Chronic Headache Case File

Chronic Headache Case File

Eugene C. Toy, MD, Ericka Simpson, MD, Pedro Mancias, MD, Erin E. Furr-Stimming, MD

CASE 19

A 38-year-old woman has a history of headaches for at least 10 years. The headaches now occur daily and are of mild to moderate intensity. Usually, the pain is located in the temples and often radiates to the neck. The patient also reports a different headache approximately three times per month that is located over one eye and is often associated with nausea, vomiting, photophobia, and phonophobia. These headaches are often worse during her menstrual cycle. The patient has a prior history of episodic migraine beginning in her twenties. These initially occurred one to three times per year, but then progressed to as much as one to three times per month. The patient has tried many over-the-counter (OTC) medications for her headaches and has used acetaminophen (Tylenol), aspirin, caffeine (Excedrin Migraine), ibuprofen, and naproxen sodium with regularity. She is currently taking three acetaminophen every 4 hours and still gets a headache. She notes that when these headaches began, 2 acetaminophen (Tylenol) usually relieves the headache, but now even the 18 acetaminophen (Tylenol) per day do not impact her headache. Her general examination is within normal limits. Her neurologic examination does not reveal neck stiffness or muscle rigidity, abnormal reflexes, weakness, or sensory changes. There is bilateral tenderness in the occipital nerve exit zone.

▶ What is the most likely diagnosis?

▶ What is the most likely next diagnostic step?

▶ What is the likely next step in therapy?

ANSWERS TO CASE 19:

Chronic Headache                                                       

Summary: A 38-year-old woman has a 10-year history of daily headaches, located in the temples radiating to the neck. The patient also reports a different headache approximately three times per month, over one eye with nausea, vomiting, photophobia, and phonophobia, exacerbated by menses. Numerous OTC medications, including large acetaminophen doses, are unhelpful. Her general and neurologic examinations are normal except for occipital tenderness.

• Most likely diagnosis: (1) Chronic daily headache with analgesic rebound, (2) tension headache with occipital neuralgia, (3) episodic migraines
• Next diagnostic step: Fundoscopic examination
• Next step in therapy: Taper acetaminophen usage and consider prophylactic treatment, including occipital nerve block

ANALYSIS

Objectives

1. Recognize chronic daily headache and be able to distinguish it from migraine and other causes of headache.
2. Know what treatments are available for chronic daily headache.
3. Know what workup is necessary for patients with chronic daily headache.

Considerations

When evaluating a patient for headache, the clinical history is of critical importance. The nature (type of pain and associated symptoms or triggers), severity, and duration of the headache are important in determining what type of headache it is and how to manage it. It is not uncommon for a single patient to experience multiple types of headaches. She reports pain around her temples and neck which occurs daily; these symptoms seem to be consistent with a tension-type of headache with associated occipital neuralgia. She also has a frequently recurring headache that is unilateral and associated with nausea and vomiting and sensitivity to light and sound, which is suggestive of an episodic migraine headache. Although she has increased frequency, the character of her headaches has not changed. Long-term, uncontrolled headaches can evolve into chronic daily headaches, particularly in the setting of medication overuse, leading to rebound pain. The history and relatively benign physical examination suggest that her headaches are unlikely to be due to other etiologies such as tumor, infection, or trauma. Nevertheless, if the patient has not had imaging of the head in the past, a magnetic resonance imaging (MRI) or computed tomography (CT) scan would be indicated. Once chronic daily headache is confirmed and other etiologies such as infection and brain tumor are ruled out, tapering of the acetaminophen, occipital nerve block, and prophylactic therapy would be the next step.

APPROACH TO:

Chronic Headache                                        

DEFINITIONS

CHRONIC DAILY HEADACHE: A daily or near-daily headache lasting at least 4 hours per day and occurring more than 15 days per month. It may be due to transformed migraine, chronic tension-type headaches, hemicrania continua, or new daily persistent headache. Medication overuse is often a contributing factor.

TRANSFORMED MIGRAINE: A form of chronic daily headache developing gradually over time. The patient should have a history of episodic migraine escalating in frequency over time, and will still have concurrent attacks of acute migraine occur intermittently.

CHRONIC MIGRAINE: Headache occurring more than 15 days per month for

3 or more months in a patient with history of migraine. On at least 8 days per month, the headache should meet migraine criteria. Other days may consist of other or less severe headaches. Transformed migraine may meet chronic migraine criteria. Secondary causes must be excluded, including medication overuse headache (although medication overuse may contribute to the syndrome).

