Approach to the Patient with Chronic and Recurrent Headache
David S. Lefkowitz
Most chronic, recurrent headaches represent a benign headache syndrome; however, headache may also be a symptom of serious CNS or systemic disease. Therefore, the differentiation of primary from secondary headache is an important goal of evaluation.
Primary Headache Disorders
Migraine, tension-type headache, cluster headache, and chronic daily headache represent the overwhelming majority of headaches seen in primary care. Chronic daily headache is more common in referral-based practices specializing in headache. The International Headache Society (IHS) revised its classification system in 2004 and defines headache syndromes on the basis of signs and symptoms. The characteristics of the primary recurrent headaches are described below and in Table 21.1.
I. MIGRAINE
Migraine is now thought to be due to the disturbed function of the trigeminovascular system rather than a primary abnormality of blood vessels. Therefore, the IHS classification no longer refers to migraine as “migraine headache of the vascular type.”
A. The clinical characteristics of migraine are outlined as follows in the IHS criteria.
1. Duration of untreated or unsuccessfully treated headache is 4 to 72 hours.
2. The quality of the pain fulfills at least two of the following: unilaterality, pulsatility, moderate to severe intensity, and aggravation by routine physical activity.
3. At least one of the following is present: nausea or vomiting, photophobia and phonophobia.
4. The diagnosis of migraine and other benign headache syndromes requires the exclusion of organic disease.
5. There should be at least five such episodes if there is no aura or at least two if an aura is present.
B. Migraine with aura (formerly classical migraine) is associated with focal neurologic symptoms and signs. It is a manifestation of cortical spreading depression. Typical migraine auras include homonymous visual disturbance, unilateral paresthesia or numbness, unilateral weakness, aphasia, or unclassifiable speech disturbance. Sensory auras typically have a cheiro-oral distribution and “march” from one body part to another. By definition, the aura meets at least three of the following criteria:
1. It consists of one or more fully reversible positive or negative symptoms of focal cerebral or brainstem dysfunction.
2. One aura symptom develops gradually over 5 or more minutes. If two or more symptoms occur, they do so in succession.
3. No aura symptom lasts more than 60 minutes, or proportionately longer if there is more than one.
4. Headache follows within 1 hour of the aura, precedes the aura, or occurs simultaneously with it. An aura need not begin before the headache.
C. Migraine auras may be prolonged or permanent. Migraine with persistent aura occurs in patients when one aura symptom persists for more than 7 days and neuroimaging studies remain normal. Migrainous infarction is defined as a complication of migraine in which aura does not completely resolve within 7 days and there is evidence of infarction on imaging.
TABLE 21.1 Characteristics of Benign Recurrent Headache Disorders | |||||||||||||||||||||||||||||||||||||||||||||
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D. Migraine aura without headache (migraine equivalent) occurs when the aura occurs without headache.
E. Migraine may be triggered by specific endogenous or environmental factors, including ingestion of tyramine-containing foods or alcohol, changes in sleep pattern, and emotional stress. There is also a relation with hormonal factors. Migraine is more frequent in women after menarche. For most women, it is more frequent or severe at or around menses, and the headaches often improve after menopause.
F. The new classification system recognizes a subgroup with chronic migraine that has more than 15 headache days per month or 180 per year.
II. TENSION-TYPE HEADACHE
Tension-type headache replaces the terms “tension” or “muscle contraction” headache because of evidence that muscle tension is not the underlying mechanism of pain.
A. Clinical characteristics of tension-type headache include the following:
1. Headache duration is 30 minutes to 7 days.
2. The pain meets two of the following criteria: pressing or tightening quality, nonpulsatility, mild to moderate intensity, bilaterality, and lack of aggravation by routine physical activity.
3. Absence of nausea or vomiting, although anorexia does occur. Photophobia or phonophobia may occur but not simultaneously.
4. No evidence of organic disease.
5. There may or may not be tenderness of pericranial muscles to palpation.
6. A history of 10 similar headaches.
B. Tension-type headache is episodic when there are fewer than 15 headache days per month or 180 days per year for at least 6 months and chronic when headache frequency exceeds these limits.
