- Written By: Josh Piemontesi, FM PGY1 – Black Diamond AB
- Peer Review By: Caleb Dusdal
Objective One
Given a patient with a new-onset headache, differentiate benign from serious pathology through history and physical examination.
before you even walk in to see the patient, you should be aware that age over 50 is more likely to be the subset of patients with high-risk headaches. So much so, that 15% of patients over the age of 65 will have a dangerous secondary headache on imaging. So keep this in mind when seeing those older patients!
We have also included the ‘Red Flag’ table with Emergent and Urgent headache features from the CFP Primary Care Headache Guideline
Now, you walk into the room with the patient. Let’s break down some key features of the history that may lead you to think this is a “high-risk headache”.
History
Onset.
The onset of the headache is crucial to delineate. I am sure you have heard of a “thunderclap headache”, which is defined by pain that reaches 7 out of 10 in intensity in less than 1 minute. You will often hear the patient say this is “the worst headache of my life” and it will come on QUICK.
This is normally associated with an intracerebral aneurysmal leak, but if the intensity further increases under exertion, that should make you wonder about a subarachnoid hemorrhage or arterial dissection.
An onset associated with Valsalva may indicate an intracranial abnormality.
If the onset is associated with a:
- seizure,
- altered LOC,
- visual disturbance, or
- focal neuro deficit
it could represent a posterior.. reversible… encephalopathy…… syndrome.
Now, if you were a bad medical student like me, you probably would have tossed that diagnosis straight out of the memory bank and to the streets, cause it’s not very high yield for your exams. But now I am an astute resident so I definitely know this condition like the back of my hand.
Basically, it’s a condition where various parts of the brain are affected by swelling, often the posterior regions of the brain like the parietal and occipital lobes, hence the name.
Pattern.
A headache with a change in usual pattern, frequency, quality, or intensity are high-risk features of a headache. So this would be the type of headache that is quite different to a patient’s usual baseline headaches. However, this may also represent an evolutional change within the same headache presentation.
Fever.
The presence of a fever with a headache raises concern for CNS infections, but keep in mind that it’s absence does not exclude the same. This is especially true in your immunocompromised patients.
Special populations.
Patients who are immunocompromised are more likely to have a high-risk headache. In addition, patients with a history of malignancy, trauma, current pregnancy, and recent pregnancy.
Malignancy can present with metastatic disease in the brain and like pregnancy, put the patient into a prothrombotic state to increase the risk of intracerebral thrombotic events.
Patients with alcohol use disorder are populations that are more likely to get serious headache pathology. They have increased risk of falls, interpersonal violence, and if with liver dysfunction can have prolonged coagulation times.
Medications.
Watch out for the following:
- chronic steroids,
- immunologic agents,
- anticoagulants, and
- antiplatelets.
The chronic steroids and immunologic agents put the patient at a higher risk for infection. The anticoagulants and antiplatelets will obviously increase hemorrhage risk.
Drugs
Cocaine and amphetamines increase the risk of intracranial hemorrhage, so watch out for those pesky drugs.
they can also cause vasospastic headaches that act like stroke
Family History.
A family history of aneurysms or sudden death in 1st degree relatives raises suspicion of the same. In addition, autosomal dominant polycystic disease increase risk of intracranial aneurysm.
Okay, so that is all the history that increases your spidey senses for a big bad headache. Now let’s dive into findings on physical exam for serious headaches.
Physical
Look out for neck stiffness with a fever and altered level of consciousness. This is the classic triad for meningitis.
Examine the head and neck for the following:
- Meningismus signs, such as Brudzinski or jolt attenuation, which would be suggestive of infection or hemorrhage.
- Otoscopy for otitis media and palpation of sinuses for sinusitis, as this can lead to extension intracranially.
- Scalp and temporal tenderness for possible temporal arteritis, AKA giant cell arteritis
- Look at the eyes for any edema, conjunctivitis, and mid-fixed dilated pupil that could suggest acute angle-closure glaucoma. Don’t forget to measure intraocular pressure to exclude!
- Ptosis on one side can suggest CN 3 compression from a mass effect intracranially.
- Check visual acuity, visual fields, examine pupils + eyelids, and check for signs of Horner’s syndrome.
