CASE 27-1
A 42-year-old woman was brought by a friend to her local hospital complaining of headache, nausea, fatigue, and altered sensation over her right side. Symptoms had gradually built over an hour. On examination, she was pale, somewhat drowsy with a Glasgow Coma Scale (GCS) of 15, and complained of the light bothering her eyes. Her neck was supple. She did not want to ambulate, and her speech was nonfluent with frequent paraphasic errors; she also had difficulty with naming and repetition. Her general physical and neurological examination was otherwise normal.
In formulating this presentation, it is essential to recognize the patient’s primary problem of concern is nonfluent dysphasia, set within the context of headache, photophobia, nausea, and altered sensation over the right side. Initially she was unable to give an accurate history due to her dysphasia. As such, her headache should be considered secondary—relating to a structural lesion or other definable perturbation of brain function, until proven otherwise.
Any process that can perturb dominant-hemisphere temporo-parietal function can cause dysphasia. The tempo of onset is a clue as to the underlying pathophysiology: stroke, trauma, infection, and inflammation all can present acutely while tumor tends to be associated with a gradual onset of symptoms. Ischemic or hemorrhagic stroke as a cause of these symptoms must be excluded in the emergent context. Less common causes of acute-onset dysphasia include lesions typically associated with focal epileptiform activity on EEG, such as herpes simplex encephalitis.
Given stroke is the diagnosis to exclude a plain CT brain is mandatory. This may show early changes related to an ischemic stroke, exclude an intra-axial bleed, and help exclude a space-occupying lesion.
Basic laboratory tests such as a full blood count and comprehensive metabolic profile, blood glucose level, coagulation screen, and blood cultures should be ordered.
A pregnancy test is mandatory in the early workup of any woman of child-bearing age, especially as she may be exposed to ionizing radiation.
A routine EKG is indicated. This may demonstrate an arrhythmia, such as atrial fibrillation, which could increase her stroke risk. It may also demonstrate ST-T changes indicative of cardiac ischemia, such as ST depression or elevation, or inverted T waves.
As she is presenting within the hyperacute period, where the use of intravenous thrombolysis or an intra-arterial intervention may be considered, in specialist centers she may also undergo a CT perfusion and CT angiogram, or an MRI brain with or without an MR angiogram. A lumbar puncture (LP) should be considered if meningoencephalitis remains a consideration.
CASE 27-1 (continued)
She was treated with an antiemetic and intravenous fluid. A plain CT brain, CT perfusion, and CTA from the origin of the vertebral arteries were unremarkable. Her blood tests, including an electrolyte panel, a coagulation screen, and blood glucose, were normal. Immediately after exiting the CT scanner, the patient was able to tell you fluently that similar episodes had occurred twice in the past; her headache and nausea gradually subsided. Soon she was able to walk normally—she had previously been reluctant as it exacerbated her headache. Investigations in a similar context had been normal in the past. She suffered from unilateral headaches every other week, often with an accompanying visual perception of a dynamic scintillating scotoma. She had been diagnosed with migraine with aura in the past. She had a strong family history of migraine with visual aura.
The primary headaches include migraine, tension-type headache, and the trigeminal autonomic cephalalgias (TACs). Both primary and secondary headaches are systematically outlined in the International Classification of Headache Disorders 3β (ICHD3β).1 Primary headaches are extremely common while secondary causes of headache are rarer. These must be actively excluded in the correct clinical context, such as in Case 27-1.
Only once the symptoms settled was the wider clinical context discernable in Case 1: this being the third such episode for this patient, in whom imaging had been normal in the past. She had a history of frequent episodic migraine, often with associated visual aura, and less commonly with sensory or dysphasic aura. This presentation is consistent with migraine with dysphasic and sensory aura.
Around one third of migraineurs have accompanying aura. Typically aura lasts less than 60 minutes, and tends to precede headache. Visual aura is the most common variety. Sensory aura is the next most common, often manifesting in a sensory spread over minutes beginning in the face or limb, rather than the abrupt onset hemi-sensory change that is more often a manifestation of stroke. Dysphasia, hemiplegia, and brainstem signs and symptoms are other less common manifestations of migraine aura.
The lifetime prevalence of headache in the general population is extremely high, approaching ubiquity. Most people have experienced a headache even if in the context of a hangover or sleep deprivation. One of the pitfalls of taking a headache history from a patient relating to both them and their immediate family is underreporting of what the patient has come to accept as “normal headache.” Primary relatives of migraineurs often suffer from occasional headaches that patients are unaware of—the symptom is never discussed, as “it is just a headache.” Across the globe in both wealthy and impoverished countries, over 10% of people have been disabled by migraine at some point, and 3% of people have migraine on most days.2
Migraine is the third most common disorder in the world behind dental caries and tension-type headache. It ranks seventh amongst global causes of disability, likely an underestimate. Migraine is the leading cause of disability worldwide amongst neurological disorders.2,3
Migraine 1-year period prevalence is 6% in men and 18% in women in the USA, and is similar in most countries in the world where it has been studied.2 The life-time incidence is 18 and 43%, respectively, for episodic migraine.4 Other primary headaches are less common. Cluster headache, the most common TAC, occurs in one in a thousand people with a ratio of three males for every female.
