Chapter 6: Headaches

Neeraj Kumar, MD; Scott C. Litin, MD, MACP

1. CASE HISTORIES
   

   Case 6-1. A 24-year-old woman presents with a 1-year history of episodic headaches. The average frequency is 2 to 3 episodes in a month. The headaches are described as being moderately severe, last for most of the day, and often cause her to miss work. The headaches are generally right-sided, although sometimes both sides can be affected, and they are often associated with light and noise sensitivity. Her mother has similar headaches, but they decreased in later years. The patient's neurologic examination is unremarkable.

   1. What type of headache is this?
   2. What type of imaging study is indicated?

   Case 6-2. A 36-year-old woman presents with increasing frequency and duration of headaches over the past year. Since her teenage years she had been having throbbing, unilateral headaches that were often related to her menstrual cycle. Subsequently the relationship to her menstrual cycle was less consistent. Those headaches did respond to naproxen or sumatriptan tablets. Over the past six years her headaches have increased in frequency and duration, and for the past year the patient has had a bilateral, dull headache almost every day. There are periodic exacerbations during which she has nausea and light sensitivity. The benefit provided by sumatriptan has decreased, and she currently takes 12 tablets of sumatriptan 100 mg in a week. The benefit provided by acetaminophen with codeine and a combination preparation containing aspirin, butalbital, and caffeine is short-lived, and she takes four to six tablets of either medication daily. She has been sleeping poorly and has been unable to take care of her responsibilities at work and home. Her other medications include propranolol long-acting 120 mg a day. These medications were introduced for headache prevention five years ago and were initially felt to be effective.

   1. What type of headache is this?

2. DISORDERS
   

   Headache is one of the most common symptoms encountered in primary care. Primary (idiopathic) headaches commonly include migraines, tension-type headaches, and cluster headache. Secondary headaches are those caused by underlying disease. (See Table 7-1 for a list of primary and secondary types of headache.) The most common type of headache in some epidemiologic studies is tension-type headache, although patients with this disorder rarely present to the physician's office with this as a chief complaint because these headaches rarely have a major impact on the patient's life. When a patient comes to the physician's office with headache as a chief complaint, the diagnosis is usually going to be migraine. The goal of the initial visit is to exclude a serious underlying disease (e.g., secondary headache: tumor, infection, etc.) as the cause of the headache and to accurately diagnose the type of primary headache. The history and examination can provide a clue to a possible underlying secondary cause and indicate whether "red flags" are found (see Section VI: Red Flags and When to Refer). Red flags will help direct subsequent workup and referral.

   Table 6-1 Primary and Secondary Headaches

   Primary Headache	Secondary Headache
   Migraine Status migrainosus Tension headache Cluster headache	Subarachnoid hemorrhage Subdural hematoma Tumor Benign intracranial hypertension Giant cell arteritis Arnold-Chiari malformation Meningitis Post-lumbar puncture headache Medication overuse headache

   The classic description of migraine is that of an episodic, moderate to severe, unilateral, throbbing headache, which increases with physical activity and may be accompanied by nausea, vomiting, photophobia, or phonophobia. Some patients may have reversible symptoms indicating focal brain dysfunction. This constitutes an aura, which may precede or accompany the headache. The aura may be preceded by a prodromal phase. The migraine prodrome occurs 24 to 72 hours prior to headache onset. Common premonitory symptoms include lethargy, depression, euphoria, yawning, and food cravings. However, the majority of migraine patients do not have an aura preceding their headaches, so clinicians who rely on the presence of aura to diagnose migraine will miss many cases. The migraine attack generally lasts several hours and often interferes with the patient's ability to work during the attack. This is an important distinguishing feature from tension-type headache, which is milder and rarely interferes with work or activities. Very prolonged migraine attacks or a series of attacks with minimal relief between them is called status migrainosus. The term menstrual migraine is used to describe migraine headaches that occur before or during the menses. Migraine equivalents (migraine aura without headache) describes visual, sensory, motor, or psychic disturbance characteristic of a migraine aura that is not followed by headache. A rare subtype of migraine with aura is hemiplegic migraine. In ophthalmoplegic migraine the headache is accompanied by paresis of the extraocular muscles. Retinal migraine is a rare type of migraine in which the headache is preceded or accompanied by monocular visual disturbance. Basilar migraine is characterized by the presence of symptoms and signs that suggest brainstem involvement such as vertigo or depressed level of consciousness. Chronic migraine is a term used when migraine occurs for 15 or more days per month over three months or longer. The most common cause of migraine-like headaches occurring on 15 or more days per month is medication overuse and is called medication overuse (rebound) headache.

   Tension-type or "muscle contraction" headaches can be considered as "featureless headaches." They can begin at any age. The headache is generally bilateral and is described as a sense of pressure or a tight band around the head. In general tension-type headaches are not associated with nausea, vomiting, photophobia, or phonophobia. They are usually mild, and patients rarely present with tension-type headaches as a chief complaint. Tension-type headaches have probably been overdiagnosed in the past.

