The Intractable Migraneous Headache

Migraine headaches results from dysfunction of brainstem pathways that normally modulate sensory input, with disordered activity of blood vessels. This leads to neurogenic inflammation in pain-sensitive arteries, the dura, and meningeal tissues, and promotes local vasodilatation. Medications such as sumatriptan and ergot derivatives cause vasoconstriction and relieve the symptoms. These triggers stimulate trigeminovascular axons causing pain and the release of vasoactive neuropeptides from perivascular axons. 

Clinical Presentation

• Headache that lasts from 4 to 72 hours
• Aggravation by routine physical activity
• Classically unilateral and pulsating (May not be unilateral)
• a/w Nausea, vomiting, and photophobia or photophobia 
• May have Scalp Tenderness 
• Aura (most common are visual auras with flashes of lights)

Key Q - Is your headache similar to your usual migraine headaches? If the news is no, then a different cause should be suspected. 

Treatment Options for Migraine

• Acetaminophen - Patients with diagnosed migraine often try paracetamol before arriving to the EDs. There is no harm in adding Paracetamol to routine migraine therapy.
• Fluids - Dehydration is a known trigger of migraine. Persistent nausea and vomiting further exacerbates the migraine. Adequate hydration might improve patient malaise and help those who are dehydrated.
• Anti-emetics - Parenteral metoclopramide, chlorpromazine, and prochlorperazine (Stemetil) all have demonstrated efficacy in randomized trials as monotherapy for acute migraine. Antiemetic medications are efficacious and are recommended for acute migraine in the ED. To reduce the risk of akathisia, diphenhydramine should be included. (Diphenhydramine does not prevent Metoclopramide induced dystonia)
• Oxygen - "High-flow" oxygen has been studied in migraine headaches. When compared with placebo, oxygen used for 15 min was more effective in pain relief with no significant adverse events.
• Steroids - Steroids prevent the lingering of attacks and recurrence as well. Everyone with a migraine attack should receive dexamethasone 10mg IV/IM or a 3 day course of presdqisolone unless there is an obvious contraindication. 
• NSAIDs - NSAIDs are effective in treating acute migraine. Ketorolac is a potent pain killer which often works for migraine.

• Triptans - Evidence suggests that SC delivery is fastest and most effective route. Common side effects include injection site reactions, dizziness, and paresthesias. Triptans are contraindicated in cardiovascular disease, pregnancy, basilar migraines, Prinzmetal angina, and ischemic stroke, and with the use of ergotamines within the previous 24 hours. Studies have also suggested that triptan therapy is less effective in patients with prolonged and severe migraine. Triptans are best reserved for patients who present within 1-2 hours of headache. Given the side effect profile, lack of efficacy in severe migraine, and relative contraindications, triptan use in the ED is of limited value. 
• Butyrophenones - (haloperidol and droperidol). RCTs have demonstrated efficacy of haloperidol and droperidol mono therapy. However, these drugs have been associated with frequent side effects (somnolence, akathisia, anxiety) and also prolong QTc interval. These are generally reserved for rescue therapy in refractory migraine. 
• Ergot Alkaloids - Dihydroergotamine (DHE), a 5-HT_1B/1D serotonin receptor agonist which highly effective in relieving headaches. However, DHE causes vomiting in a significant proportion of patients. Patients therefore should be pretreated with an antiemetic such as metoclopramide or prochlorperazine.
• Opioids - Use opioids only as the last sort for refractory headache. When compared with NSAIDs, DHE, and antiemetic medications, opioids are less effective for migraine. The frequent use of opioids in chronic and recurrent headache conditions may lead to adverse effects and may even exacerbate headaches.
• Propofol - Evidence for using propofol is not robust but it is again something to consider when everything else fails. Follow the procedural sedation protocol and doe as 10-20 mg IVP every 3-4 minutes up to 1 mg/kg. Our aim is mild sedation, not apnea.
• Magnesium - Magnesium is ideally used for refractory migraine, migraine patients with true aura, those with hypoMg. Administer 2 g IV Mg over 20min. Evidence for the use of Migraine is still conflicting though. 

• Sphenopalatine Ganglion Block

Bottomline: It is reasonable to start with fluids/O2, Metoclopramide. Do not forget to add steroids. Add Acetaminophen/NSAIDs if symptoms persist. Other  potential options (Ergot Alkaloids/Mg/Propofol, Ganglion Blocks) are all based on your comfort and local protocols. Opioids are your last option. 

