New Developments in Hypertensive Encephalopathy.

PURPOSE OF REVIEW: This review summarizes the latest science on hypertensive encephalopathy and posterior reversible encephalopathy syndrome (PRES). We review the epidemiology and pathophysiology of these overlapping syndromes and discuss best practices for diagnosis and management. RECENT FINDINGS: Diagnosis of hypertensive encephalopathy largely relies on exclusion of other neurological emergencies. We review the extensive causes of PRES and its imaging characteristics. Management strategies have not changed substantially in the past decade, though newer calcium channel blockers simplify the approach to blood pressure reduction. While this alone may be sufficient for treatment of hypertensive encephalopathy in most cases, management of PRES also depends on modification of other precipitating factors. Hypertensive encephalopathy and PRES are overlapping disorders for which intensive blood pressure lowering is critical. Further research is indicated to both in diagnosis and additional management strategies for these critical conditions.

Hypertensive brain stem encephalopathy.

A 48-year-old man presented with headache and extreme hypertension. Computed tomography showed diffuse brain stem hypodensity. Magnetic resonance imaging revealed diffuse brain stem vasogenic edema. Hypertensive brain stem encephalopathy is an uncommon manifestation of hypertensive encephalopathy, which classically occurs at parietooccipital white matter. Because of its atypical location, the diagnosis can be challenging. Moreover, the coexistence of hypertension and brain stem edema could also direct clinicians toward a diagnosis of ischemic infarction, leading to a completely contradictory treatment goal.

[THE EFFECTIVENESS OF DIAGNOSIS AND TREATMENT OF CEREBROVASCULAR DISORDERS IN HYPERTENSIVE ENCEPHALOPATHY IN ELDERLY PATIENTS BY STUDYING THE CHARACTERISTICS OF CEREBRAL HEMODYNAMICS AND CEREBRAL PERFUSION STATUS].

Intrigue progression of hypertensive encephalopathy (HE) in older patients is that the development of cognitive impairment and high blood pressure underestimated, aslo exist without clinical manifestations. In recent decades convincing proved that the basis for the development of various diseases is cerebral dysfunction systems regulating brain blood flow, including--autoregulation system, which largely affects the blood supply to the brain. This explains the fact that patients with chronic brain ischemia cerebral hemodynamic status largely depends on the condition and stability of the regulatory mechanisms of systemic and cerebral hemodynamics, particularly of systemic blood pressure, regional cerebral blood supply, normalization which, in the early stages of development disorders, prevents of serious complications. In this paper the theoretical generalization and new solution of scientific and practical problems of hypertension influence on the formation of chronic cerebral ischemia in elderly patients on a background of hypertension--specified risk factors and especially the formation of a comprehensive study on the basis of clinical and neurological data, tool sand methods for neuroimaging research developed and improved methods of diagnosis. Found that in elderly patients with HE and HBP observed significant (P < 0.05) increase in the thickness of the intima-media complex was significantly higher (dextra--1.12 ± 0.03 and sinistra--1.11 ± 0.03), than middle-aged patients with hypertension at HE, which constitutes a violation of the elastic properties of the vascular wall. Established correlation data radionuclide study ultrasonic duplex scanning of vessels of the head and neck. A negative correlation of intima-media and severity of lesions according to hypoperfusion of computer tomography single photon emission (r = -0.49; P < 0.05); confirming the progression of HE in elderly patients needs improvement and treatment.

Hypertensive encephalopathy as a late complication of autonomic dysreflexia in a 12-year-old boy with a previous spinal cord injury.

UNLABELLED: The scope of paediatric autonomic disorders is not well recognised, and paediatricians seem to be generally unaware of the complexity and diversity of their clinical manifestations. We report a 12-year-old boy presenting with hypertensive encephalopathy caused by autonomic dysreflexia. CONCLUSION: This observation emphasises the importance of the recognition of this rare autonomic disorder, which can have potentially life-threatening neurological complications.

Acute disseminated encephalomyelitis presenting with hypertensive emergency.

We report a 12-year-old girl presenting with acute disseminated encephalomyelitis (ADEM) along with hypertensive emergency. Hypertension persisted for few weeks following recovery and subsided with oral clonidine. Although autonomic instability in ADEM has been reported before, hypertensive emergency was not previously documented as presenting feature of ADEM.

