History

Fact Explanation
History of hypertension Almost all patients have a history of hypertension. [4,5]
Drug history Patients who are taking sympathomimetic agents or illicit drugs (amphetamines, cocaine, monoamine oxidase inhibitors or phencyclidine) are at risk of developing hypertensive encephalopathy (HE). [6]
Irritability Irritability and lethargy are the earliest clinical symptoms which are sometimes neglected by the patient. [1]
Headache Severe headache is also a common presentation. When the systemic hypertension develops cerebral perfusion pressure increases. In order to prevent the cerebral hyper-perfusion, cerebral auto-regulatory mechanisms cause cerebral vasoconstriction, leading to cerebral ischemia and edema. This in turn increases the intracranial pressure and activates the Cushing reflex and further increases the systemic blood pressure. This process then continues as a vicious cycle, leading to diffuse cerebral dysfunction. [1]
Confusion and coma Due to cerebral dysfunction. [1]
Visual disturbances Blurred vision is a common complain. This is due to diffuse cerebral dysfunction. [1]
Seizures [1] Due to diffuse cerebral dysfunction. Generalized seizures are the commonest presentation of HE in children. [2] Often seizures are preceded by headache. [1]
Nausea and or vomiting Nausea and vomiting are common complaints. [7]
Epistaxis This is a late presentation. [1]
Hematuria and or oliguria [8] Due to hypertensive nephropathy.
Symptoms of cerebrovascular accidents Cerebrovascular accidents can occur in HE. Patients can present with hemiplegia, transient cortical blindness or hemiparesis. [1]
Symptoms of ischemic heart disease Ischemic heart disease is an end organ complication of HE. Patients can present with symptoms of myocardial infarction (severe retrosternal chest pain which radiates to the left arm and neck.) or heart failure (shortness of breath on exertion or at rest, orthopnea). [1]
Symptoms of aortic dissection Aortic dissection is a complication of the disease. Sudden, severe tearing type of chest, back, or abdominal pain, which radiates to the back, is characteristic of acute aortic dissection. [3]
References
  1. SHARIFIAN M. Hypertensive Encephalopathy Iran J Child Neurol [online] 2012, 6(3):1-7 [viewed 01 June 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3943026
  2. NATIONAL HIGH BLOOD PRESSURE EDUCATION PROGRAM WORKING GROUP ON HIGH BLOOD PRESSURE IN CHILDREN AND ADOLESCENTS. The fourth report on the diagnosis, evaluation, and treatment of high blood pressure in children and adolescents. Pediatrics [online] 2004 Aug, 114(2 Suppl 4th Report):555-76 [viewed 01 June 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/15286277
  3. BRAVERMAN A. C.. Acute Aortic Dissection: Clinician Update. Circulation [online] December, 122(2):184-188 [viewed 01 June 2014] Available from: doi:10.1161/CIRCULATIONAHA.110.958975
  4. ELLIOTT WILLIAM J.. Clinical Features and Management of Selected Hypertensive Emergencies. J Clin Hypertension [online] 2004 October, 6(10):587-592 [viewed 02 June 2014] Available from: doi:10.1111/j.1524-6175.2004.03608.x
  5. TISDALE J. E. Risk factors for hypertensive crisis: importance of out-patient blood pressure control. Family Practice [online] 2004 August, 21(4):420-424 [viewed 02 June 2014] Available from: doi:10.1093/fampra/cmh412
  6. SRIKANTH PENUMETSA JAYA MALLIDI. Management of Hypertensive Emergencies. J Hypertens [online] 2013 December [viewed 02 June 2014] Available from: doi:10.4172/2167-1095.1000117
  7. ILLE O., WOIMANT F., PRUNA A., CORABIANU O., IDATTE J. M., HAGUENAU M.. Hypertensive Encephalopathy After Bilateral Carotid Endarterectomy. Stroke [online] 1995 March, 26(3):488-491 [viewed 02 June 2014] Available from: doi:10.1161/01.STR.26.3.488
  8. FELDSTEIN C. Management of hypertensive crises. Am J Ther [online] 2007 Mar-Apr, 14(2):135-9 [viewed 03 June 2014] Available from: doi:10.1097/01.pap.0000249908.55361.de

