Migraine / Headache
Headache – which involves a pain primarily centered in the head – is profoundly common. While Primary Care and Emergency Medicine providers often seen people who suffer from headache, the Neurologist is truly the best expert in this matter. The majority of headaches are primary headaches – suggesting that they have no underlying worrisome cause. However, it is often the fear of an undiagnosed worrisome cause – a secondary headache – that distresses patients. In this way, the Neurologist can offer expertise in both treating the headache and addressing this worry.
Features of headaches
A detailed and accurate description of the headache history is often the most important contributor to accurately diagnosing headaches. It is important to know when the headaches first started, how often they happen, how severe they are, if they respond to medications, and how often pain medications are taken. Furthermore, the precise location of the pain, the description of the pain type, and any factors that worsen the pain are helpful to know. At times, there can be other symptoms associated with the headache, such as an “aura” (sensation) that the headache is coming, sensitivity to light, sensitivity to sound, nausea, changes in vision, tear production, eye redness, or nasal drainage. The presence of these features can lead to an accurate diagnosis. It is important to see if there are any triggers for the headaches, such as certain foods, changes in weather, association with the menstrual cycle, stress, bright lights, stress, coughing, exercise, or others.
Primary headaches are those in which there is no distinct underlying cause or structural abnormality in the brain. While these can be painful and disabling if not adequately treated, these are not associated with underlying life-threatening diseases.
- Migraine headache is the most common headache type in patients who see the doctor for headache, with about 13% of people the US having migraines. It can lead to significant pain, suffering, and disability if not adequately treated. Migraines usually start in adolescence and is more common in women than men.
- Migraine headaches last hours to days. They are often located around an eye and may be associated with nausea, sensitivity to light, and sensitivity to sound.
- An “aura” may come before the headache, which is an abnormal symptom such as visual changes, changes in sensation, or language difficulties that last generally less than an hour. More rare auras may include vertigo, double vision, or weakness; in these cases, testing must be pursued to rule out other causes. Migraine with aura accounts for about 20% of migraines, while about 80% of migraines are without aura.
- If migraines occur often and cause disability, it is recommended to start a medication to prevent or “prophylaxis” against migraine headaches. In general, this would be recommended if a person has 6 more or headaches a month, but other factors can be taken into account. Options include medications in the classes of beta-blockers (such as propranolol), calcium channel blockers (such as verapamil), anti-epileptics (such as topiramate), or antidepressants (such as amitriptyline). There is a new class of medications called CRGP blockers, which attack a pro-inflammatory molecule associated with migraine. There are many medication options, and the Neurologist will take the whole person into account when partnering on this recommendation. There are also non-medication options for migraine prevention, such as Botox injections.
- Migraine “abortive” medications should be considered to stop the migraine. These are most effective when taken as soon as possible after a person knows that a migraine headache is starting. The class of medications called “triptans” (such as sumatriptan) is typically used first. These are available as pills, dissolvable tablets, nasal sprays, or injections. There is a new CRGP blocker used to aborting headaches (Ubrelvy), which is a good option for those that do not have relief from triptans or have a history of stroke or heart attack. If a headache is very severe or lasts a long time, the Neurologist may recommend you for a headache infusion, which could include steroids, Toradol, magnesium, Depakote, and / or fluids. If the migraine will not go away despite all treatment – called “status migrainosus” – an infusion of DHE can be considered.
- Tension headache is the most common headache in the general population, but these headaches are not disabling and therefore people do not often seek medical treatment for this.
- Tension headaches are dull, and the person may describe a “tightness.” In contrast with migraine, there is no significant nausea or sensitivity to light or sound.
- To stop a tension headache, typically a person can take acetaminophen or an NSAID (such as ibuprofen or naproxen). It is important to not take these medications too often, as it can lead to rebound headaches.
- If tension headaches are very frequent, the Neurologist can consider preventative medications such as amitriptyline or therapies such as biofeedback or cognitive behavioral therapy.
- Cluster headaches involve periods of weeks to months where these headaches occur at least daily, usually from 30 minutes to 2 hours. These are more common in men than women. The headaches may be triggered by alcohol or occur more often at night.
- Cluster headache pain is on one side, usually around the eye or near the temple. It is often described as searing or sharp, with rapid onset. It is often associated with drooping of the eyelid, tearing, red eye, or runny nose. This pain can be profoundly severe and disabling.
- Cluster headache abortive therapies including oxygen therapy or injections of sumatriptan
- Cluster headache prevention can be helped with the use of a calcium channel blocker called verapamil.
- There are other headache types in the same class of “trigeminal autonomic cephalgias” which have some similarities. These all center around activation of the trigeminal nerve. The duration and frequency of the headache can make some difference in diagnosis. At times, a trial of the NSAID Indomethacin can help differentiate the headache.
Secondary headaches are those in which the headache is a symptom of an underlying disease. The Neurologist will direct an appropriate workup to find and address the process that is causing the headache. This is less common than primary headaches, but there is a great importance to discover the underlying cause in a timely manner.
- Despite this being a common worry for a person who has headache, it is quite uncommon to have brain tumors cause headaches. In fact, only around 1% of people with brain tumors have headaches as the initial symptom. When these headaches do occur, they may be more long-lasting than many primary headaches, have more severe nausea, and get worse with Valsalva (increased pressure, such as “bearing down”) or with exertion. This would typically be diagnosed with MRI of the brain. Steroids can help with this headache, but the key is to address the underlying tumor.