Recurrent posterior reversible encephalopathy syndrome in a pediatric patient with end-stage renal disease.

BACKGROUND: Posterior reversible encephalopathy syndrome (PRES) is a condition manifested by altered mental status, seizures, headaches, and visual loss. Specific abnormalities are seen by computed tomography or magnetic resonance imaging. Awareness of this syndrome is important for Emergency Physicians because visual loss can be reversible with prompt treatment of the underlying cause. OBJECTIVE: We present a case of recurrent PRES in a 14-year-old female who presented to the Emergency Department (ED) for headache and photosensitivity. CASE REPORT: A patient with a history of end-stage renal disease of unknown etiology was brought in by her mother for headache and photosensitivity. The patient developed blurry vision, seized in the ED, and required intubation. She was discharged 2 weeks later with complete return of vision. The same patient presented to the ED many times during the next several months for hypertensive emergencies, with three subsequent episodes that involved either seizures, vision loss, or both. Each of the episodes resolved with aggressive control of blood pressure in the pediatric intensive care unit. CONCLUSIONS: PRES should be considered in all patients presenting to the ED with visual loss, seizures, or headache, and can be recurrent in some individuals. Prompt treatment can help prevent permanent vision loss.

Changes in middle cerebral artery velocity after carotid endarterectomy do not identify patients at high-risk of suffering intracranial haemorrhage or stroke due to hyperperfusion syndrome.

OBJECTIVES: To determine if significant increases in middle cerebral artery velocity (MCAV) or pulsatility index (PI) during and immediately after carotid endarterectomy (CEA) were predictive of patients suffering a stroke due to the hyperperfusion syndrome (HS) or intracerebral haemorrhage (ICH). METHODS: Transcranial Doppler (TCD) mean/peak MCAV and PI were recorded pre-operatively; pre-clamp; 1-min post-declamping; 10-min post-declamping and 30-min post-operatively. The study was divided into two time periods; Group 1 (1995-2007); where there was no formal guidance for managing post-CEA hypertension (PEH) and Group 2 (2008-2012); where written guidelines for treating PEH were available. RESULTS: 11/1024 patients in Group 1 (1.1%) suffered a stroke due to HS/ICH, compared to 0/426 patients (0.0%) in Group 2 (p = 0.02). In Group 1; intra-operative increases >100% in mean/peak MCAV and PI at 1 and 10-min post-clamp release had positive predictive values (PPV) of 1.2%, 6.3% and 20.0% and 2.9%, 8.0% and 16.6% respectively. Post-operatively; a >100% increase in mean and peak MCAV had a PPV of 6.3% and 2.7% respectively. CONCLUSION: We were unable to demonstrate that significant increases in MCAV and PI were able to predict patients at increased risk of suffering a post-operative stroke due to HS or ICH. The provision of written guidance for managing PEH in Group 2 patients was associated with virtual abolition of ICH/HS.

Hippocampal sclerosis and chronic epilepsy following posterior reversible encephalopathy syndrome.

Chronic epilepsy has rarely been reported after posterior reversible encephalopathy syndrome (PRES) and the association with hippocampal sclerosis has been suggested only once before. We report the case of a girl admitted at the age of 8 years with idiopathic nephrotic syndrome. On the second day of admission, she presented with focal complex seizures and cerebral MRI showed posterior encephalopathy and no hippocampal sclerosis. MRI after one month confirmed the diagnosis of PRES. The seizures recurred and the girl developed pharmacoresistant epilepsy and was admitted to our hospital for further investigation. Cerebral MRI three years after the diagnosis of PRES showed hippocampal sclerosis which was not present on the initial MRI. We conclude that there is a triggering role of PRES in the development of hippocampal sclerosis. Hippocampal sclerosis may have resulted from seizure-associated damage, alternatively, hypertensive encephalopathy may have led to hippocampal damage via a vascular mechanism.

Brainstem swelling and noncommunicating hydrocephalus caused by hypertensive brainstem encephalopathy.

