh of the Medical University of South Carolina has no relevant financial relationships to disclose.
See Profile -
Ganga Navada MD
Dr. Navada of the Medical University of South Carolina has no relevant financial relationships to disclose.
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Editor
-
Douglas J Lanska MD MS MSPH
Dr. Lanska of the University of Wisconsin School of Medicine and Public Health and the Medical College of Wisconsin has no relevant financial relationships to disclose.
See Profile
Former Authors
- Sashank Prasad MD
- Catherine Albin MD
Patient Profile
- Age range of presentation
-
- 6 years to 65+ years
- Sex preponderance
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- male=female
- Heredity
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- none
- Population groups selectively affected
-
- none selectively affected
- Occupation groups selectively affected
-
- none selectively affected
ICD & OMIM codes
- ICD-9
-
- Hypertensive encephalopathy: 437.2
- ICD-10
-
- Hypertensive encephalopathy: I67.4
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