The Primary Headaches: Clinical Features and Treatment
Dr. Paul Kiritsis, PsyD, MScMed, is a licensed medical psychologist practicing in Redwood City, California. He specializes in the diagnosis and multimodal treatment of neuropsychiatric and functional neurological disorders, as well as coordinating care for patients suffering from these ailments. He offers heterogeneous... more
I’m not sure that there’s a person on the planet that hasn’t suffered from the disabling effects of a headache at some point in their lives. It is a nagging symptom that crops up anywhere and everywhere–the hospital ED, specialist clinics, and primary care centers. Now in its third edition, the International Classification of Headache Disorders (ICHD-3) lists over 200 types. Clues about the density and scope of the document may be gleaned by perusing the ICHD-3 website which informs, “Even members of the classification committee are unable to remember all of it. It is a document that should be consulted time and time again… when the diagnosis is uncertain.”
While a deep dive into this document falls outside the conversational scope of this article, its chief components should be illuminated. In a nutshell, the ICHD-3 makes the following nosological distinctions: (1) the primary headaches, which are de novo entities and cannot be attributed to any underlying systemic or neurologic disease process, (2) the secondary headaches, essentially direct epiphenomena of existing disease processes, and (3) the craniofacial pains, a category which encompasses painful cranial neuropathies and the dreaded trigeminal neuralgia, an instantaneous and transient paroxysmal electric shock-like pain emanating from either the maxillary or mandibular region. The latter may be triggered by cold wind caressing the cheek, chewing, or brushing teeth. This third category, craniofacial pains, can be either primary or secondary.
As a general rule of thumb and somewhat counterintuitive to the layperson, there can sometimes be a confusing incongruence between theoretical distinctions made by the ICDH-3 and real-world clinical presentations: symptom pictures at the doctor’s office may combine classic elements of both tension-type and migraine headaches, for instance, and not pool exclusively from a symptom-cluster associated with just one. These common clinical presentations illuminate shared mechanisms of action, and by default the notion that these clinical phenomenologies are nuanced expressions of a single underlying pathophysiologic process. Our current nosological distinctions are arbitrary and ambiguous, to put it kindly.
Of the primary headaches, the tension-type variants are the easiest to identify as they’re inexplicably linked with and precipitated by psychosocial stressors–interpersonal conflicts, work-personal life imbalances, vocational frustration and dissatisfaction, and the list goes on and on. There are sometimes attributed to persistent and chronic muscle contraction of the neck, jaw, and scalp musculature, but this is not always the case. Qualitative characteristics include palpable sensations of a holo cephalic squeezing, pressing, or tightening band around the head that is not exacerbated by physical activity, lasts minutes to days, and is mild to moderate in intensity. There may be accompanying hypersensitivity to either light or sound [known as photophobia and phonophobia] but never both, and there is a paucity of nausea, vomiting, aura, and any autonomic change.
For mild permutations of tension-type headache, acute treatment is straightforward and uncomplicated–a prescription of simple analgesics, either a nonsteroidal anti-inflammatory drug (NSAID) like aspirin (Ibuprofen}or naproxen (Naprosyn) or paracetamol. Moreover, a significant reduction in stress levels will often translate to symptom abatement. When they occur more frequently, say between 1-14 days per month or 12-180 days per annum, a prophylactic agent like the tricyclic antidepressant amitriptyline may be prescribed.
The most common debilitating headache type is the dreaded migraine. The word itself is a derivative of hemicrania, a Greek word that translates to “half head” and alludes to the unilateral distribution of pain present in roughly 3 out of 4 individuals who suffer from the condition. In 2012, the Lancet named the migraine headache as the eighth most common cause of disability-affected life years, or the number of years lost to disability, injury, or disease in the general population. It is now recognized as a genetically-based neurobiological condition involving brain dysfunction and secondary vascular effects; it affects about 12% of the population, with a 3:1 preponderance in favor of women (18% of women, 6% of men).