Hypertensive encephalopathy is a life-threatening medical condition manifested by headache, confusion, seizures, and visual disturbance, and, if treatment is delayed, it may progress to coma and death [1, 2] (Chester et al., Neurology 28:928-939, 1978; Vaughan and Delanty, Lancet 356:411-417, 2000). Involvement of the brainstem with or without supratentorial lesions has been reported and is termed hypertensive brainstem encephalopathy (HBE). Cases of HBE involving supratentorial deep gray and white matter are rare and extensive hyperintensity was predominantly seen in brainstem regions on fluid-attenuated inversion recovery and T2-weighted magnetic resonance images. We present radiologic findings of a patient with HBE involving deep supratentorial gray and white matter, causing tonsillar herniation and noncommunicating hydrocephalus by mass effect.

Clinicopathologic and MRI characteristics of presumptive hypertensive encephalopathy in two cats and two dogs.

Two dogs and two cats were evaluated for the acute-onset of abnormal mentation, recumbency, and blindness. All cases had systemic hypertension, ranging from 180 mm Hg to 260 mm Hg. MRI of the brain disclosed noncontrast-enhancing, ill-defined, T2-weighted (T2W) hyperintensities in the white matter of the cerebrum in the areas of the frontal, parietal, temporal, and occipital lobes. Lesions were also observed in the caudate nuclei and thalamus (n = 1 in each). Intracranial hemorrhage was observed in one animal. Diffusion-weighted imaging (DWI) was consistent with vasogenic edema in two animals. Retinal lesions were observed in three animals. Hypertension was secondary to renal disease in three animals. A primary underlying disorder was not identified in one animal. Normalization of blood pressure was achieved with amlodipine either alone or in combination with enalapril. In one cat, hypertension spontaneously resolved. In three cases, neurologic improvement occurred within 24-48 hr of normalization of blood pressure. The presumptive diagnosis of hypertensive encephalopathy was supported by the MRI findings and neurologic dysfunction coincident with systemic hypertension in which the neurologic dysfunction improved with treatment of hypertension. The prognosis appears good for the resolution of neurologic deficits with normalization of blood pressure in animals with hypertensive encephalopathy.

Hypertensive encephalopathy as the initial manifestation of Guillain-Barré syndrome in a 7-year-old girl.

We report a case involving a 7-year-old girl who initially presented with seizure and hypertensive encephalopathy, serious autonomic manifestations associated with Guillain-Barré syndrome, followed by subsequent bilateral ascending flaccid paralysis. Cerebrospinal fluid analysis showed cytoalbuminous dissociation. Nerve conduction velocity tests showed features of demyelinating polyradiculoneuropathy. An immunofluorescence test of her serum was positive for mycoplasma immunoglobulin M antibody. The present case highlights the importance of considering the initial autonomic manifestations of Guillain-Barré syndrome in the differential diagnosis of unexplained acute hypertensive crisis.

Manifestations of hypertensive encephalopathy in cats.

OBJECTIVES: Hypertensive encephalopathy in cats is an important entity but is underestimated in clinical practice. This could be explained, in part, by non-specific clinical signs. The objective of this study was to characterise the clinical manifestations of hypertensive encephalopathy in cats. METHODS: Cats with systemic hypertension (SHT) recognised by routine screening, associated with underlying predisposing disease or a clinical presentation suggestive of SHT (neurological or non-neurological), were prospectively enrolled over a 2-year period. Confirmation of SHT was based on at least two sets of measurements of systolic blood pressure >160 mmHg by Doppler sphygmomanometry. RESULTS: Fifty-six hypertensive cats with a median age of 16.5 years were identified; 31 had neurological signs. In 16/31 cats, neurological abnormalities were the primary complaint. The other 15 cats were first presented to the medicine or ophthalmology service, and neurological disease was recognised based on the cat's history. The most common neurological signs were ataxia, various manifestations of seizures and altered behaviour. Individual cats also showed paresis, pleurothotonus, cervical ventroflexion, stupor and facial nerve paralysis. In 28/30 cats, retinal lesions were detected. Of these 28 cats, six presented with a primary complaint of visual deficits, and neurological signs were not the primary complaint; nine presented with non-specific medical issues, without suspicion of SHT-induced organ damage; in 13 cats, neurological issues were the primary complaint and fundic abnormalities were detected subsequently. CONCLUSIONS AND RELEVANCE: SHT is common in older cats and the brain is an important target organ; however, neurological deficits are commonly ignored in cats with SHT. Gait abnormalities, (partial) seizures and even mild behavioural changes should prompt clinicians to consider the presence of SHT. A fundic examination in cats with suspected hypertensive encephalopathy is a sensitive test to support the diagnosis.

