Headache after headache
A 47-year-old woman with a history of infrequent headaches presented to the neurology clinic for a nearly daily headache over the past 3 months. But never fear! Dr. Mike Rubenstein is there to help. She tells Mike it is an excruciating, pressure-type pain just over her right eye, which is usually where she gets her headaches, without any associated visual disturbances, nausea or vomiting. She does notice it causes her to tear up and sometimes her eyelid becomes droopy. The headache comes on suddenly without any obvious trigger, and it usually resolves after 10 or 15 minutes. But she gets them several times a day. Lately, they’ve been coming on every day or every other day in waves of 3 to 5 at a time, for which she has scheduled an appointment in your neurology clinic. She’s tried Tylenol and ibuprofen without much relief. Her PCP gave her a prescription for Fioricet, which helped initially but now it seems to barely touch the pain and she’s taking it practically every day at the onset of symptoms. What kind of headache is this, and what will you do about it Mike?
In this week’s clinical case, Dr. Rubenstein walks us through the evaluation of a patient with headaches that have autonomic features. This patient’s headache has many of the qualities of a trigeminal autonomic cephalgia. This includes paroxysmal hemicrania, cluster headache, and SUNCT or SUNA syndrome. SUNCT meaning short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing and SUNA meaning short-lasting unilateral neuralgiform headache attacks with cranial autonomic features. These headaches are characterized by mostly unilateral symptoms, brief duration, with autonomic involvement (tearing and possible oculosympathetic disturbance). But could our patient be having another type of headache?
Mike argues that she fits the wrong demographic for a chronic cluster headache–which is a variant of your episodic cluster headache. Chronic cluster headache attacks occur at least once every other day, but could be several times a day as in our patient, and this persists for over one year. Patients with chronic cluster headache can still be classified as having chronic cluster headache if they have remission lasting less than 1 month, according to the International Headache Classification Disorders. For our patient, it is unclear if she meets these criteria yet, or like Mike says, she is evolving into a chronic cluster headache. But this is just a matter of semantics. Technically, having 3 or more months of headache that occurs 15 or more days a month qualifies the patient for a diagnosis of chronic daily headache—another relatively non-specific diagnosis. But these headaches don’t have to occur every day to be chronic daily headaches, they just have to take place half the days per month. And when you see patients who meet this diagnosis, it should make you wonder what the primary inciting factor or factors are for the persistent headache. Over 90% of chronic daily headaches began out of a primary headache disorder. For our patient with associated autonomic symptoms, it seems to be driven by a potential underlying autonomic trigeminal cephalgia. But is her headache being aggravated by something else? For instance, one physician reported chronic headaches attributed to binge-reading Harry Potter books. The Lord of the Rings is not the same, but it’s an awesome series as well.
Medication overuse headache, as we reviewed way back in episode 12, is more commonly seen in migraine sufferers or patients with tension-type headaches who unfortunately get caught up in a cycle of trying to take medications to break their frequent headaches. And even taking something like Tylenol more than 3 days a week is enough to throw you into a downward spiral. Typically headaches caused by medication overuse are non-specific and don’t involve any autonomic symptoms, but they can seem like somewhat milder migraines. The headaches in medication overuse headache are almost continuous, lasting all day, occasionally relieved by more NSAIDs, don’t interrupt their sleep but can wake them from sleep. And often the prior headache can be superimposed on top of it. So a migraine or cluster headache can interrupt this chronic head pain.
Red flags to be on the lookout for include:
- New headaches that wake you from sleep
- A sudden onset worst headache of life
- Headache with a new focal neurologic deficit
- Headache with positional changes either standing up or lying down
- New nausea and vomiting
- Any deviation in the characteristic of a prior headache
These features might buy the patient an MRI in the appropriate clinical circumstance…But in this case, our patient had none of those findings. She did have some autonomic symptoms, which raised Mike’s suspicion for a trigeminal autonomic cephalgia. And given the laterality to it, it more accurately fits with paroxysmal hemicrania (about 85-97% of patients with paroxysmal hemicranias have strictly unilateral symptoms).
If it were not paroxysmal hemicrania, it might be a cluster headache. Treatment of an acute cluster attack involves supplemental oxygen, usually in the form of a 100% non-rebreather with a flow rate of 7 L/min. This is probably the only therapy we have in neurology which comes with zero side effects. 60% of patients with cluster headaches respond to oxygen. Triptans like sumatriptan are also effective at breaking an attack of cluster headaches in 75% of patients, but for someone like this who may be likely to abuse an oral agent, you might avoid prescribing it for now. Other potential therapies for cluster involve the intranasal injection of lidocaine and the subcutaneous injection of 100ug of octreotide. More importantly, patients often benefit from preventative therapies like verapamil and topiramate.
For the sake of this case, let’s say the patient has a history of paroxysmal hemicrania that has evolved into hemicrania continua. Current experts still don’t have a perfect understanding of the pathophysiology that underlies HC, but there are two features everyone agrees on: strictly unilateral headache symptoms, and responsiveness to indomethacin (which happens to be the treatment). The patient should have the head pain on only one side, although 3-15% of patients may report alternating sides in paroxysmal hemicranias. And when it does switch sides like that, you should think of migraine, cluster, or tension-type headache as well. The episodic version of HC is paroxysmal hemicrania, and these symptoms typically last from 2 to 30 minutes—most are about 15 minutes, as in our patient’s. And they will recur several times throughout the day. During acute attacks, patients often report feeling restless (80% in some studies). The ICHD-3 criteria also require at least one autonomic symptom, although this doesn’t have to be present in every headache attack. Lacrimation is the most common reported symptom, but conjunctival injection, rhinorrhea, facial flushing, ptosis, and unilateral facial sweating can occur. In contrast, features not seen in HC would be things like a known aura, clear triggers, or associated vomiting. They are not more frequent at night nor should they necessarily wake a person from sleep, as you see in cluster headaches and intracranial pathology.
Lastly, there are some vitamins and supplements which some people benefit from. Butterbur has the highest level of evidence supporting its use and is recommended by the American Academy of Neurology (Level A evidence). Feverfew, magnesium, and riboflavin are also useful adjuncts in the prevention of recurrent migraine, with a level of evidence B. Treatments considered possibly effective, level of evidence C, include estrogen, CoQ10, cyproheptadine, and mefenamic acid. So, these are worth thinking about if you’re getting a headache from reading too many BrainWaves blogs…
BrainWaves audio podcasts and online content are intended for medical education purposes only and should not be used for routine clinical decision making. Chronic daily headaches suck. Talk to your doctor about how you should manage your symptoms. The featured image is from https://www.flickr.com/photos/zazasvq/8128109002/ under a CC license.
- Dodick DW. Clinical practice. Chronic daily headache. The New England journal of medicine. 2006;354:158-65.
- Halker RB, Hastriter EV and Dodick DW. Chronic daily headache: an evidence-based and systematic approach to a challenging problem. Neurology. 2011;76:S37-43.
- Bigal ME and Lipton RB. The differential diagnosis of chronic daily headaches: an algorithm-based approach. J Headache Pain. 2007;8:263-72.
- Silberstein SD and Lipton RB. Chronic daily headache. Curr Opin Neurol. 2000;13:277-83.
- Pareja JA, Antonaci F and Vincent M. The hemicrania continua diagnosis. Cephalalgia. 2001;21:940-6.
- Prakash S and Patell R. Paroxysmal hemicrania: an update. Curr Pain Headache Rep. 2014;18:407.
- Goadsby PJ. Trigeminal autonomic cephalalgias. Continuum (Minneap Minn). 2012;18:883-95.