The classic permutation of a migraine attack is comprised of four phases: (1) the premonitory phase, (2) the aura, (3) the headache, and (4) the postdrome. Roughly 1-2 hours before the headache itself patients may experience an abrupt disruption of homeostasis punctuated by excessing yawning and thirst, neck pain, mild cognitive dysfunction, altered mood with a predilection for irritability, blurred vision, and sweet cravings. Auras occur in about one-third of patients, with sensory predominance in the visual, tactile, and language domains. Visual auras–the most common type–involve the experience of a scintillating scotoma, that is phosphorescent or evanescent spots marching through the visual field while a somatosensory aura might involve a unilateral singling sensation that suffuses across one side of the body, the hand, arm, shoulder, or face. A small percentage of migraineurs will develop a brainstem aura, a cluster of focal neurological deficits (i.e., dysarthria, aphasia, weakness, dizziness), and even syncope during their attacks, throwing another spanner into the works. Cases of this type are diagnostically challenging and concomitantly urgent because the clinician must recourse to an ipsative assessment and determine whether the current symptom picture respects the patient’s idiosyncratic medical history or whether something qualitatively different and henceforth disquieting has manifested (i.e., something that does not respect diagnostic criteria for migraine). Auras may precede and be temporally contiguous with the headache itself, or they may cooccur. The headache proper is unilateral in scope, throbbing or pulsating in quality, and moderate to severe in intensity, and may emerge in concert with photophobia, phonophobia, nausea, vomiting, and neck pain. The migraine postdrome, on the other hand, might mimic the premonitory phase and involve a “hangover” symptom constellation of weakness, fatigue, dizziness and lightheadedness, somnolence, neck stiffness, and hypersensitivity to light and sound.
Duration and frequency of a migraine attack are variable, with attacks lasting anywhere from several hours to days. In many instances, there are specific triggers–chocolate, cheese, alcohol, monosodium glutamate, processed meats, pickled foods, menstrual periods in women, and disruption of emotional balance and psychological adjustment instigated by stress. A typical patient narrative will culminate with withdrawal from stimulus-rich environments and solitary confinement in a quiet and dark room with the shades drawn and all technological devices muted.
Many patients can emancipate themselves from migraine headaches by eliminating or modifying their respective triggers, whatever they may be. But there is also a subset of migraineurs who require abortive and/or prophylactic pharmacotherapies to reduce the duration, severity, and/or frequency of attacks, if not quash them altogether. Acute therapy involves the ingestion of an abortive agent for symptomatic relief, while prophylaxis aims to decrease the frequency and improve response to acute therapy in patients who experience four or more migraines per month. The degree of efficacy is directly contingent on early administration, preferably at the inception of the headache proper or during the aura (if there is one).
The most common migraine-specific abortive agents are triptans and ergotamine, both of which exhibit strong agonist activity at the 5-HT serotonergic receptor which mediates cranial vessel constriction. These are prescribed when other over-the-counter analgesics–an NSAID like aspirin, ibuprofen, or diclofenac potassium–are dismally ineffective at controlling the pain. Nausea and vomiting, when present, are treated with antiemetic medications like prochlorperazine (Compazine) and metoclopramide (Reglan). For patients with severely disabling and chronic migraines, a physician will prescribe either an antihypertensive beta-blocker like propranolol (Inderal) or metoprolol (Lopressor) or a calcium channel blocker like verapamil (Covera). For this purpose, commensurate efficacy is demonstrated by some antidepressants, for example, tricyclic antidepressants and SSRIs, and in some instances antiepileptic drugs like valproate and topiramate. Botulinum toxin injections are sometimes considered in treatment-refractory patients. Adjuvant nonpharmacological treatments like neck stretching exercises, acupuncture, physical therapy, biofeedback, nerve blocks and nerve stimulators, clinical hypnosis, and cognitive behavioral therapy may accompany any of these pharmacological first-line treatments. The abovementioned are all complemented by rehydration with electrolyte-rich water and subsistence in a quiet dark place.
