Characteristics and Prognosis of Patients With Hypertensive Encephalopathy: A French Nationwide Cohort Study.

BACKGROUND: Hypertensive encephalopathy (HE) constitutes a serious condition, usually observed in patients with long-lasting hypertension. Hypertension-associated HE is sometimes differentiated from the stroke-associated hypertensive emergency. Whether prognosis of hypertension-associated and stroke-associated HE is different is unclear. METHODS: Characteristics and prognosis of HE were assessed in this nationwide retrospective cohort study in all patients with an administrative code of HE compared with age-, sex- and year of inclusion-matched controls admitted to French hospitals during the 2014 to 2022 period. RESULTS: HE was identified in 7769 patients. Chronic kidney disease (19.3%), coronary artery disease (13.8%), diabetes (22.1%), and ischemic stroke (5.2%) were frequent but thrombotic microangiopathy, hemolytic-uremic syndrome, systemic sclerosis or renal infarction were <1%. HE prognosis was poor (death: 10.4%/y, heart failure: 8.6%/y, end-stage kidney disease: 9.0%/y, ischemic stroke: 3.6%/y, hemorrhagic stroke: 1.6%/y, dementia: 4.1%/y). The risk of death was increased to a similar extent in patients with HE, regardless of the presence of known hypertension or concomitant stroke (versus patients without HE). Among patients with HE, known hypertension was significantly associated with increased risks of ischemic stroke, hemorrhagic stroke, heart failure, vascular dementia, and all-cause dementia and to a lesser extent with chronic dialysis in multivariable analyses including adjustment on concomitant stroke. CONCLUSIONS: HE remains a considerable health burden and is associated with a poor prognosis. The distinction between hypertension- versus stroke-associated HE is relevant as these 2 situations convey different risks of stroke, heart failure, vascular dementia, and end-stage kidney disease.

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.

Charles Bonnet Syndrome Associated With Recurrent Hypertensive Crisis.

Charles Bonnet syndrome (CBS) is a disorder of visual hallucinations in psychologically normal patients with ocular disease or damage to visual pathways. The etiology of CBS is not fully understood. It is associated with various triggers, with age-related macular degeneration the most common; other triggers are systemic diseases such as stroke, multiple sclerosis, and anemia as well as lighting issues, fatigue, and medical or surgical eye treatments. Visual disturbances such as decreased visual acuity, visual field deficits, or visual hallucinations are common in association with hypertensive encephalopathy. We describe a patient with episodic CBS triggered by recurrent hypertensive crises, which resolved with blood pressure management in the hospital setting.

Homozygous familial hypercholesterolaemia in a patient presenting with hypertensive encephalopathy.

Homozygous familial hypercholesterolaemia (HoFH) is a disorder affecting low-density lipoprotein (LDL) receptor genes. Patients typically have a triad of elevated LDL-cholesterol (LDL-C), xanthomatosis and premature atherosclerotic cardiovascular disease. Our patient, a preteen boy, presented with signs of hypertensive encephalopathy. Physical examination showed arcus cornealis, planar xanthomas and tuberous xanthomas. After appropriate investigations, a direct aetiology of the hypertension could not be elucidated; however, our patient's hypertension resolved with the reduction in serum lipid levels. ß-hydroxy ß-methylglutaryl coenzyme A reductase and cholesterol absorption inhibitors were administered as first-line treatment. A significant proportion of patients with HoFH continue to have elevated LDL-C levels, thereby requiring second-line agents, such as proprotein convertase subtilisin/kexin type inhibitors (evolocumab), microsomal triglyceride transfer protein inhibitors (lomitapide) and angiopoietin-like protein inhibitors (evinacumab). This case report aimed to raise awareness among paediatricians to consider HoFH as a possible aetiology in a child presenting with hypertension and suggestive physical findings.

Primary pigmented nodular adrenal disease presenting as hypertensive crisis.

We present a case of a young girl who presented with hypertensive crisis and recent onset weight gain with hirsutism. On evaluation for Cushing syndrome (CS), her cortisol concentration was high, showed a paradoxical cortisol rise on dexamethasone suppression and the adrenocorticotropic hormone (ACTH) was low. Adrenal imaging showed normal adrenal morphology. Genetic diagnosis of primary pigmented nodular adrenal disease (PPNAD) was made. She was operated for bilateral adrenalectomy and histopathology confirmed the diagnosis of PPNAD. Our case highlights the rare aetiology of PPNAD as a cause of CS resulting in a hypertensive crisis. To the best of our knowledge, this is the youngest case of ACTH independent CS presenting as hypertensive encephalopathy.

Status epilepticus in black African patients with hypertensive encephalopathy: a rare entity that must not be underrated.