References:

• Singhal AB, Maas MB, Goldstein JN, et al. High-flow oxygen therapy for treatment of acute migraine: A randomized crossover trial. Cephalalgia. 2016 May 20.
• Balbin JE, Nerenberg R, Baratloo A, Friedman BW. Intravenous fluids for migraine: a post hoc analysis of clinical trial data. Am J Emerg Med. 2016 Apr;34(4):713-6.
• Bigal ME, Bordini CA, Tepper SJ, Speciali JG. Intravenous magnesium sulphate in the acute treatment of migraine without aura and migraine with aura. A randomized, double-blind, placebo-controlled study. Cephalalgia. 2002 Jun;22(5):345-53.
• Colman I, Friedman BW, Brown MD, Innes GD, Grafstein E, Roberts TE, Rowe BH. Parenteral dexamethasone for acute severe migraine headache: meta-analysis of randomised controlled trials for preventing recurrence. BMJ. 2008 Jun 14;336(7657):1359-61.
• Colman I, Rothney A, Wright SC, Zilkalns B, Rowe BH. Use of narcotic analgesics in the emergency department treatment of migraine headache. Neurology. 2004 May 25;62(10):1695-700.
• Friedman BW, Bender B, Davitt M, Solorzano C, Paternoster J, Esses D, Bijur P, Gallagher EJ. A randomized trial of diphenhydramine as prophylaxis against metoclopramide-induced akathisia in nauseated emergency department patients. Ann Emerg Med. 2009 Mar;53(3):379-85.
• Friedman BW, Greenwald P, Bania TC, Esses D, Hochberg M, Solorzano C, Corbo J, Chu J, Chew E, Cheung P, Fearon S, Paternoster J, Baccellieri A, Clark S, Bijur PE, Lipton RB, Gallagher EJ. Randomized trial of IV dexamethasone for acute migraine in the emergency department. Neurology. 2007 Nov
• Regan LA, Hoffman RS, Nelson LS. Slower infusion of metoclopramide decreases the rate of akathisia. Am J Emerg Med. 2009 May;27(4):475-80.
• Soleimanpour H, Taheraghdam A, Ghafouri RR, Taghizadieh A, Marjany K, Soleimanpour M. Improvement of refractory migraine headache by propofol: case series. Int J Emerg Med. 2012 May 15;5(1):19.
• Taggart E, Doran S, Kokotillo A, Campbell S, Villa-Roel C, Rowe BH. Ketorolac in the treatment of acute migraine: a systematic review. Headache. 2013 Feb;53(2):277-87.

Utility of Lying/Standing Blood Pressure (Orthostatic Vitals)

What is Orthostatic Hypotension?
A patient is considered to have orthostatic hypotension when the systolic blood pressure falls by more than 20 mm Hg,m or diastolic blood pressure drop by more than 10 mm Hg, or pulse rise of more than 20-30 beats per minute within 3-5 minutes of standing. 

Don't be surprised if you hear different numbers from med students/nurses. There is a great deal of variation in how Orthostatics are checked and what numbers are used to call them significant. 

Image taken from http://www.antonygormley.com/sculpture/item-view/id/249

Ideally, Orthostatic Vitals should be done after the patient has been supine for about 5 minutes with the head flat. A drop in BP and increase in HR is a normal compensatory mechanism but it is thought that in mild dehydration, this normal response is exaggerated. Vitals are checked first in supine position and then after 3-5 minutes of standing. 

Symptoms of orthostatic hypotension are lightheadedness, dizziness, blurred vision, weakness, fatigue, nausea, palpitations, headache, and syncope.


When do we get Orthostatic Vitals?
Lying/Standing blood pressure or Orthostatic Vitals are generally done for patients with h/o volume loss (vomiting, dehydration, syncope, those who are on diuretics, alpha blockers) to make an assumption about their volume status. Majority of physicians treat orthostatic hypotension with Oral/IV fluids regardless of a patient's Orthostatic Symptoms. 

I often get asked about this whenever I try and admit a high risk syncope under Internal Medicine. Emergency Physicians are aware of these pitfalls of Orthostatic Vitals, but we continue to play this game of numbers to sell admits to our colleagues who strongly believe in Orthostatics. Watch Anand Swaminathan diving into the literature behind these numbers.