   Cluster headache is predominantly a disorder of men and is characterized by a circadian and circannual periodicity. The attacks occur in groups and are separated by periods of remission. The attacks are strictly unilateral, often retro-orbital and temporal, and nearly always have ipsilateral autonomic signs such as conjunctival injection, lacrimation, nasal congestion, rhinorrhea, facial sweating, miosis, ptosis, or eyelid edema. Cluster headache frequently occurs at night, awakening the patient from sleep. In contrast to migraine, patients with cluster headache are restless and pace rather than lie down. The approximate duration of a cluster headache is about an hour, and typically one to three attacks may occur in a day.

   Other less common headache types include ice-pick headaches, characterized by sudden lancinating pain, and can be sensitive to treatment with indomethacin. Other indomethacin-sensitive conditions might include coital headache or exertional headache.

3. HISTORY
   

   Some patients may have more than one type of headache, and each headache type may need to be separately categorized. It is often best to ask patients to describe their worst headache type first. A headache history should include a detailed description of the pain. Questions (Table 6-2) should focus on the type, site, and severity of the headache and a detailed medication history. It is important to address lifestyle issues, including sleep and eating habits, stress, and caffeine use. The presence or absence of autonomic symptoms (lacrimation, rhinorrhea) is important, as is the overall psychosocial effect of the headaches on the patient. Analgesic use should be specifically discussed, as accelerating headaches can sometimes be related to overuse of these agents.

   Table 6-2 Historical Questions for Primary Headache Types

   Primary Headache Type	Ask the Patient
   Migraine	Intermittent, unilateral, throbbing, nausea, vomiting, light sensitivity, sound sensitivity; patient likes to stay still; sleep helps; precipitated by certain foods, menses, weather change, physical activity, neurologic symptoms that precede the headache; interferes with activity
   Tension headache	Bilateral tight band around head, mild not interfering with work
   Cluster headache	Groups of headaches followed by headache-free interval, unilateral retro-orbital, usually at night, ipsilateral lacrimation and rhinorrhea; patient likes to pace

   Important historical questions to rule out secondary headaches are listed in Table 6-3. Lightening-like onset of a severe headache may suggest a vascular cause with common considerations being subarachnoid hemorrhage, bleeding into a mass, or hemorrhage from an arteriovenous malformation. Patients with subarachnoid hemorrhage may indicate that the headache is the worst headache of their life, and the sudden onset of the headache that peaks rapidly has been referred to as a "thunder clap" headache. The possibility of an enlarging mass such as a tumor or subdural hematoma needs consideration. Metastasis, infectious or neoplastic meningitis, or brain abscess are likely considerations in recent headache onset in a patient with a malignancy or immunocompromised state. The presence of a systemic illness or meningeal signs with fever should prompt a search for infectious etiologies. The presence of focal symptoms or signs needs evaluation for an underlying structural cause, such as a tumor or arteriovenous malformation.

   Table 6-3 Historical Questions Suggestive of a Secondary Headache Type

   Secondary Headache Type	Ask the Patient
   Subarachnoid hemorrhage	Lightening-like onset, worst headache of life
   Subdural hematoma	Head trauma, weakness/numbness on one side of body
   Tumor	Gradually progressive over days to weeks, weakness/numbness on one side of body
   Benign intracranial hypertension	Transient visual obscurations
   Giant cell arteritis	Transient visual obscurations, constant temporal headache, jaw claudication, polymyalgia rheumatica
   Arnold-Chiari malformation	Valsalva-induced headache
   Meningitis	Stiff neck, fever
   Post-lumbar puncture headache	Absent when lying down, comes on quickly when upright
   Medication overuse headache	Daily or every-other-day use of analgesics, narcotics, sinus medications

4. EXAMINATION
   

   Between attacks, patients with primary headaches should have a normal neurologic examination. During an attack of migraine the patient may be intolerant to light and might prefer to lie down in a quiet, dark room. In contrast, patients with cluster headache often pace. Chronic tension-type headache may be associated with tenderness in the cervical paraspinals or temporalis muscles. This can occur in migraines as well. The temporalis muscles should be palpated for tenderness in anyone over the age of 50 with chronic headache. The presence of papilledema may indicate the presence of an underlying mass or pseudotumor cerebri. Neurologic deficits in a patient with headaches suggest an underlying structural cause. The physician should look for signs of meningeal irritation.

5. CLUES TO DIAGNOSIS
   

   Patients who call their headaches "stress headaches" or "sinus headaches" often have migraine. Any episodic headache should be considered as a possible migraine. The likelihood increases if the pain is asymmetric, has a throbbing quality, or is accompanied by nausea, vomiting, light sensitivity, or noise sensitivity. Patients complaining of headaches should be asked about nausea, photophobia, or the need to limit activities. Patients who answer positively to two out of these three symptom questions have a 93% chance of a migraine diagnosis and, if all three are answered positively, a 98% chance of a migraine diagnosis. Relatively "featureless headaches" may be tension-type headaches. The presence of autonomic symptoms and signs such as conjunctival injection, eyelid edema, lacrimation, or rhinorrhea and unilateral pain may suggest cluster headache. Secondary headaches should be considered if one of the red flags is present. In idiopathic intracranial hypertension (pseudotumor cerebri) the headache may be accompanied by pulsatile tinnitus, diplopia, or transient visual obscurations. Many patients can be thoroughly evaluated for a long-standing headache disorder without imaging, but imaging is important when there is a dramatic change in character of the headaches, or there is reason to suspect a venous thrombosis or cerebral aneurysm. A sedimentation rate should be performed in everyone presenting over the age of 50 with new headaches, jaw claudication, visual disturbances, or scalp tenderness.