Examination

Fact Explanation
Blood pressure Blood pressure should be measured in both arms. In most patients diastolic blood pressure is more than 120 mm Hg or systolic blood pressure is more than 180 mm Hg. [4]
Ophthalmoscopic (funduscopic) examination Grade IV retinal changes are associated with hypertensive encephalopathy (papilledema, hemorrhage, exudates, and cotton-wool spots). Papilledema is only present in malignant hypertension. [1,2,3]
Examination of the nervous system Presence of focal neurological signs, which are commonly transient will favor the diagnosis of stroke. Some patients present with altered mental status, or coma. [2,3]
Examination of the cardiovascular system Peripheral edema, elevated neck veins and dyspnea can be seen if the patient is in cardiac failure. Tachycardia and presence of gallop rhythm with third heart sound (S3) are all suggestive of cardiac failure. After myocardial infarction cardiac murmurs can occur due to valve lesions. Absent or delayed pulsations are suggestive of aortic dissection. [2,3,4]
Examination of the respiratory system Pulmonary edema, rales, and wheezes can be present with congestive cardiac failure. Pulmonary edema can occur secondary to hypertensive nephropathy as well. [2]
References
  1. AHMED ME, WALKER JM, BEEVERS DG, BEEVERS M. Lack of difference between malignant and accelerated hypertension. Br Med J (Clin Res Ed) [online] 1986 Jan 25, 292(6515):235-237 [viewed 31 May 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1339207
  2. ELLIOTT WILLIAM J.. Clinical Features and Management of Selected Hypertensive Emergencies. J Clin Hypertension [online] 2004 October, 6(10):587-592 [viewed 02 June 2014] Available from: doi:10.1111/j.1524-6175.2004.03608.x
  3. SRIKANTH PENUMETSA JAYA MALLIDI. Management of Hypertensive Emergencies. J Hypertens [online] 2013 December [viewed 02 June 2014] Available from: doi:10.4172/2167-1095.1000117
  4. FELDSTEIN C. Management of hypertensive crises. Am J Ther [online] 2007 Mar-Apr, 14(2):135-9 [viewed 03 June 2014] Available from: doi:10.1097/01.pap.0000249908.55361.de

Differential Diagnoses

Fact Explanation
Status epilepticus Status epilepticus can also cause high blood pressure due to autonomic disturbances. [2]
Subarachnoid hemorrhage Subarachnoid hemorrhage can present with acute severe headache and patients can have high blood pressure. CT or MRI aids in making the diagnosis. Lumbar puncture will show xanthochromic appearance or cerebrospinal fluid can be frankly blood stained. [1]
Acute cerebral infarction Examination of the nervous system will localize the possible area of involvement and CT or MRI will be helpful in differentiating ischemic and hemorrhagic strokes. [1]
Head trauma Head trauma can activates the Cushing's reflex resulting hypertension and bradycardia. [1]
Encephalitis [3] Low grade fever, mild headache, reduced appetite and fatigue are usual complains. Some may present with seizures, confusion, photophobia and unsteady gait. [4]
Uremic encephalopathy Uremic encephalopathy can present with neurological manifestations of the disease like, confusion, seizures, altered behavior and coma. [5]
Pheochromocytoma Pheochromocytoma can present with either sustained or paroxysmal hypertension, episodes of severe headache, palpitations and sweating. Excess of catecholamines is detected by assessing 24-h urinary catecholamine levels. CT or MRI of the abdomen will localize the adrenal tumor. [6,7]
References
  1. ELLIOTT WILLIAM J.. Clinical Features and Management of Selected Hypertensive Emergencies. J Clin Hypertension [online] 2004 October, 6(10):587-592 [viewed 02 June 2014] Available from: doi:10.1111/j.1524-6175.2004.03608.x
  2. NANDHAGOPAL R. Generalised convulsive status epilepticus: an overview Postgrad Med J [online] 2006 Nov, 82(973):723-732 [viewed 02 June 2014] Available from: doi:10.1136/pgmj.2005.043182
  3. KENNEDY P. VIRAL ENCEPHALITIS: CAUSES, DIFFERENTIAL DIAGNOSIS, AND MANAGEMENT J Neurol Neurosurg Psychiatry [online] 2004 Mar, 75(Suppl 1):i10-i15 [viewed 02 June 2014] Available from: doi:10.1136/jnnp.2003.034280
  4. Encephalitis Paediatr Child Health [online] 1998, 3(1):47-48 [viewed 03 June 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2851264
  5. KANG E, JEON SJ, CHOI SS. Uremic Encephalopathy with Atypical Magnetic Resonance Features on Diffusion-Weighted Images Korean J Radiol [online] 2012, 13(6):808-811 [viewed 03 June 2014] Available from: doi:10.3348/kjr.2012.13.6.808
  6. REISCH N, PECZKOWSKA M, JANUSZEWICZ A, NEUMANN HP. Pheochromocytoma: presentation, diagnosis and treatment. J Hypertens [online] 2006 Dec, 24(12):2331-9 [viewed 03 June 2014] Available from: doi:10.1097/01.hjh.0000251887.01885.54
  7. GULLER U, TUREK J, EUBANKS S, DELONG ER, OERTLI D, FELDMAN JM. Detecting Pheochromocytoma: Defining the Most Sensitive Test Ann Surg [online] 2006 Jan, 243(1):102-107 [viewed 03 June 2014] Available from: doi:10.1097/01.sla.0000193833.51108.24

