Diagnosis and Treatment of Primary Headache Disorders in Older Adults

Thomas Berk, MD; Sait Ashina, MD; Vincent Martin, MD; Lawrence Newman, MD; Brinder Vij, MD

Disclosures

J Am Geriatr Soc. 2018;66(12):2408-2416. 

In This Article

Abstract and Introduction

Abstract

Objectives: To provide a unique perspective on geriatric headache and a number of novel treatment options that are not well known outside of the headache literature.

Design: Review of the most current and relevant headache literature for practitioners specializing in geriatric care.

Results: Evaluation and management of headache disorders in older adults requires an understanding of the underlying pathophysiology and how it relates to age-related physiological changes. To treat headache disorders in general, the appropriate diagnosis must first be established, and treatment of headaches in elderly adults poses unique challenges, including potential polypharmacy, medical comorbidities, and physiological changes associated with aging.

Conclusion: The purpose of this review is to provide a guide to and perspective on the challenges inherent in treating headaches in older adults.

Introduction

Most headache disorders present between the ages of 18 and 44,[1] and the incidence of headache in general decreases steadily with age.[4] The prevalence of headache remains high in elderly adults,[2,3] and many secondary causes of headache also increase with age.[5] Older adults, particularly those aged 65 and older, are more likely to experience a number of pain syndromes in general,[6] as well as polypharmacy, multiple comorbid medical conditions, and intolerance to pain medication.[7] These factors can complicate the process of making an accurate diagnosis in this population.

Headache Diagnoses

The International Headache Society has published a classification system of headache disorders, the International Classification for Headache Disorders, currently in its third edition (ICHD-3). This classification system[8] provides diagnostic criteria for all headache disorders and categorizes them into primary headaches, secondary headaches, painful cranial neuralgias, other facial pain, and other headaches. The classification is based on criteria that include the quality, location, and duration of the pain and associated symptoms. The frequency of a headache provides a further diagnostic criterion – namely, whether a headache is episodic or chronic (≥15 headache days/month for a minimum 3 months)[8] which can also differentiate appropriate treatments.

Primary Headaches

The term "primary headache" refers to headache pain in the absence of another organic disorder known to cause headache. Tension type headache (TTH) is the most common primary headache worldwide, with a prevalence in elderly adults of 35.8%. Migraine is the next most common headache disorder, with a prevalence of 5.7% in elderly adults.[2,3] Women entering menopause may experience a worsening of headaches before ultimate improvement once entering the postmenopausal state[9,10] A list of common types of primary headaches occurring in individuals aged 65 and older is presented in Table 1.

Migraine. Migraine is characterized by a moderate to severe, usually throbbing, headache attack associated with nausea and light or sound sensitivity lasting 4 to 72 hours. A characteristic aura that can be a visual, sensory, language, or speech change can precede a migraine. Migraine without aura and aura without migraine are the most common phenotypes in elderly adults. Aura without headache occurs in older people with a prior history of migraine. As they age, the frequency of aura may begin to increase, whereas painful headache attacks generally become less severe and frequent. The decrease in incidence and frequency of migraine after menopause results in a narrowing of the prevalence gap between men and women with age.[11]

Migraine aura without headache was first described as "late-life migraine accompaniments"—episodic auras in older individuals that mimic transient ischemic attacks.[12] Other outdated terms for migraine aura without headache include "acephalgic migraine," "typical aura without headache," "migraine equivalents," and "complicated migraine." The presence of aura has been noted as a statistically significant vascular risk factor and may need to be considered alongside other concomitant vascular risk factors for primary prevention in individuals with multiple comorbidities. The use of estrogen-containing medications in postmenopausal women with a history of migraine with aura is somewhat controversial as well, although less so than the use of oral contraceptives containing estrogen.

Late-life aura with and without headache typically presents as visual symptoms but could also occur as speech (dysphasic) or sensory symptoms.[13] A wide variety of transient neurological disturbances in migraine aura can mimic other serious neurological conditions such as transient ischemic attacks and stroke and can be easily misdiagnosed. Meticulous clinical correlation, neurological examination (including funduscopic examination), and other appropriate investigations are crucial to exclude secondary causes. Familial or sporadic hemiplegic migraine, due to a motor aura, presents as transient hemiplegia. In older adults, especially those with risk factors for cerebrovascular disease, it is essential to consider and exclude transient ischemic attack or stroke presenting with hemiplegia.

The majority of older people with migraine experience a significant improvement in headache symptoms with age, with less intensity and less frequent attacks.[14] A positive family history of a primary headache disorder and a history of motion sickness during childhood have been associated with greater risk of chronic migraine in older women.[15]

Tension-Type Headache. TTH is the most common headache worldwide, as well as in elderly adults.[16] It has been famously called "the featureless headache" because it is most notable for what people do not experience—the typical migraine features and associated symptoms such as photo- and phonophobia, nausea, and autonomic symptoms. Pericranial muscle tenderness and myofascial pain may be prominent features in some individuals of TTH, which is classically bilateral, pressing, dull, or achy in quality and mild to moderate in intensity.[17] TTH can be episodic or chronic depending on the number of headache days per month, as described above.