CLINICAL APPROACH

The clinical entity of chronic daily headache encompasses several headache syndromes. It is classified into chronic/transformed migraine headache, chronic tension-type headache, new daily persistent headache, and hemicrania continua. All of these entities can be subdivided based on whether they occur with or without medication overuse.

Chronic/Transformed Migraine Headache

Chronic/transformed migraine is most often seen in women who have had a history of intermittent migraines, which usually begin in their teens or twenties. The headaches become much more frequent over the years, and as the frequency increases, the associated photophobia, phonophobia, nausea, and vomiting decreases. This transformation eventually results in the patient experiencing daily headaches resembling tension-type headaches without any associated migraine symptoms. Even as these headaches become more chronic, many patients still have intermittent episodic migraines, and these breakthrough, random migraine headaches can be associated with nausea, vomiting, photophobia, and phonophobia. A significant number of chronic migraine patients suffer from underlying depression and anxiety. The defining characteristic of chronic migraine is the transformation from episodic migraines to chronic daily headaches with superimposed episodes of acute migraines. Thus, chronic migraine has the following characteristics: daily or almost daily headache more than or equal to 15 days per month lasting at least 4 hours a day. There is usually a history of episodic migraine during this chronic phase (at least eight per month per the international guidelines) with associated symptoms (unilateral, throbbing with nausea/vomiting, photophobia, and phonophobia). This transformation to a more chronic picture usually takes place over a 3- to 6-month period of time.

Chronic Tension-Type Headache

Patients with a history of episodic tension headaches can progress to a more chronic tension-type headache. There is usually the absence of the typical features of migraine (nausea, vomiting, photophobia, phonophobia, etc). The patient usually is affected more than 15 days per month and for usually more than 4 hours per day. The pain is usually bilateral in the temporal region, described as a nonpulsatile, pressing or tightening, of mild to moderate severity, and not exacerbated by physical activity. It is often in a hatband distribution. There can be associated pain and tenderness in the occipital area as well as in the posterior strap muscles of the neck. These patients can also have an occasional breakthrough migraine headache, but the dominant headache is clearly the more frequent bitemporal headache. Essentially, the chronic tension-type headache is defined by what it is not—that is, migraine.

Hemicrania Continua

Hemicrania continua is a headache syndrome characterized by a constant, unremitting unilateral headache of mild to moderate intensity at baseline, with superimposed episodes of severe sharp and stabbing pain occurring over one side of the head. It is considered one of the “trigeminal autonomic cephalalgias” along with cluster headaches and short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT). Exacerbations of more severe pain occurs almost daily and lasts anywhere from 30 minutes to 3 days and is often accompanied by ipsilateral autonomic symptoms (ptosis, conjuntival injection, lacrimation, nasal congestion, rhinorrhea), migraine-like features (nausea/vomiting, unilateral photophobia, etc), brief stabbing pain, and a sensation of a foreign body in the eye ipsilateral to the headache. It may be remitting, in which headaches are interrupted by remission periods of 1 day or more, but most cases are unremitting. It is often misdiagnosed as migraine or cluster headache due to patients reporting either only the chronic unilateral baseline headache with migraine-like features or the flare-ups with autonomic symptoms. Its absolute response to indomethacin treatment is a defining feature.

New Daily Persistent Headache

New daily persistent headache is the abrupt development of a daily headache over a short period of time, usually less than 3 days, in a patient with no prior history of any headache syndrome. The patient recalling the date or circumstances during which the headache began is pathognomonic. There can be a precipitating event, often an antecedent viral illness. Once the headache has begun, the average frequency is more than 15 days per month of headache, and the duration is more than 4 hours a day if untreated. It is a primary headache syndrome, but it is also a diagnosis of exclusion after first ruling out secondary causes of acute headache.

Females predominate by about 2:1, and peak onset is in young adults. The pain is usually bilateral and moderate. The quality may be variable, with dull aching or throbbing reported by different patients. Although the International Classification of Headache Disorders (ICHD) II diagnostic criteria classifies this headache as having features resembling tension-type headaches, the patient may also experience symptoms characteristic of migraines. Thus, the type of pain should not be used in making the diagnosis, but rather the nature of abrupt onset of chronic headaches should be used.