This can be memorized by “PAM is horny”. The “PAM” part of the mnemonic represents ptosis, anhidrosis, and miosis, which you would find unilaterally on the affected side. - Perform a fundoscopic exam to look for any papilledema suggestive of increased intraarterial pressure.
However, you’re attending probably wouldn’t even believe you if you did see papilledema. Especially if you said you also saw pale discs with a cup to disc ratio of 0.3. I will say though, if you can get your hands on one of those panoptic ophthalmoscopes, that can make the fundoscopic exam a lot more manageable. - Last but not least, do a FULL neurologic exam. This includes mental status, cranial nerves, upper and lower extremities, pronator drift, Babinsky, gait and coordination testing.
Okay so that is generally what we would be on the lookout for to identify serious headache pathology, but there are a few benign headache presentations that are worth mentioning. These are headaches you will frequently encounter in your family practice.
Migraine Headaches.
These will affects up to 12% of the general population! Female to male ratio is about 2 to 1.
There are generally 4 phases to a migraine headache:
- The first stage is a prodrome, which often represents vegetative symptoms 24-48 hours prior.
- Following this, a lot of people will report a migraine aura, which is around 25%. 95% of the time the aura will be visual, but can be sensory, verbal, or motor.
- The third stage is the actual headache – Typically lasting between 4 to 72 hours, associated with nausea/vomiting, phonophobia and photophobia.
So I think this means they are scared of using the phone and getting pictures taken of them.
They will also get unilateral “pounding” or “throbbing” and it will be worse with activity, being quite debilitating.
- The last stage is the postdrome, where sudden head movement may cause pain in the location of the headache and the patient will be quite fatigued.
A good mnemonic to remember the presentation of migraines would be “POUND”.
- Pulsatile
- Onset 4 to 72 hours
- Unilateral
- Nausea and vomiting (associated)
- Debilitating
- Onset 4 to 72 hours
Meeting at least 4 of the above criteria would suggest the patient has a migraine.
There are ICHD-2 Diagnostic Criteria for both migraine with typical aura and migraine without aura.
It should be worth mentioning that the frequency for migraines is quite variable, with >15 headaches per month representing a chronic migraine and <15 per month being an episodic migraine.
Migraine triggers to look out for include stress, poor sleep, weather changes (Shoutout to the Calgary Chinook folk), menstruation, fasting, wine, and more!
Tension Headache:
This affects ~40% of adult population worldwide.
Generally these present as bilateral as opposed to unilateral in location with migraine, feel like a “pressure” pain without any associated symptoms.
ICHD-2 Diagnostic Criteria:
- Headache lasts 30 mins to 7 days.
- At least 2 of the following: bilateral location, pressing or tightening quality, mild or moderate intensity, not aggravated by routine physical activity such as walking or climbing stairs.
- Both of the following: no nausea or vomiting, either photophobia or phonophobia.
Further classification would be split into episodic tension headache and chronic tension headache.
Chronic would be greater than 15 episodes per month for 3 or more months. The episodic classification is further subdivided into infrequent and frequent.
Infrequent would be less than 1 episode per month and Frequent would be more than 1 episode per month, but less than 15 episodes total per month for 3 or more months.
Cluster Headaches.
The pathogenesis of cluster headaches is incompletely understood, but it is thought to be due to hypothalamic activation with secondary activation of the trigeminal-autonomic reflex, whatever that means.
Mostly it will affect males, typically between the ages of 20 to 40, but that can vary significantly.
The risk factors include family history, tobacco use, and head trauma.
The way this type of headache would present is usually with a sharp or stabbing pain in the periorbital or temporal area. The patient will be in extreme discomfort, often restless where they are pacing, rocking back and forth. It is almost always unilateral.
It lasts 15 minutes to 3 hours. Can be as often as once every other day to 8 times a day. So quite variable with the duration and freuquency, but the pattern is that the episodes will generally occur in a cluster or bout with periods of remission in-between.
Usually, the remission periods would be months in duration. Cluster headaches can also have ipsilateral autonomic symptoms, such as ptosis, miosis, lacrimation, conjunctival injection, rhinorrhea, and nasal congestion.
You can see the ICHD-2 Diagnostic Criteria for this, which splits it into episodic and chronic cluster headaches.
We will cover the treatment options for these headaches in a bit. Stay tuned!