The severity of a blood pressure spike is more closely associated with serious organ dysfunction, which can be life-threatening in the short term, than with the blood pressure level itself. A hypertensive emergency is defined as the presence of high blood pressure associated with acute organ dysfunction. The specific nature of high blood pressure in black patients may cause more frequent hypertensive emergencies. In this retrospective case study, we report our experience and highlight the specific prognosis for black African patients. We examined three patients, aged 27, 47, and 59 years, admitted to intensive care for a hypertensive emergency with neurological distress, and all in status epilepticus. Average blood pressure was 171 mm HG. Treatment included intubation, ventilation, and induction of a barbiturate coma, plus antihypertensive treatment. The outcome was favorable, with an average stay of 5 days. The frequency of hypertensive emergencies varies according to age, ethnic origin, and period studied. Black patients often suffer from more severe forms of high blood pressure, arising at an earlier age. Hypertensive encephalopathy can occur in patients with or without chronic hypertension. Without treatment, the encephalopathy induces a coma that can quickly become fatal. Its spontaneous course is catastrophic (10-20% survival at one year), but more favorable with adequate treatment (60-80% survival at five years).

A novel mutation in the glucocorticoid receptor gene as a cause of severe glucocorticoid resistance complicated by hypertensive encephalopathy.

BACKGROUND: Glucocorticoid resistance syndrome (GRS) is caused by mutations of the glucocorticoid receptor (coded by the NR3C1 gene) and presents with signs of mineralocorticoid and/or androgen excess. PATIENT: A female patient presented at the age of almost 3 years with hypertensive and hypoglycemic seizure. She was diagnosed with GRS and was treated with antihypertensive medications and dexamethasone. She was later found to have MRI findings of punctuate microinfarcts at the basal ganglia, left thalamus and pons, possibly associated with uncontrolled hypertension. Increase of the dexamethasone dose up to 14 mg/day resulted in sufficient control of her symptoms. RESULTS: Two mutations in the NR3C1 gene were identified: a novel mutation in exon 2 (p.E198X), and a previously described mutation in exon 8 (p.R714Q). CONCLUSION: GRS may present with life-threatening complications; this is the first report of hypertensive encephalopathy in association with GRS. Successful management of patients might require high doses of dexamethasone to control blood pressure.

Advanced Pulmonary Hypertension Due to Congenital Double-shunt Successfully Treated with Surgical Repair and Up-front Combination Therapy.

This case report concerns a 22-year-old woman with large patent ductus arteriosus and atrial septal defect. She was referred to our hospital because of exertional dyspnea and was revealed to have advanced pulmonary arterial hypertension (PAH) with a mean pulmonary arterial pressure (PAP) of 79 mmHg. Although both shunts had bidirectional flow, based on the results of acute pulmonary vasoreactive testing, one-stage surgical closure was performed followed by up-front combination therapy for post-operative pulmonary hypertensive crisis and residual PAH. At 14 months after the surgery, her symptoms were markedly improved, and her mean PAP had dramatically decreased to 13 mmHg.

Managing hypertriglyceridemia in children with systemic lupus erythematosus: two sides of the same coin / Manejo de la hipertrigliceridemia en niños con lupus eritematoso sistémico: 2 caras de la misma moneda

Hypertriglyceridemia is common in children with systemic lupus erythematosus (SLE). A retrospective analysis of the baseline clinical-pathological presentation and treatment outcome (status of lipid profiles) was performed in two children with SLE, who presented with extreme hypertriglyceridemia over a follow-up period of four weeks. The children were treated with prednisolone, mycophenolate mofetil (MMF), hydroxychloroquine and hypolipidemic agents, depending on their disease status. On serial follow-up, the first child showed a significantly raised serum triglyceride level after receiving one week of oral prednisolone therapy. Anti-lipoprotein-lipase (LPL) autoantibody was absent. Lipid profile levels of this child gradually improved after replacing oral prednisolone with another immunosuppressant, namely MMF. The second child presented with extreme hypertriglyceridemia with positive anti-LPL autoantibody. She responded to plasmapheresis followed by increasing the dose of immunosuppressant. So, extreme hypertriglyceridemia in children with SLE may be steroid induced or due to presence of anti-LPL auto antibody. Management should be individualized depending on the etiology (AU)

La hipertrigliceridemia es común en niños con lupus eritematoso sistémico. Un análisis retrospectivo de la presentación clínico-patológica al inicio del estudio y los resultados del tratamiento (estado de los perfiles lipídicos) se llevó a cabo en 2 niños con lupus eritematoso sistémico, que presentaron hipertrigliceridemia extrema durante un período de seguimiento de 4 semanas. Los niños fueron tratados con prednisolona, micofenolatomofetil, hidroxicloroquina y agentes hipolipidemiantes, dependiendo de su estado de salud. En el seguimiento, el primer niño mostró un nivel de triglicéridos en suero significativamente elevado después de recibir una semana de tratamiento con prednisolona oral. No existían anticuerpos antilipoproteína lipasa. Los niveles de perfil de lípidos de este niño mejoraron gradualmente después de sustituir la prednisolona oral con otro inmunosupresor, a saber micofenolatomofetil. El segundo niño presentó hipertrigliceridemia extrema con autoanticuerpos antilipoproteína lipasa-positivos. Ella respondió a la plasmaféresis seguida del incremento de la dosis de inmunosupresores. Por lo tanto, la hipertrigliceridemia extrema en niños con lupus eritematoso sistémico puede ser inducida por esteroides o debido a la presencia de autoanticuerpos antilipoproteína lipasa. El manejo debe ser individualizado en función de la etiología (AU)

A Case of Hypertensive Encephalopathy with Enlarged Optic Nerve Sheath Measured by Transorbital Sonography.