Investigations - for Diagnosis

Fact Explanation
CT or MRI of the head Cerebral edema can be detected. [2] Symmetrical and sub-cortical hyper-intense lesions are seen in MRI and focal hypodensities are seen in CT. [1]
References
  1. JOSÉ BILLER, JOSÉ M. FERRO. Neurologic Aspects of Systemic Disease Part I: Handbook of Clinical Neurology. Newnes, Jan 9, 2014, pp. 162.
  2. KARAMPEKIOS S K, CONTOPOULOU E, BASTA M, TZAGOURNISSAKIS M, GOURTSOYIANNIS N. Hypertensive encephalopathy with predominant brain stem involvement: MRI findings. J Hum Hypertens [online] 2004 February, 18(2):133-134 [viewed 03 June 2014] Available from: doi:10.1038/sj.jhh.1001654

Investigations - Followup

Fact Explanation
Full blood count Microangiopathic hemolytic anemia is a known complication of HE. [1]
Urine toxicology screen Helps to diagnose drug induced HE. [5]
CT or MRI of the head Detects intracranial hemorrhages, which can occur as a complication of HE. [3]
Chest X-ray Chest X-ray helps in diagnosing heart failure (pulmonary edema, Kerley B lines, cardiomegaly, upper lobe diversion, pleural effusions). Cardiomegaly is also seen in chronic hypertension. Aortic dissection is favored by the presence of abnormal aortic contour and or wide aortic silhouette. [2,3]
ECG Detects myocardial infarction. ECG shows evidence of chronic hypertension (left ventricular hypertrophy) and arrhythmia as well. [3,4]
Cardiac biomarkers Assessment of cardiac biomarkers enables the diagnosis of myocardial infarction. [4]
Urine full report In renal hypertension urine full report will show hematuria and or proteinuria. [1,4]
Blood urea nitrogen Elevated in renal hypertension. [1,4]
Serum creatinine Elevated in renal hypertension. [1,4]
Renal biopsy If the initial investigations are suggestive of a renal etiology for hypertension renal biopsy will be helpful for the differentiation of post streptococcal glomerulonephritis and membranoproliferative glomerulonephritis which are common histoligical types. [1]
References
  1. SHARIFIAN M. Hypertensive Encephalopathy Iran J Child Neurol [online] 2012, 6(3):1-7 [viewed 01 June 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3943026
  2. BRAVERMAN A. C.. Acute Aortic Dissection: Clinician Update. Circulation [online] December, 122(2):184-188 [viewed 01 June 2014] Available from: doi:10.1161/CIRCULATIONAHA.110.958975
  3. ELLIOTT WILLIAM J.. Clinical Features and Management of Selected Hypertensive Emergencies. J Clin Hypertension [online] 2004 October, 6(10):587-592 [viewed 02 June 2014] Available from: doi:10.1111/j.1524-6175.2004.03608.x
  4. SRIKANTH PENUMETSA JAYA MALLIDI. Management of Hypertensive Emergencies. J Hypertens [online] 2013 December [viewed 02 June 2014] Available from: doi:10.4172/2167-1095.1000117
  5. JOSÉ BILLER, JOSÉ M. FERRO. Neurologic Aspects of Systemic Disease Part I: Handbook of Clinical Neurology. Newnes, Jan 9, 2014, pp. 162.