Objective Two
Given a patient with worrisome headache suggestive of serious pathology (e.g., meningitis, tumour, temporal arteritis, subarachnoid bleed):
Do the appropriate work-up (e.g., biopsy, computed tomography [CT], lumbar puncture [LP], erythrocyte sedimentation rate).
You should get some basic laboratory testing, such as CBC, basic metabolic profile, coagulation panel, ESR, CRP, and blood cultures if you are suspecting infection.
The decision to get imaging for your patient is dependent on the presentation of the headache. If it is a typical history without features for a high-risk headache, normal neuro exam, and it responds to treatment, then further imaging likely is not needed.
In contrast, any high-risk features, discussed in objective one, should prompt you to get either a CT head non-contrast +/- with contrast or MRI head with gadolinium contrast.
Non-contrast CT is fastest and usually the most appropriate initial imaging, and it is the most sensitive for acute intracranial hemorrhage. CT head with contrast would notably be indicated if you are suspecting dissection, occlusion, and ischemia for intracranial vasculature.
There are various special circumstances where you would get an MRI w/ gadolinium contrast, but would be outside of the scope for most family physicians and you would usually be in consultation with specialists to make this decision if it were the case.
The ACEP 2008 clinical policy has some good criteria for patients that require neuroimaging. Any one of the following criteria should prompt you to get imaging:
- Headache and new abnormal findings on neuro exam (level B)
- New sudden-onset severe headache (level B)
- HIV-positive patients with a new type of headache (Level B)
- Age >50 with new headache but with normal neuro exam (level C)
Lastly, there may be circumstances where you would want to obtain a lumbar puncture. This can be both diagnostic and therapeutic in headache. Keep in mind, you would perform this with patient in lateral decubitus position if you wanted an accurate measurement of opening pressure. Seated position does not allow for an accurate measurement.
This would be vital for diagnostic clarity in some headaches, such as idiopathic intracranial hypertension.
However, if you are suspecting meningitis, an opening pressure will likely not be needed since it would not change your management.
Normal opening pressures range from
- 10 to 100 mm H2O in young children,
- 60 to 200 mm H2O in children older than 8 years old, and
- up to 250 mm H2O in obese patients.
Opening pressures greater than 250 mm H2O would be diagnostic for intracranial hypertension.
The lumbar puncture will involve acquiring four different samples of CSF in test tubes. The appearance of which should be noted, as it may be indicative of multiple pathologies.
For example. orange, pink, or yellow may suggest blood breakdown products; called xanthochromia.
Green and yellow may indicate hyperbilirubinemia, which is common in newborn CSF.
In subarachnoid hemorrhages, xanthochromia is present in more than 90 percent of patients within 12 hours of onset. So the appearance of the fluid is actually quite helpful.
When you send the fluid to the lab, you will want to get it analyzed for cell count, cell differential, microscopic examination (gram stains and more), protein level, glucose level, and culture. Sometimes you may order additional tests if suspicious for certain diagnoses, such as polymerase chain reaction.
Make the diagnosis.
High-risk features of headache and signs of intracranial pathology on imaging will lead you to various diagnoses, such as intracranial bleeds, meningitis, and temporal arteritis.
Look out for some classic distinctions on the CT head to distinguish between different types of intracranial bleeds:
- Think of subdural hemorrhage if you see a bleed that is crescent-shaped and crosses suture lines. Results from tearing of the bridging veins and often seen in alcoholics and the elderly.
- Epidural if you see a bleed that is biconvex (lens) shaped and does not cross the suture lines. Results from tearing of the arteries outside of the brain, but intracranial; most common is the middle meningeal artery.
- Subarachnoid if you see a bleed that is present within the circle of Willis, cisterns, and fissures of the brain. These can commonly occur with ruptures of berry aneurysms.
- Intracerebral if you see a bleed that is in the parenchyma and ventricles. More commonly seen in patients with chronic hypertension.
Look out for signs of severely increased ICP, such as the classic cushing’s triad which includes:
- decreased heart rate,
- irregular respirations, and
- widened pulse pressure.