Posterior reversible encephalopathy syndrome.

Posterior reversible encephalopathy syndrome (PRES) is characterized by headache, altered mental status, visual disturbances, and seizures. Radiological features typically include edema of the posterior cerebral regions, especially of the parietooccipital lobes. Atypical imaging features, such as involvement of anterior cerebral regions, deep white matter, and the brain stem are also frequently seen. Vasoconstriction is common in vascular imaging. Different conditions have been associated with PRES, but toxemia of pregnancy, solid organ or bone marrow transplantation, immunosuppressive treatment, cancer chemotherapy, autoimmune diseases, and hypertension are most commonly described. The pathophysiology of PRES is unclear and different hypotheses are being discussed. Posterior reversible encephalopathy syndrome is best managed by monitoring and treatment in the setting of a neurointensive care unit. The prognosis is usually benign with complete reversal of clinical symptoms within several days, when adequate treatment is immediately initiated. Treatment of severe hypertension, seizures, and withdrawal of causative agents represent the hallmarks of specific therapy in PRES. Delay in diagnosis and treatment may lead to permanent neurological sequelae. Therefore, awareness of PRES is of crucial importance for the intensivist.

Posterior reversible encephalopathy syndrome in childhood: report of four cases and review of the literature.

BACKGROUND: Posterior reversible encephalopathy syndrome (PRES) is a recently described disorder with typical radiological findings of bilateral gray and white matter abnormalities in the posterior regions of the cerebral hemispheres and cerebellum. Its clinical symptoms include headache, decreased alertness, mental abnormalities such as confusion, diminished spontaneity of speech, and changed behavior ranging from drowsiness to stupor, seizures, vomiting, and abnormalities of visual perception such as cortical blindness. In this study, the clinical and radiological findings of 4 children with this syndrome due to a variety of conditions are reported. METHODS: The records of 4 children with a diagnosis of PRES were retrospectively analyzed. RESULTS: PRES is associated with a disorder of cerebrovascular autoregulation of multiple etiologies. Four patients with PRES who had primary diagnoses of severe aplastic anemia, nephritic syndrome, Henoch-Schönlein purpura, and acute poststreptococcal glomerulonephritis are presented. This syndrome has been described in numerous medical conditions, including hypertensive encephalopathy, eclampsia, and with the use of immunosuppressive drugs. CONCLUSIONS: Early recognition of PRES as a complication during different diseases and therapies in childhood may facilitate precise diagnosis and appropriate treatment.

Malignant hypertension with reversible brainstem hypertensive encephalopathy and thrombotic microangiopathy.

A 42-year-old woman presented with headache and nausea. Severe hypertension, renal dysfunction, thrombocytopenia, and anemia were present. A magnetic resonance imaging (MRI) scan of her head revealed widespread hyperintense lesions located in the brainstem and cerebellum on T2-weighted and fluid-attenuated inversion recovery imaging. Hypertensive encephalopathy was suspected, and antihypertensive therapy was started. A second MRI of the patient's head on day 12 of hospitalization revealed that the hyperintensities in the brainstem and cerebellum had almost disappeared, and that thrombocytopenia, anemia, and renal dysfunction had also gradually improved. Test results led to a diagnosis of malignant hypertension. This patient was regarded as suffering from malignant hypertension with reversible brainstem hypertensive encephalopathy (RBHE) and thrombotic microangiopathy (TMA). RBHE and TMA are known to occur as complications of malignant hypertension, but there has been no previous report of them occurring simultaneously. RBHE and TMA related to malignant hypertension are both conditions that can be improved by the rapid institution of antihypertensive therapy, and as such, early diagnosis and treatment are important. When treating patients with malignant hypertension, the possibility that it may be complicated by both RBHE and TMA must be kept in mind.