How reliable are Orthostatic Vitals to predict mild dehydration?
Current literature states that orthostatic vital signs are highly unreliable to predict mild volume loss. Almost 50% can test positive for orthostatic vitals even when asymptomatic. In addition, elderly may have multiple other reasons such as autonomic neuropathy, medications that interfere with orthostatic vitals making them even more unreliable. Looking for orthostatic symptoms is more reliable than treating the numbers. 

Orthostatic Hypotension Debunked - Anand Swaminathan from Statenislandem on Vimeo.


Problems with Orthostatic Vitals

• Poor Sensitivity and Specificity
• Poor reliability in elderly due to altered physiology, mediations, neuropathies
• Time Consuming (Nurses hate doing it!)
• Wide variation in how these numbers are interpreted 
• Often not done in an ideal manner

Take Home:

• Treat the patient, not these numbers (Treat Orthostatic Symptoms)
• Orthostatic Vitals are not reliable to predict mild volume loss

References:

1.         Naccarato M, Leviner S, Proehl J, et al. Emergency Nursing Resource: orthostatic vital signs. Journal of emergency nursing: JEN : official publication of the Emergency Department Nurses Association. Sep 2012;38(5):447-453.

2.         Koziol-McLain J, Lowenstein SR, Fuller B. Orthostatic vital signs in emergency department patients. Annals of emergency medicine. Jun 1991;20(6):606-610.

3.         Ooi WL, Barrett S, Hossain M, Kelley-Gagnon M, Lipsitz LA. Patterns of orthostatic blood pressure change and their clinical correlates in a frail, elderly population. Jama. Apr 23-30 1997;277(16):1299-1304.

4.         Skinner JE, Driscoll SW, Porter CB, et al. Orthostatic heart rate and blood pressure in adolescents: reference ranges. Journal of child neurology. Oct 2010;25(10):1210-1215.

5.         Stewart JM. Transient orthostatic hypotension is common in adolescents. The Journal of pediatrics. Apr 2002;140(4):418-424.

6.         Baraff LJ, Schriger DL. Orthostatic vital signs: variation with age, specificity, and sensitivity in detecting a 450-mL blood loss. The American journal of emergency medicine. Mar 1992;10(2):99-103.

7.         Witting MD, Wears RL, Li S. Defining the positive tilt test: a study of healthy adults with moderate acute blood loss. Annals of emergency medicine. Jun 1994;23(6):1320-1323.

8.         Johnson DR, Douglas D, Hauswald M, Tandberg D. Dehydration and orthostatic vital signs in women with hyperemesis gravidarum. Academic emergency medicine : official journal of the Society for Academic Emergency Medicine. Aug 1995;2(8):692-697.

Diffuse Esophageal Spasms (DES)

Diffuse Oesophageal Spasm, also called as "Prinzmetal Angina of the GI tract" is characterized by oesophageal contractions that are uncoordinated, simultaneous, or rapidly propagated. Usually, several segments of the esophagus contract simultaneously, preventing the propagation of the food bolus. 

Several patients are discharged from ED with a diagnosis of Non-Cardiac Chest pain or MSK pain when the ACS work up turns out to be negative. However,  Esphageal Spasm is not a ED based diagnosis. It often requires a trial of medications such Nitrates/CCBs or studies such as Manometry or Barium Swallow to reach a definitive diagnosis. Regardless, it is important for Emergency Physicians to be aware of this condition to arrange appropriate follow with Gastroenterologists. 

Symptoms:

• Non-Exertional Retrosternal Chest Pain which frequently radiates to the back, and can be more severe than angina (May sound like dissection, pancreatitis, GERD, ACS)
• Globus (ie, the sensation that an object is trapped in the throat)
• Dysphagia, which is more consistent and reproducible during investigative studies
• Regurgitation and Heartburn 

The spasms of the oesophageal muscles can lead to a feeling of food sticking, food obstructing, regurgitation, and chest pain. Symptoms may be worse with cold foods or drinks, but may improve with warm liquids.