Management - General Measures

Fact Explanation
Health education Patients with chronic hypertension should be advised to exercise regularly and to reduce weight if the BMI is high. Low salt diet, abstinence from alcohol and tobacco use are also helpful. Importance of regular clinic follow up and antihypertensive drug treatment should also be emphasized. [1]
Furosemide [2] Effective in pulmonary edema and volume overload (congestive cardiac failure or renal failure).
Oxygen [2] Keep the patient at ease especially when pulmonary edema is present.
References
  1. JOSÉ BILLER, JOSÉ M. FERRO. Neurologic Aspects of Systemic Disease Part I: Handbook of Clinical Neurology. Newnes, Jan 9, 2014, pp. 164.
  2. FELDSTEIN C. Management of hypertensive crises. Am J Ther [online] 2007 Mar-Apr, 14(2):135-9 [viewed 03 June 2014] Available from: doi:10.1097/01.pap.0000249908.55361.de

Management - Specific Treatments

Fact Explanation
Blood pressure control The primary goal of the treatment should be to reduce the blood pressure without reducing the cerebral perfusion pressure, as the cerebral auto regulatory mechanisms take some. Mean arterial pressure should be reduced by 20% and the diastolic blood pressure should be reduced to 100-110 mm Hg during the 1st hour. Blood pressure should not be reduced by more than 95% of the presenting value till 24- 48 hours. [1,3,4]
Sodium nitroprusside This is the drug of choice in HE and administered dose is 0.5 μg/kg/min. Sodium nitroprusside dilates both arteries and veins and reduces myocardial oxygen demand. [1,2,4]
Nitroglycerin Allows rapid reduction of blood pressure by its venodilatory action. When cardiac failure and or myocardial ischemia are associated with HE nitroglycerin is useful as it can improve both conditions. The use should be limited to 24 to 48 hours as tolerance to nitroglycerin develops. [1,4]
Nicardipine A dihydropyridine-derivative calcium channel blocker, with cerebral and cardiac vasodilatory effects. It is effective in reducing cerebral ischemia as well. Reflex tachycardia is a side-effect of the treatment. [4]
Fenoldopam A dopaminergic-1 receptor agonist which causes arterial vasodilatation. By acting on the kidneys it increases renal blood flow and natriuresis. [4]
Hydralazine Hydralazine acts very slowly and maximum blood pressure reduction is achieved after about 30minutes, making hydralazine less effective in HE. [3]
Labetalol Labetalol is a nonselective beta-blocker which reduces the systemic blood pressure without affecting the cerebral perfusion pressure. [4]
Phentolamine This is an alpha blocker, used to treat hypertension secondary to pheochromocytoma and secondary to cocaine or other sympathomimetic drug use. Phentolamine is administered as 5 to 10 mg, boluses. [1,4]
References
  1. ELLIOTT WILLIAM J.. Clinical Features and Management of Selected Hypertensive Emergencies. J Clin Hypertension [online] 2004 October, 6(10):587-592 [viewed 02 June 2014] Available from: doi:10.1111/j.1524-6175.2004.03608.x
  2. SRIKANTH PENUMETSA JAYA MALLIDI. Management of Hypertensive Emergencies. J Hypertens [online] 2013 December [viewed 02 June 2014] Available from: doi:10.4172/2167-1095.1000117
  3. SHARIFIAN M. Hypertensive Encephalopathy Iran J Child Neurol [online] 2012, 6(3):1-7 [viewed 03 June 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3943026
  4. FELDSTEIN C. Management of hypertensive crises. Am J Ther [online] 2007 Mar-Apr, 14(2):135-9 [viewed 03 June 2014] Available from: doi:10.1097/01.pap.0000249908.55361.de