6. RED FLAGS AND WHEN TO REFER
   

   All primary headaches (migraine, tension, cluster) can be managed or referred to neurology on a subacute or routine time frame based on the degree of incapacitation. Table 6-4 describes the urgency with which patients with secondary headache should be evaluated. Secondary headaches are caused by an underlying disease and may represent the onset of a life-threatening illness. A clue provided by the history or examination can suggest a specific underlying cause and direct appropriate investigations, which most commonly include neuroimaging and/or cerebrospinal fluid analysis. Migraine, cluster headache, and paroxysmal hemicrania may all be due to a secondary cause. Giant cell arteritis is very important to recognize in patients over the age of 50 with temporal headaches and who may also have jaw claudication, polymyalgia rheumatica, eye pain, or transient visual obscurations.

   Table 6-4 Time Frame for Evaluation and Referral

   Evaluate and Refer	Acute	Subacute	Chronic
   Immediate	Recent head trauma Lightening-like worst pain Altered sensorium, weakness/numbness, papilledema, seizures Age >50, temporal pain Fever, stiff neck Gradually progressive pain
   Within 72 hours		Cluster headache Post-lumbar puncture headache Worsened by Valsalva
   Within 2 weeks			Migraine headaches Tension headaches

7. CASE DISCUSSIONS
   

   Case 6-1. Her headache description is typical for migraine without aura. As in her case, a family history of migraines is often present. Possible migraine triggers should be identified and avoided. Abortive medications might be offered, with the caveat that if the headaches occur more than twice a week one might consider a prophylactic medicine as well.

   Given the severity of her headaches and associated symptoms such as nausea, photophobia, and phonophobia, specific migraine abortives such as triptans or dihydroergotamine or ergotamine are more appropriate agents to use. The headache should be treated early in the attack. Abortive medications should not be used more than two to three days per week to avoid the risk of "transforming" the migraine into a medication overuse headache. Failure to respond to one triptan should not preclude trying another, and careful attention should be paid to the dose and route of administration. A particular triptan may need to be tried on more than one occasion prior to making a determination about its effectiveness. The common side effects that can be seen with triptans are paresthesias, chest and neck pressure, dizziness, or somnolence. Patients should be counseled about this. The triptans are contraindicated in the presence of ischemic heart disease, cerebrovascular disease, or uncontrolled hypertension. Because the history is consistent with migraine, the neurologic examination is normal, and red flags are absent, neuroimaging is not indicated.

   Case 6-2. This patient started out with episodic migraines. She now has chronic daily headache. Associated exacerbations still have migrainous features. The most likely reason for the transformation to chronic migraine is rebound headaches due to excessive use of pain medications for acute symptomatic therapy. Overuse of over-the-counter analgesics leads to a rebound headache and makes prophylactics less effective. The excessive use of short-acting over-the-counter abortives should be avoided in order to prevent development of this problem.

8. CME QUESTIONS
   

   1. Which of the following is not a "primary headache?"

   a. Cluster headache
   b. Migraine headache
   c. Postural headache
   d. Tension headache

   2. A 32-year-old man presents with a severe right-sided headache that began when he was lifting weights two days ago. Which of the following types of headaches would not be a concern?

   a. Migraine headache
   b. Postural headache
   c. Possible Arnold-Chiari malformation
   d. Subarachnoid hemorrhage

   3. A 30-year-old obese woman presents with a constant headache for the past 6 months. She also has had intermittent graying out of her vision in one eye or the other when arising from a seated position over the past month. What is the most likely diagnosis in this patient?

   a. Benign intracranial hypertension
   b. Cluster headache
   c. Migraine with aura
   d. Postural headache

   4. A 42-year-old woman presents with daily bilateral headaches for the past 5 years. She alternates between Excedrin Migraine and Fioricet, which she takes daily. What is the most likely type of headache in this patient?

   a. Cluster headache
   b. Medication overuse headache
   c. Migraine headache
   d. Tension headache

   5. A 56-year-old man presents with a gradually progressive global headache over the past three months. The pain gets worse when he coughs. His spouse has mentioned to him that his memory is not what it used to be, and he has been unable to keep up with his work duties very well during this time period. Which of the following diagnoses would not be of concern for this patient?

   a. Arnold-Chiari Malformation
   b. Subdural hematoma
   c. Tension headache
   d. Tumor

   CME ANSWERS

   1. c. postural headache
   2. b. postural headache
   3. a. benign intracranial hypertension
   4. b. medication overuse headache
   5. c. tension headache