This case report describes our experience in using transorbital sonography to evaluate pathological changes in the central nervous system in hypertensive encephalopathy. A 49-year-old man with nausea, headache, and mild confusion was diagnosed with hypertensive encephalopathy by brain magnetic resonance imaging (MRI), which revealed vasogenic edema in the bilateral thalamus and the brain stem. Lumbar puncture showed no severe intracranial hypertension. Transorbital sonography showed an increase in the optic nerve sheath diameter (ONSD). Repeated examination revealed a return of the ONSD to an almost normal range after a reduction in blood pressure and a resolution of symptoms. An improvement in cerebral vasogenic edema was confirmed by brain MRI. ONSD might be related to the severity of cerebral vasogenic edema. Repeated measurement of ONSD by transorbital sonography may be useful to assess the pathological course and the effect of treatment in hypertensive encephalopathy.

Does this patient have hypertensive encephalopathy?

A 63-year-old man was admitted to our hospital for further investigation and management of brain metastases. The patient was initially presented with a 4-day history of confusion. On the day of admission, the patient was confused, agitated, disorientated in place and time, and had visual disturbances. His blood pressure was repeatedly recorded high, with levels of systolic blood pressure between 170-210 mm Hg. A brain magnetic resonance imaging showed areas of high signal on T2 and fluid-attenuated inversion recovery images, located bilaterally in the white matter of the occipital regions and unilateral in the left frontal lobe, suggestive of posterior reversible encephalopathy syndrome. Aggressive treatment of hypertension resulted in complete resolution of the clinical and radiologic features of the syndrome.

Reversible Obstructive Hydrocephalus from Hypertensive Encephalopathy: A Condition that Neurosurgeons Must Know / Hidrocefalia obstrutiva reversível secundária a encefalopatia hipertensiva: uma condição que o neurocirurgião deve conhecer

The vasogenic edema in structures of posterior fossa secondary to elevated hydrostatic pressure can cause obstructive hydrocephalus, a condition called "reversible obstructive hydrocephalus from hypertensive encephalopathy." A case of a 27-year-old woman with arterial hypertension and sign of raised intracranial pressure is reported. Her radiologic studies have showed vasogenic cerebellar edema without structural lesion. This edema leads to obstruction of the cerebral aqueduct. We discuss the clinical and therapeutic aspects of this condition. In this clinical situation, the accurate control of blood pressure is themain goal on medical care, and the need of permanent ventricular shunt is quite infrequent.

O edema vasogênico secundário a elevação da pressão hidrostática capilar pode causar hidrocefalia obstrutiva, a chamada Hidrocefalia Obstrutiva Reversível secundária a Encefalopatia Hipertensiva. Nós apresentamos umcaso de umamulher de 27 anos com hipertensão arterial e sinais de hipertensão intracraniana. Sua investigação radiológica evidenciou sinais de edema vasogênico cerebelar bilateral sem lesão estrutural, determinando obstrução do aqueduto cerebral Nós discutimos os aspectos clínicos e terapêuticos deste caso. Nesta condição, o adequado controle da pressão arterial é a principal medida terapêutica, sendo a necessidade de derivação liquórica permanente infrequente.

[Efficacy of the energy-modifier cytoflavin in the treatment of patients with hypertensive encephalopathy].

AIM: To evaluate the efficacy of cytoflavin in the treatment of patients with hypertensive encephalopathy (HE). SUBJECTS AND METHODS: One hundred and forty patients aged 39 to 73 years, diagnosed with HE, were examined and randomized to 2 groups. A study group (n = 74) received cytoflavin in a dose of 2 tablets b.i.d. on days 1 to 25 days inclusive during standard basic therapy. A comparison group (n = 66 persons) had standard basic therapy only. A control group consisted of 30 apparently healthy individuals. The investigators studied the frequency of headache, dizziness, and other complaints and the intensity of cephalalgic syndrome, by using a visual analog scale, the quality of life by the Medical Outcomes Study 36-Item Short-Form Health Survey (MOS SF-36) questionnaire, that of sleep by the subjective sleep characteristics questionnaire elaborated at the Moscow City Somnological Center, the level of asthenia by a subjective asthenia rating scale (Multidimensional Fatigue Inventory (MFI-20), and autonomic status, by applying objective and subjective scales on days 1 and 25 of therapy. RESULTS: The study has shown that cytoflavin used in the above dose for 25 days reduces the frequency and magnitude of complaints of headache, dizziness, "venous" complaints, the degree of autonomic and asthenic disorders, and impairments in the quality of sleep and life in the patients with HE at all disease stages. A stepwise discriminant analysis has indicated that the degree of cephalgic syndrome, and autonomic disorders, and worse sleep quality are the most effective points for using the energy-modifier cytoflavin. CONCLUSION: HE treatment based on the current pathogenetic principles may have a preventive impact on the development of HE or slow down the rate of its progression.