Finally, we come to a family of headache disorders called the trigeminal autonomic cephalalgias (TACs) which a denizen of a more antiquated animistic worldview might construe as the spawn of a diabolical and pernicious chaos demon. This headache type produces very stereotyped attacks and can be differentiated from migraine and tension-type headaches by their periodicity and autonomic features. Phenomenologically, they are characterized by an excruciatingly sharp, ice-pick-like stabbing pain that is unilateral in scope and consistently emergent on the same side of the head. The pain may be described as steady and of extreme intensity, irradiating from the temporal or periorbital regions and sometimes maximal over the jaw or cheek. Some patients liken the experience to a hot iron tamping rod being thrust through the eye socket or the eye being pushed out by an imperceptible hand. Occurring on the same side of the head the associated autonomic features include miosis (pupillary constriction)or ptosis (drooping eyelid) of the eye, facial sweating or flushing, eyelid edema, other pupillary abnormalities, nasal congestion and rhinorrhea ( runny nose), lacrimation (tearing), and conjunctival injection (enlargement of blood vessels in the eye). Nausea and vomiting may be present. In contradistinction to migraineurs, TAC sufferers experience their headaches on the same side of the head and usually become aggravated and aggressive during attacks; they avoid the recombinant position and will partake in vigorous physical activity or self-detrimental activity like striking themselves with something and running into a wall to distract themselves from the intolerable pain.
The TCAs are five in number and are classified in the context of two conversely related diagnostic criteria: from highest to lowest in attack frequency and from shortest to longest duration of acute attacks. Iterated here from shortest to longest headache length, they are: (1) short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT), (2) short-lasting unilateral neuralgiform headache attacks with cranial autonomic symptoms (SUNA), (3) paroxysmal hemicrania, (4) cluster headache, and (5) hemicrania continua. These headache syndromes are clinical rarities, and hence necessitate vascular imaging to screen for a space-occupying lesion or some other underlying etiology. Cluster headache, the most common TAC, is ten to fifty times less common than migraine with a male to a female preponderance of 3:1.
SUNCT and SUNA can occur up to 100 times a day, with each attack lasting ten minutes or less; the former comes replete with multiple autonomic features (including lacrimation and conjunctival injection), while the latter is usually only associated with either lacrimation or conjunctival injection. Paroxysmal hemicrania, a disorder associated with very transient unilateral pain, can occur ten times a day, while hemicrania continua–a disorder characterized by an unremitting unilateral headache of moderate intensity overlapping with transient periods of excruciating stabbing pain in the retroorbital regions and subtle autonomic signs–may last twenty minutes to several days. A cardinal feature of the cluster headache is periodicity and rhythmicity, with an inaugural cluster period persisting for 6-12 weeks and then abating for months or years. There may be 1-3 or more isolated attacks of pain within 24 hours and onset 1-2 hours after falling asleep, frequently during a REM-bound state. These may awaken the patient, who might henceforth pace back and forth in a vexed state.
The nature of cluster attacks, that being rapid onset and an almost instantaneous course to the peak intensity of pain renders prescriptions of orally administered abortive agents futile. For these one would recommend: oxygen inhalation, 100% by mask at 8-10 L/minute for 10-15 minutes; intranasal lidocaine, administered by dripping 4% viscous lidocaine in the ipsilateral nostril; or a 4-6 mg dose of sumatriptan via subcutaneous injection. Prophylactic pharmacotherapy is the lay of the land for patients who experience more frequent cluster attacks–first-line prophylactic agents include a calcium channel blocker like verapamil (Covera) or lithium carbonate. Patients with bona fide paroxysmal hemicrania or hemicrania continua have an absolute response to prophylactic indomethacin (Indocin); a complete response to the latter is essentially pathognomonic of these conditions. The more esoteric entities SUNCT and SUNA may respond to prophylactic antiepileptics, specifically lamotrigine (Lamictal), topiramate (Topamax), and gabapentin (Neurontin).