III. BENIGN RECURRENT HEADACHE
Migraine without aura and episodic tension-type headache may be difficult to differentiate in some patients because both can be bilateral, nonthrobbing, moderately severe, and associated with anorexia, photophobia, or phonophobia without violating IHS criteria. Some patients may have mixed headaches with features of both or have both types independently. It has been postulated that migraine and tension-type headache are phenotypic expressions of a common abnormality of serotoninergic nociceptive mechanisms referred to as benign recurrent headache. As headache frequency increases, the severity and association with autonomic and neurologic symptoms decrease.
IV. TRIGEMINAL AUTONOMIC CEPHALGIAS
This is a family of disorders manifested by brief, intense, unilateral headaches associated with signs of parasympathetic hyperactivity. The most frequent is cluster headache. Trigeminal autonomic cephalgias are associated with activation of the inferior posterior hypothalamus.
A. Cluster headache. The clinical manifestations of cluster headache are quite characteristic, and the disorder is readily diagnosed from the history and physical examination.
1. A diagnosis can be made using IHS criteria after five or more attacks with the following features:
Severe unilateral orbital, supraorbital, or temporal pain lasting 15 to 180 minutes. The pain is usually described as boring.
Patients invariably have at least one ipsilateral autonomic sign, including conjunctival injection, eyelid edema, lacrimation, nasal congestion or rhinorrhea, forehead and facial sweating, miosis, or ptosis and agitation.
Attacks occur from once every other day to eight times a day. There is a tendency for attacks to occur at the same time each day or to awaken the patient from sleep, usually in the early morning during rapid eye movement sleep.
2. The typical episodic pattern consists of periods of headache lasting 1 week to 1 year separated by remissions of at least 2 weeks. Chronic cluster occurs when headaches persist for at least 1 year without interruption or if remission lasts <2 weeks. Secondary chronic cluster headache occurs if chronic cluster develops after a period of typical episodic headaches.
3. During cluster periods, headache may be precipitated by alcohol, nitrates, and histamine.
B. Cluster headache variants. Several headache disorders, some of which are of mostly historical significance, are thought to represent cluster headache. Raeder originally described a parasellar syndrome of trigeminal pain, oculosympathetic paresis, and cranial nerve dysfunction. The term Raeder’s syndrome came to be used for non-neuralgic head pain and oculosympathetic paresis without other cranial nerve palsies. “Lower-half headaches” such as Sluder’s syndrome, sphenopalatine neuralgia, vidian neuralgia, and greater superficial petrosal neuralgia, which manifest as facial pain and ipsilateral nasal congestion or rhinorrhea may also represent cluster headache.
C. Chronic paroxysmal hemicrania is an unusual disorder mainly affecting women with multiple, brief, unilateral, cluster-like headaches occurring at least five times a day and absolute responsiveness to indomethacin. The pain is generally severe and lasts <30 minutes. There is at least one ipsilateral autonomic symptom during the headache including conjunctival injection, lacrimation, rhinorrhea, nasal congestion, eyelid edema, or oculosympathetic paresis.
D. Short-lasting unilateral neuralgiform cephalgia with conjunctival injection and tearing (SUNCT) is the rarest of the trigeminal autonomic cephalgias. The headaches are more frequent than in the other syndromes, are briefer and less severe, and autonomic involvement generally consists of ipsilateral lacrimation and conjunctival injection. The pain is sharp or pulsating and lasts <4 minutes. There may be as many as 200 attacks a day. Patients without conjunctival injection or lacrimation but with other autonomic symptoms may have a subgroup of this disorder.
V. OTHER PRIMARY HEADACHE DISORDERS
A. Hemicrania continua is a type of chronic daily headache that is strictly unilateral, constant, moderately severe, and usually associated with autonomic signs such as lacrimation, conjunctival injection, rhinorrhea or nasal congestion, ptosis or miosis, and completely responsive to indomethacin.