Trigeminal Autonomic Cephalalgias. The trigeminal autonomic cephalalgias (TACs) are a unique group of primary headache disorders characterized by unilateral headache attacks in the V1 distribution of the trigeminal nerve. Attacks are associated with ipsilateral autonomic features such as lacrimation, ptosis, pupillary irregularity, flushing, conjunctival injection, and rhinorrhea. Late-onset unilateral headache with ipsilateral autonomic features warrants evaluation for a potential secondary cause because of the high frequency of secondary TAC due to pituitary abnormalities and other intracranial pathology.[18] The TACs are cluster headache, hemicrania continua, the paroxysmal hemicranias, and short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) or cranial autonomic features (SUNA). Although they seem similar, they have differences in their pain quality, attack frequency, duration, and associated symptoms.

Cluster headache is the most common TAC, with a prevalence of 53 per 100,000 persons of all ages.[19] To meet the ICHD-3 beta criteria for cluster headache, one must experience a minimum of 5 attacks fulfilling the following: severe or very severe unilateral orbital, supraorbital, or temporal pain lasting 15 to 180 minutes when untreated and either or both of the following: sense of restlessness or agitation and ipsilateral autonomic features (conjunctival injection or lacrimation, nasal congestion or rhinorrhea, eyelid edema, forehead and facial sweating, forehead and facial flushing, sensation of fullness in the ear, miosis or ptosis).

Cluster headache attacks can occur as little as 1 every other day to 8 per day for more than half of the time when this headache disorder is active. Attacks typically occur in series lasting for weeks or months (so-called cluster periods) separated by remission periods usually lasting months or years. Cluster headache is approximately 4 times as common in men as in women.[19] The peak incidence has been reported to be aged 40 to 49 for men and 60 to 69 for women[20] and has been observed in men and women aged 70 and older.[21]

Other TACs

Hemicrania continua is a persistent and constant unilateral headache with intermittent ipsilateral autonomic features that uniquely responds to indomethacin, Paroxysmal hemicrania (PH) is a rare TAC characterized by unilateral, intermittent, stabbing pain lasting 2 to 30 minutes that also uniquely responds to indomethacin. SUNCT and SUNA are characterized by extremely short, severe attacks associated with autonomic symptoms typically lasting 1 to 600 seconds and occurring up to 200 times daily. The incidence of these rare TACs in elderly adults is unknown, but they have been described as primary headache disorders and as manifestations of secondary headache.

Other Primary Headache Disorders

Hypnic Headache. Hypnic headache is a rare form of primary headache that occurs almost exclusively in older adults,[22] with 92% occurring in those aged 50 and older.[23] It is a recurrent primary headache attack that occurs during sleep and wakes the person 3 to 5 hours after falling asleep. The headache itself is usually not migrainous (although it can be), is short lasting (from 15 minutes to 4 hours), and occurs at least 10 days per month.[8] Most of the literature on this form of headache is from case reports;[23] secondary causes of headache must be excluded in these patients, and brain imaging is generally recommended for evaluation of brainstem or posterior fossa lesions.[24] Head pain resembling the hypnic headache phenotype has been reported with medication overuse[25] and withdrawal from angiotensin-converting enzyme inhibitors[26] and lithium.[27] Nocturnal hypertension is another differential diagnosis to be considered and may require 24-hour ambulatory blood pressure monitoring.[28]

New Daily Persistent Headache. New daily persistent headache is a primary headache disorder that is daily from onset and unremitting.[8] The affected individual is likely to remember the abrupt onset and, in many cases, the date it started. Although it can be benign and self-limiting, it is frequently persistent, refractory to treatment, and unremitting and can have features of migraine or TTH. A detailed evaluation of potential secondary causes, including cerebral venous thrombosis, intracranial mass lesions, and intracranial hypotension, is necessary before making a diagnosis of new daily persistent headache.[29]

Secondary Headache

Secondary headache refers to headache pain in temporal relation to a documented and proven disorder known to cause headache.[8] Most new-onset acute and subacute headaches should be evaluated for secondary causes in all ages, and change in the pattern of a known characteristic headache disorder should prompt appropriate clinical examination. Table 2 lists a number of important secondary causes of headache that are especially relevant to consider in elderly adults. Potential red flags that might suggest a serious underlying secondary cause of headache are presented in Table 3. A common pneumonic device used to describe red flags in headache disorders is "SNOOPP" – Systemic changes, Neurological signs on exam, sudden Onset, new headache in patients Older than 50 and Pregnancy or Positional change to the headache. Depending on the person's clinical presentation, physical examination, and risk factors for secondary causes, the diagnostic examination for headache can include blood testing, lumbar puncture, brain imaging studies, and temporal artery biopsy (Table 4).

Imaging should be considered if the individual has any noticeable change to headache quality (if the headache is described as "it just doesn't feel the same as my headache") or with the presence of any of the above-noted red flags. A change in aura, for instance, a longer aura or a sensory aura when visual aura only previously occurred, would also warrant neuroimaging. Most importantly, as noted above, new-onset headache in anyone aged 50 and older warrants consideration of neuroimaging. When considering the modality of imaging, magnetic resonance imaging (MRI) is generally recommended because it avoids radiation and the images are of high quality; if time is of the essence, or MRI is not available, computed tomography may be substituted.[30]

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