Occipital Neuralgia

Occipital neuralgia is a headache in which the greater occipital nerves are irritated and inflamed. This causes intense pain radiating from the exit point to the occipital nerve, behind the ear up to the temples or behind the eyes, in the distribution of the occipital nerve. The pain may be bilateral or unilateral in location and occurs as paroxysmal attacks of severe shooting/stabbing or “shock-like” pain that lasts seconds to minutes. There may be scalp tenderness over the affected nerve branches and trigger points at the exit point of the occipital nerve. The pain is often associated with dysesthesia or allodynia with scalp or hair stimulation. It tends to occur with tension headaches because surrounding muscle tension in the neck and scalp can irritate the nerves. It may occasionally trigger migraine at times. Occipital nerve block with steroid and local anesthetic is both diagnostic and therapeutic as it reduces the nerve inflammation. It can also be treated conservatively with physical therapy, low-dose antiepileptic drugs or tricyclic antidepressants (TCAs). Pulse radiofrequency or occipital nerve stimulation can be used for refractory cases.

Medication Overuse Headache

Medication overuse headache occurs in the setting of chronic, excessive analgesic or headache medication use usually over 3 or more months in a patient with a preexisting headache disorder. Excessive analgesic or headache medication use is defined as >10 days per month of triptans, ergots, combination analgesics or opioids; >15 days per month of nonsteroidal anti-inflammatory drugs (NSAIDs); or more than one medication for headache for >10 days per month. The headaches typically occur more than 15 days per month. The history is notable for worsening headaches and increased headache frequency despite medication, and headaches often result in escalating doses of medication without relief. Abrupt discontinuation of the medication results in severe headache. If the drug is an opioid, benzodiazepine, or barbiturate, there is significant risk of additional physical withdrawal symptoms that may be life threatening. Medication overuse headache requires weaning of the overused agent along with concurrent management of the withdrawal symptoms and the original headache syndrome. Some patients may require admission to the hospital for weaning therapy, but many can be slowly weaned in the outpatient setting.

Evaluation

It is important to consider that all of the chronic daily headache types may be exacerbated by analgesic rebound, and a vigorous attempt should be made to get patients off regular use of OTC analgesics. Most patients with chronic daily headache have been seen by multiple physicians because of the chronicity of the headache. Imaging studies have typically been performed in the past and are normal; if unavailable, then an MRI/magnetic resonance angiography (MRA) should be done to evaluate for ischemia, signs of abnormal intracranial pressure, or a space-occupying lesion. Serum chemistries, complete blood count (CBC) with differential, thyroid panel, and a sedimentation rate should be drawn. A lumbar puncture (LP) after MRI clearance should be considered in those patients with headache of acute origin over a short period of time to rule out an infectious or inflammatory cause(s).

Treatment

About 30% of patients with chronic daily headaches report significant improvement with therapy, but most patients gain some improvement. There are both medical and nonmedical treatments available, and both should be pursued, especially if there is a significant neck component to these headaches.

The nonmedical treatment of chronic daily headache can include biofeedback and relaxation therapy, stress management, psychological interventions (cognitive behavioral therapy [CBT], individual/family counseling), and lifestyle changes (dietary modifications, sleep hygiene, daily exercise program). Keeping a headache diary can be helpful in identifying triggers as well as recognizing patterns of frequency and intensity of attacks, which can help guide treatment, especially with medication overuse. Many patients can benefit from physical therapy by a head and neck rehabilitation specialist. Massage therapy has also been shown to be helpful in certain patients.

As noted previously, the first intervention in a plan of medical therapy for chronic daily headache is removal of any overused medications, which can include NSAIDs, opioids, triptans, and ergots. As the overused medication is weaned, the patient must be bridged with alternative analgesics while prophylactic therapy is initiated and titrated to therapeutic levels. The type of medication being weaned determines the rate of weaning, the medication used as a bridge, and if it is performed as an outpatient or inpatient. Rapid weaning protocols should include alternative analgesic options such as NSAIDs, steroids, triptans, or ergots. Preventative therapy for the chronic daily headache should begin at the same time and is similar to that used for migraine management. Studies using OnabotulinumtoxinA injections or topiramate show the strongest evidence for preventative therapy, but other medications may also be used, including other antiepileptics (ie, valproate) and beta-blockers (ie, propranolol, metoprolol). The only Food and Drug Administration (FDA)-approved treatment for chronic migraines is OnabotulinumtoxinA injection. Acute medication is also recommended in addition to preventative therapy to substitute for the overused medication for patients with chronic migraines. However, the use of acute medication should be limited to 2 or fewer days per week. The most useful classes of medication for acute treatment are triptans, NSAIDs, or ergots, and the choice of acute medication is based on the class of the overused medication. In patients with tenderness in the occipital nerve exit zone, consider greater occipital nerve block with an injection of corticosteroid and anesthetic. Injection therapies can provide fast-acting therapy until prophylactic therapy takes effect.