Idiopathic intracranial hypertension (IIH)
- IIH, also commonly referred to as Pseudotumor Cerebrii, is due to increased CSF pressure, meaning that the fluid in the brain is under too high of pressure. It is not due to a brain tumor, but the headache descriptions of often quite similar to that of brain tumors. This headache is often accompanied by vision difficulties and a pulsatile ringing in the ears. IIH is more common in young women. Being overweight increases the risk of IIH. This would be diagnosed by a lumbar puncture to check opening pressure and an MRI of the brain to rule out any mass. There also may be imaging of the veins of the head, to rule out any blockage. This is typically treated with a class of medications called carbonic anhydrase inhibitors (such as topiramate) to decrease CSF production. If vision is worsened or symptoms are not controlled with medications, procedures to get rid of excessive CSF may be considered, such as a VP shunt or optic nerve sheath fenestration.
Low CSF pressure
- In contrast to IIH, a person can also have headaches due to low CSF pressure, such as from a leak. These headaches are often “orthostatic,” in that they worsen upon standing and improve upon lying down. The most common cause for a low pressure CSF headache is after a lumbar puncture to have an ongoing leak of CSF. This can be treated with a blood patch to the area. This can also happen after trauma, such as a motor vehicle accident, or even spontaneously. If someone has a shunt in place and it is “over-shunting,” it can cause this headache. MRI brain can be helpful to diagnose this, as there are typical findings such as “sagging” of the brain down and pachymeningeal enhancement. Many times rest, hydration, and caffeine use can help with this headache. Caffeine increases the production of CSF.
Giant cell arteritis / Temporal arteritis
- Giant cell arteritis (GCA) or sometimes called Temporal Arteritis (TA) is an inflammatory condition affecting the large arteries, especially in the head. This tends to cause a temporal headache with scalp tenderness. It often is accompanied by vision loss and pain or fatigue while chewing. It may also be associated with low grade fevers, fatigue, achy muscles, or weight loss. It is rarely associated with strokes. The Neurologist will check inflammatory biomarkers in the blood, such as ESR, CRP, or fibrinogen. Steroids are started as soon as this is suspected, so as to prevent vision loss. The final diagnosis requires surgical biopsy of the temporal arteries to evaluate for characteristic findings. When present, treatment is long-term. Steroids continue to be a mainstay, but there are now some steroid-sparing medications that can be used, such as tocilizumab.
- Trigeminal neuralgia (TN) is the most common recurrent facial pain. It is more common after age 40. This typically presents as episodes of brief severe electrical “shocks” or shooting pain through one side of the face, most typically the mid or lower face. The pain episodes typically last only a few seconds and can be triggered by cold air, wind, food touching the teeth, or other causes. While TN can be idiopathic or have no clear cause, it can be caused by other diseases such as multiple sclerosis, tumors, or blood vessels that push on the trigeminal nerve. The workup includes an MRI of the brain and vascular imaging. Carbamazepine is the drug of choice, but others can be considered. If medications do not help, some procedures such as microvascular decompression or gamma-knife radiofrequency ablation can be considered.
- Uncontrolled high blood pressure can result in a non-specific headache, although this may be less common than once thought.
Medication overuse headaches
- Frustratingly, overuse of well-intentioned over the counter or prescribed pain medications can in truth result in more rebound headaches. It is important for the Neurologist to know how often you are using pain medications for headache to assess for this. If this is the case, a detailed plan for decreasing the amount of pain medication used should to an improvement in the headaches.
Headaches with abrupt onset
- “Thunderclap headaches” – named for the characteristic of sudden onset to maximum severity over a few seconds – are quite worrisome
- The most concerning cause of this headache would be a subarachnoid hemorrhage, which is bleeding around the covering of the brain. This is most commonly due to a ruptured cerebral aneurysm, which is when a weakening in the wall of a blood vessel of the head bursts and leaks blood. This can result in up to 50% mortality. CT of the head and occasionally lumbar puncture can be emergently performed to evaluate for this.
- Recurrent thunderclap headaches are rare and can be due to a condition cause Reversible Cerebral Vasoconstriction Syndrome (RCVS)
There are a series of characteristics of headaches which raise some concern and need for more thorough evaluation. These are considered “red flags,” as they warrant urgent evaluation and diagnostics. These include: a thunderclap / abrupt onset, the “worst headache” of the person’s life, a change in headache pattern, a new headache in a person over the age of 50, neurological symptoms that last more than an hour, a new headache in a person who has cancer or has a weak immune system, or a headache associated with losing consciousness.
While taking a detailed history and description of the headache is often the most important step for an accurate diagnosis, the Neurologist may consider further testing. Examples including MRI of the brain, imaging of the arteries or veins of the head, or lumbar puncture for CSF analysis. If there are no “red flags” or atypical features, no testing may be warranted.
It can be profoundly helpful to keep a “headache diary” where a person records which days headaches occur, any triggers noted, how long the headaches lasted, and what headache abortive agents are used. This can help with diagnosing the headaches, following response to treatments, and identifying triggers. A paper calendar can suffice, but there are now many quality smartphone applications that can be used to even transmit data to the Neurologist, such as “Migraine Buddy.”
Resources from Migrainebuddy
Symptoms by Mayoclinic
Overview of headache
Patient education material from American Headache Society