[Cognitive impairment in elderly patients with acute hypertensive encephalopathy].

Acute hypertensive encephalopathy in elderly patients appears reversible mild cognitive impairment. The erythrocyte sedimentation rate and blood creatinine measured during a hypertensive crisis are predictors of decline of visual-spatial orientation after two weeks of treatment.

Acute cortical blindness in preeclampsia--a case of reversible posterior encephalopathy syndrome.

BACKGROUND: Cortical blindness is one the most disturbing symptoms of reversible posterior encephalopathy syndrome in preeclamptic and eclamptic patients. The disease has been previously associated with a hypertensive breakthrough in the autoregulation of posterior cerebral arterioles followed by extravasation of the fluid into the brain tissue. CASE: 22-year-old primigravida in the 39th week of gestation diagnosed with gestational diabetes mellitus presented with mild preeclampsia and was admitted to our hospital. Antihypertensive treatment was initiated. Her blood pressure remained between 120/80 to 140/90 mm Hg. Glucose levels were within acceptable range. Before the labor induction she developed acute cortical blindness. Magnetic resonance imaging showed vasogenic edema localized in occipital lobes. Cesarean section was performed and anti-edematous treatment initiated. Blindness resolved by the fifth day postpartum. CONCLUSIONS: Reversible posterior encephalopathy developed in our patient in spite of normalized blood pressure that remained within autoregulation limits. Alternative pathogenesis and precipitating factors are discussed.

Posterior reversible encephalopathy syndrome in childhood cancer.

BACKGROUND: Posterior reversible encephalopathy syndrome (PRES) is characterized by seizures, headaches, altered mental status, cortical blindness and typical transient lesions on magnetic resonance imaging. PATIENTS AND METHODS: We describe seven childhood cancer patients with clinical and radiological symptoms of PRES, and reviewed all well-documented PRES cases reported during childhood cancer treatment. RESULTS: Fifty-six children with PRES, including our 7 cases, were identified in the literature. Mean age at onset was 9 (range: 2-17) years. Primary diagnoses were acute lymphoblastic leukemia (n = 31), acute myeloid leukemia (n = 5), non-Hodgkin lymphoma (n = 7) and solid tumors (n = 13). PRES patients presented with seizures (n = 50), altered mental status (n = 20), visual disturbances (n = 24) and/or headaches (n = 17). PRES was associated with hypertension in 49 patients. About 86% of the patients had both clinical and radiological reversible symptoms. Four patients developed epilepsy, in one patient ataxia remained and one patient had a persistent mydriasis. CONCLUSION: Although PRES has predominantly been described in leukemia patients, it occurs in children with solid tumors as well. Hypertension seems to be the most important trigger for the occurrence of PRES during childhood cancer treatment. Seizures are the most common accompanying sign. Symptoms and radiological findings normalize in ∼90% of the cases, but in 10% neurological symptoms remain.

Posterior reversible encephalopathy syndrome after liver transplantation in children: a rare complication related to calcineurin inhibitor effects.

PRES is a neuroclinical and radiological syndrome that results from treatment with calcineurin inhibitor immunosuppressives. Severe hypertension is commonly present, but some patients may be normotensive. We report herein two children who received liver transplants, as treatment for biliary atresia in the first case and for Alagille's syndrome in the second one. In the early postoperative, both patients presented hypertension and seizures. In both cases, the image findings suggested the diagnosis of PRES. The CT scan showed alterations in the posterior area of the brain, and brain MRI demonstrated parietal and occipital areas of high signal intensity. Both children were treated by switching the immunosuppressive regimen and controlling arterial blood pressure. They displayed full recuperation without any neurologic sequelae. Probably, the pathophysiology of PRES results from sparse sympathetic innervation of the vertebrobasilar circulation, which is responsible for supplying blood to the posterior areas of the brain. In conclusion, all liver-transplanted children who present with neurological symptoms PRES should be considered in the differential diagnosis, although this is a rare complication. As treatment, we recommend rigorous control of arterial blood pressure and switching the immunosuppressive regimen.