Signs of meningismus and fever with high white count, positive blood cultures, or LP findings can point you towards infectious causes such as meningitis. Don’t forget the Brudzinski and kernig’s signs! (I have some data on the LR for these maneuvers to add) Temporal head pain, amaurosis fugax and elevated CRP would make you think about temporal arteritis. You will often see this in patients with current or recent polymyalgia rheumatica.
Begin timely appropriate treatment (i.e., treat before a diagnosis of temporal arteritis or meningitis is confirmed).
Intracranial bleed treatment may or may not require surgical intervention and this would be determined alongside consultation with neurosurgery. Regardless, you can aim to decrease ICP with certain measures such as hyperventilation, elevation of head of bed, and intravenous mannitol or 3% saline depending on local protocols
Meningitis you would cover with empiric antibiotics +/- antivirals. Make sure you start these AFTER blood cultures. And also ensure you are giving at meningitic doses which are often higher than what you would normally give.
Antipyretics can be used as needed for fevers.
Temporal Arteritis needs immediate treatment with steroids alongside consultation with rheumatology or ophthalmology. You will also need a temporal artery biopsy arranged, usually with plastic surgery.
- if there is any visual changes, rheumatology will often request pulse dosing following by high daily steroid dosing
Do not assume that relief of symptoms with treatment excludes serious pathology.
A lot of headache treatment involves giving analgesia to treat the pain, but with any red flag features you must get appropriate investigations such as imaging to truly rule out serious pathology.
Objective Three
Given a patient with a history of chronic and/or relapsing headache (e.g., tension, migraine, cluster, narcotic-induced, medication- induced), treat appropriately, and avoid narcotic, barbiturate dependence.
Tension headaches:
- Acutely, NSAIDs +/- acetaminophen will do the trick
- For prophylaxis, 1st line are TCAs, such as amitriptyline and nortriptyline. 2nd line would be SSRIs or SNRIs, such as mirtazapine and venlafaxine.
Acute abortive therapy includes ibuprofen, naproxen or acetaminophen as first line.
Second line includes:
- Triptans
- antiemetics such as domperidone or metoclopramide
Third line is naproxen in combination with a triptan
Prophylactic options for migraine headaches should be discussed if:
- acute meds are not effective and more than 3 days per month of migraines, or
- migraines happening more than 8 days in a month, or
- migraines are disabling despite acute medications
Options here are many, table included, but briefly:
- First line includes:
- Propranolol
- Metoprolol
- Nadolol
- Amitriptyline
- Nortriptyline
- Second line gets into more vague options:
- Topiramate
- Candesartan (who knew?)
- Gabapentin – the drug looking for a home
Cluster headaches:
- Acutely, you have a few options here for aborting a cluster headache.
- Firstly, try 100% Oxygen. At least 12 L/min through a non-rebreather mask. This is especially a good first option since giving oxygen has minimal side effects for the patient.
- If the oxygen doesn’t work, you can try Sumatriptan 6mg subcutaneously. Intranasal triptans are an option too.
- If all of the above don’t work, you can then try intranasal lidocaine, oral ergotamine, or IV dihydroergotamine.
- Prophylaxis would usually be indicated for a chronic pattern of cluster headaches. These would look like a remission interval less than 3 months or long-lasting duration that is greater than 2 months. Verapamil is the first line agent that would be used for prophylaxis. Patients with less frequent attacks can also be put on oral prednisone 50-100mg once daily for 5 days total, followed by a taper with dose reduction of 10mg. Other options include lithium and topiramate.
Narcotic-induced headaches:
- Educate the patient about their headaches likely being related to their narcotic medication use. Easier said than done, but aim to work with the patient to create a plan to discontinue the medication, but doing so gradually to reduce withdrawal symptoms.
- Use triptan and ergots during withdrawal for symptomatic relief of their headaches. Consider prophylactic medication rather than acute medications.
Medication-induced:
- As above, but can discontinue medication abruptly.
Objective Four
In a patient with a history of suspected subarachnoid bleed and a negative CT scan, do a lumbar puncture.
Some patients with subarachnoid hemorrhage will present with isolated headache, a normal exam, and no findings on the CT head.
We talked about lumbar punctures earlier, but don’t forget to include the opening pressure, cell counts (with that being WBCs and RBCs) and visual inspection for xanthochromia.
The classic findings of SAH include increased opening pressure, elevated RBC count throughout tubes 1 to 4, and xanthochromia.