Diagnosis:

It is reasonable to rule out ACS with ECGs/Troponins even when there is slightest of concern. Other helpful investigation are:

• Bedside ECHO (Cardiac Contractility, RWMAs, Tamponade, Dilated Right heart, PTx)
• CXR (Pneumonia/PTx, Dilated mediastinum)
• Amylase (Pancreatitis)

The diagnostic modalities of choice for DES are barium swallow and esophageal manometry. Diffuse esophageal spasm has a characteristic appearance of multiple simultaneous contractions causing a corkscrew appearance with segmentation. It is important to remember that Barium Swallow will show this typical corkscrew pattern only if done during an episode of spasm. 

Treatment:

• Calcium channel blockers and nitrates are first-line therapy. Other Treatment options: Sildenafil, Botulinum toxin, Diltiazem
• Surgical Treatment - Myotomy/ Esophagectomy

Author:

              

     Lakshay Chanana

     

     Speciality Doctor

     Northwick Park Hospital

     Department of Emergency Medicine

     England

     @EMDidactic

                    

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Posterior Reversible Encephalopathy Syndrome (PRES)

Introduction

Posterior Reversible Encephalopathy syndrome PRES (also known as reversible posterior leukoencephalopathy syndrome) is a constellation of clinical and radiological findings which presents with rapid onset headache, seizures, altered consciousness, and visual disturbances. 

It strongly associated with with renal disease, vascular and autoimmune diseases, immunosuppressive medications, and organ transplantation. Nearly 3/4 patients with PRES are hypertensive, but others may have normal or only mildly elevated blood pressure.

Pathophysiology

Failure of Cerebral Autoregualtion, endothelial injury, disruption of blood brain barrier leading to Vasogenic edema is a proposed mechanism. However, the exact pathophysiology remains unclear. 

Diagnosis 

Prompt recognition of PRES is important to prevent permanent damage due to ongoing cerebral ischemia. Having a high index of suspicion is important. PRES must be added to our routine list of differentials of Posterior Circulation Stroke, SAH, Cerebral Venous Thrombosis. Focal neurologic deficits are uncommon in PRES and Seizures are the most common presentation. 

It is reasonable to start with CT Head in the Emergency Department to rule out other CNS Catastrophes. However, MRI is a better tool to diagnose PRES. Magnetic Resonance Imaging recordings showing white matter abnormalities without infarction. Classical MRI findings of vasogenic edema involving bilateral parietal-occipital lobes. 

PRES appears to be a misnomer as the syndrome is not always reversible, and it may not be localised to either the white matter or the posterior regions of the brain. Atypical features— including hemorrhage, asymmetrical changes, isolated involvement of the frontal lobes, and cortical lesions are common.

Management

• Rapid withdrawal of the trigger (Eclampsia, Drugs)
• Prevent Seizures
• Aggressive blood pressure management 

Take Home:

• PRES should be considered in patients who present with seizures, altered consciousness, visual disturbances, or headache, particularly with acute hypertension.
• PRES has been associated with chronic and acute kidney disease, solid organ transplantation, and use of immunosuppressive drugs.
• Typical MRI findings include reversible, symmetrical, posterior subcortical vasogenic edema.
• If recognized and treated promptly, the rapid-onset symptoms and radiologic features usually fully resolve within days to weeks.

References:

• Hinchey J, Chaves C, Appignani B, et al.  A reversible posterior leukoencephalopathy syndrome. N Engl J Med. 1996;334(8):494-500
• Hobson, E. V., Craven, I., & Blank, S. C. (2012). Posterior Reversible Encephalopathy Syndrome: A Truly Treatable Neurologic Illness. Peritoneal Dialysis International : Journal of the International Society for Peritoneal Dialysis, 32(6), 590–594. http://doi.org/10.3747/pdi.2012.00152
• Fugate, J. E., Claassen, D. O., Cloft, H. J., Kallmes, D. F., Kozak, O. S., & Rabinstein, A. A. (2010). Posterior Reversible Encephalopathy Syndrome: Associated Clinical and Radiologic Findings. Mayo Clinic Proceedings, 85(5), 427–432. http://doi.org/10.4065/mcp.2009.0590
• https://radiopaedia.org/articles/posterior-reversible-encephalopathy-syndrome-1

Author:

              

     Lakshay Chanana

     

     Speciality Doctor

     Northwick Park Hospital

     Department of Emergency Medicine

     England

     @EMDidactic