B. Idiopathic stabbing headache has previously been referred to as jabs and jolts or ice pick pains. It is frequently associated with other primary headaches such as migraine or cluster headache. The clinical pattern is distinctive and diagnosed as follows:
1. Pain is localized to the head, mainly to the distribution of the ophthalmic division of the trigeminal nerve.
2. The pain is stabbing in quality, lasts for a fraction of a second, and may occur singly or in series.
3. Headache recurs at irregular intervals of hours to days.
4. There are no structural changes at the site of the pain or in the distribution of the affected nerve.
C. Primary cough headache. This is a bilateral headache precipitated by Valsalva. Some patients with cough headache have posterior fossa mass lesions or cranio-vertebral junction abnormalities.
VI. CHRONIC DAILY HEADACHE
A. Chronic daily headache is a primary headache disorder in which headaches occur at least 15 days per month. The new IHS classification system recognizes chronic forms of migraine and tension-type headache.
B. Most patients with chronic daily headache evolve from a pattern of episodic migraine.
C. New daily persistent headache is a form of chronic daily headache, which arises acutely without antecedent migraine or tension-type headache and remains constant and unremitting from onset. Most patients can specifically identify the time of onset. The pain is bilateral, nonpulsatile, moderately severe, and unaffected by routine exertion. There may be mild nausea, photophobia, or phonophobia.
D. Analgesic rebound headache. Rebound or withdrawal headache related to overuse of narcotics, butalbital-containing analgesics, ergotamine, or over-the-counter pain medications is frequently a factor in chronic daily headache. These patients have a rhythmic cycle of headache and medication use. The patient awakens with early morning headache resulting from medication withdrawal, and the headache is relieved only by the next dose of medication. Patients may begin to use analgesics in anticipation of pain. Other symptoms of medication withdrawal include irritability, asthenia, and insomnia.
Secondary Headache Disorders
Headache may be a symptom of a variety of disorders of the nervous system.
I. TUMOR
A. Head pain among patients with brain tumors arises from traction or pressure on painsensitive intracranial structures or from production of increased intracranial pressure (ICP).
B. There is nothing pathognomonic about headache associated with brain tumor. In most cases, headache is dull, aching, or pressure-like in quality. The headache is intermittent and moderately severe in most patients. Bending or Valsalva may aggravate the pain. Headache from brain tumor rarely mimics migraine.
C. The “typical” history of severe headache worse in the morning and associated with nausea or vomiting or a history of postural, cough, or exertional headache is relatively infrequent.
D. Unilateral headache usually is on the side of the lesion. Bilateral headache usually is due to increased ICP or either midline or bilateral tumor.
E. Supratentorial tumors generally produce frontal or bifrontal headache. Infratentorial tumors generally cause occipital pain.
F. Increased ICP produces severe headache in the frontal area, vertex, or neck with nausea and vomiting.
G. Involvement of the dura or skull may produce localized pain.
II. CEREBROVASCULAR DISEASE
A. Intracranial hemorrhage. Headache, nausea, and vomiting are more commonly associated with intracranial hemorrhage than with ischemic stroke. In some patients, recurrent subarachnoid hemorrhage (SAH) may resemble migraine. Patients with aneurysmal SAH often have a warning leak or sentinel headache several days to months before substantial hemorrhage occurs.
B. Unruptured aneurysms. It is controversial how often unruptured aneurysms cause recurrent headache. In some patients, sudden onset of an intense headache with normal imaging and spinal fluid, referred to as thunderclap headache, simulates SAH. Unruptured aneurysms and segmental vascular narrowing have been reported in patients with thunderclap headache. Data suggest that in most of these cases the aneurysm is incidental. Prospective data demonstrate that SAH is rare in patients with recurrent primary thunderclap headache.
C. Arteriovenous malformation (AVM). Chronic headache occurs in approximately 15% of patients with AVMs. These headaches may be clinically indistinguishable from migraine. This relation may be coincidental because migraine is common in the general population and AVMs are rare in large series of patients with migraine who undergo imaging studies.
D. Ischemic stroke. Headache occurs in 30% to 40% of patients with cerebral infarction and 25% to 40% of those with transient ischemia. It is usually nonthrobbing, ipsilateral to the infarct, and self-limited. In patients with chronic or recurrent headache associated with stroke, the following differential diagnoses should be considered.
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