CASE CORRELATION

• See also Case 12 (Subarachnoid Hemorrhage) and Case 18 (Migraine Headache)

COMPREHENSION QUESTIONS

19.1 A 33-year-old woman is noted to have chronic daily headaches. The workup has been negative. Which of the following is an important principle in the management of this disorder?

A. Maintain analgesic dose and start antiepileptic therapy.

B. Increase the analgesic dose while initiating biofeedback therapy.

C. Lower the analgesic dose while beginning other therapy.

D. Reassure the patient, and refer to psychiatrist.

19.2 A 24-year-old woman presents with the complaint of severe headaches. The pain is typically unilateral and throbbing, and is associated with nausea and photophobia. In the past, she had headaches one or twice a month, but these have been increasing in frequency to almost daily in the last few months. OTC medications no longer provide relief. Which headache syndrome is this patient describing?

A. Occipital neuralgia

B. Transformed migraine

C. Chronic tension-type headache

D. New daily persistent headache

19.3 A 55-year-old man comes into the physician’s office with a 2-year history of daily headaches. The sharp, stabbing pain peaks in attacks lasting about 20 minutes about five times per day. It occurs on the right side of his head, and is associated with photophobia and nausea. What is the best therapy for this patient?

A. Indomethacin

B. Oxygen

C. Topiramate

D. Sumatriptan

ANSWERS

19.1 C. Analgesic overuse often contributes to headaches or migraines becoming chronic. Therefore, the first intervention in a plan of medical therapy for chronic daily headache is removal of any OTC medications, which can include either acetaminophen or aspirin. At the same time, bridge the patient with an alternative analgesic and also begin preventative therapy for the chronic headache.

19.2 B. This patient has a transformed migraine that is likely evolving into a chronic daily headache of the migraine type. Migraine headaches are commonly unilateral and have a throbbing quality. These can transform from episodic to chronic over a period of several months. Other types of headaches can contribute to the syndrome of chronic daily headache, including occipital neuralgia and medication overuse. However, not enough information was given about this patient to implicate other etiologies. A new daily persistent headache would not apply to this patient due to her history of headaches similar to this one.

19.3 A. This patient is most likely suffering from hemicrania continua. Response to indomethacin is both therapeutic and diagnostic. Oxygen therapy has not shown benefit in these patients, but it is helpful for patients with acute cluster headaches. Sumatriptan is only rarely effective for hemicrania, and it would not be a first-line agent like it may be for migraine or cluster headache abortive therapy. Topiramate is used to prevent migraine headaches.

    CLINICAL PEARLS    

▶ Transformed migraine is migraine headache that transforms into daily, less severe headaches punctuated by severe and debilitating migraine attacks. ▶ Overuse of pain relievers is a major contributing factor in transformed migraines. ▶ Tension-type headaches, associated with a band-like constant bilateral pressure and pain from the forehead to the temples and often the neck, are the most common form of headache. ▶ New daily headaches that begin abruptly in a patient with no prior headache history require more extensive evaluation to rule out secondary causes of headache such as infection, inflammation, intracranial pressure abnormalities, or mass lesions.

REFERENCES

Bigal ME, Sheftall FD. Chronic daily headaches and its subtypes. Continuum (Minneap Minn). 2006;12(6):133-152. 

Derman H. Current Neurology. St. Louis, MO: Mosby; 1994:179. 

Dougherty C. Occipital neuralgia. Curr Pain Headache Reps. 2014;18:411. 

Goadsby PJ. Trigeminal autonomic cephalalgias. Continuum (Minneap Minn). 2012;18(4):883-895. 

Kaniecki RG. Tension-type headache. Continuum (Minneap Minn). 2012;18(4):823-834. 

Lipton RB. Risk factors for and management of medication-overuse Headache. Continuum (Minneap Minn). 2015;21(4):1118-1131. 

Newman LC. Trigeminal autonomic cephalalgias. Continuum (Minneap Minn). 2015;21(4):1041-1057. 

Saper JR, Silberstein SD, Gordon CD, et al. Handbook of Headache Management, a Practical Guide to Diagnosis and Treatment of Head, Neck, and Facial Pain. Baltimore, MD: Williams & Wilkins; 1993. 

Silberstein SD, Lipton RB, Goadsby PJ. Headache in Clinical Practice. London, UK: Martin Dunitz; 2002. 

Tepper SJ. Medication-overuse headache. Continuum (Minneap Minn). 2012;18(4):807-822. 

Tyagi A. New daily persistent headache. Ann Indian Acad Neurol. 2012;15(suppl 1):S62-S65.

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