In one study of American national data, 5% of presentations to the Emergency department (ED) were due to headache; migraine accounted for most of these.5 In a single-institution series two thirds of patients presenting to the ED with headache had a final diagnosis of a primary headache disorder.6
A debilitating migraine attack that goes for longer than 72 hours and less than 3 months.1
Patients may present to the ED. They may require admission if initial treatment is unsuccessful.
What causes of secondary headache that tend to present with a gradual onset may be seen in an inpatient setting1?
Infection (See Chapter 7)
Viral
Viral meningitis is a common cause of the symptom complex of meningism—nuchal rigidity, photophobia, and nausea may accompany headache. Examples include the herpes family Epstein-Barr virus (EBV) where symptoms may complicate a bout of “glandular fever,” or enteroviruses such as echovirus and coxsackievirus.
Encephalomeningitis, where focal neurological signs and altered conscious state may accompany meningism, can be caused by a wide variety of agents such as Herpes simplex.
Bacterial
Pneumococcal or meningococcal meningitis causes florid meningism with systemic signs, and can be rapidly progressive, while Listeria can cause a more indolent syndrome in at-risk populations such as patients with chronic kidney disease. Borreliaburgdorferi may cause meningitis and facial nerve palsy.
Abscess
Mycobacterial
Tuberculosis may rarely present with a tuberculoma in the CNS causing focal signs. It may also cause basal meningitis.
Fungal
A number of presentations are possible, from fulminant Cryptococcus gattii infection in an immunocompetent host with accompanying raised intracranial pressure, to an invasive mucormycosis in a drug addict.
Rickettsial
Trauma or injury to the head or neck
Head injury with or without loss of consciousness may occur (see Chapter 15).
This may involve overt brain injury such as an intracerebral, extradural, subdural, and subarachnoid hemorrhage (SAH).
Diffuse axonal injury may accompany acceleration–deacceleration injuries and not be readily apparent on a CT brain.
Headache may be part of a concussion syndrome in this context.
Ongoing headache in patents with concussion is more common if patients have a history or family history of migraine.
Whiplash injury. In this context, structural damage such as vertebral and carotid artery dissection and bony fracture must be excluded.
Craniotomy
Stroke
Headache occurs in around 50% of patients with intracranial hemorrhage and 25% of patients with ischemic stroke (see Chapter 13). The size of the stroke does not influence the likelihood of headache. Posterior circulation events are more likely to precipitate headache. Unilateral headaches tended to occur ipsilateral to the stroke side. Patients with a history of migraine are more likely to have this symptom.7
Drugs
Examples include dipyridamole used in stroke secondary prevention, granulocyte colony-stimulating factor occasionally used in neutropenic patients, and nitric oxide donors such as sodium nitroprusside used in malignant hypertension.
Drug withdrawal, such as from opioids, barbiturates, cannabinoids, or caffeine.
Inflammation
Multiple sclerosis (MS), systemic lupus erythematosus (SLE), and sarcoidosis are examples. The latter can cause pachymeningeal changes that can constitute chronic basal meningitis with associated headache. Focal neurological signs may be present if infarction or infiltration has occurred.
Malignancy
Both primary and secondary lesions can trigger this symptom. A common example can occur when a primary tumor such as lung or breast cancer causes leptomeningeal spread. Symptoms in this case can be protean and include irritability and mild nausea. Leptomeningeal involvement can be difficult to diagnose; large-volume lumbar puncture (often repeated to increase sensitivity) and MRI with and without contrast are required. Raised intracranial pressure (ICP) may occur due to impaired CSF drainage.
Tumor present in the central nervous system (CNS) can raise ICP. Papilledema may be present (see below).
Headache may be a remote complication, and may occur in primary lung or bowel cancer without metastatic disease, for example.
Derangement of homeostasis
Electrolyte imbalance relating to sodium, potassium, and calcium, for example
Hyperbaric or hypobaric environment such as when diving and at altitude, respectively
Hypoxia or hypercarbia
Hypotension or hypertension such as in pheochromocytoma
Chronic kidney disease with or without acid–base derangement
Hormonal imbalance such as hypothyroidism
Miscellaneous other causes:
Mitochondrial disorders such as mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS).
Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL)
Headache as an expression of angina pain, so-called cardiac cephalalgia
There are many other causes of secondary headache. A more comprehensive list is available from the International Headache Society website (http://www.ihs-headache.org) or the ICHD3β1
CASE 27-2
A 22-year-old football player presented with severe bilateral headache, vomiting, and profound photophobia and phonophobia that had slowly built in intensity around 2 hours after finishing his game. There was accompanying neck discomfort and fatigue. He had gone to bed in a dark room and did not want to move about, preferring to lie still. He said he woke that morning feeling “tired and clumsy,” and had not played particularly well. The game was otherwise unremarkable—he had not struck his head or been injured. Similar episodes had occurred several times in the past, often associated with exercise. His mother and two sisters all have migraine.
Migraine is a familial episodic disorder characterized by headache and associated features such as nausea and light and sound sensitivity. Pathophysiology relates to dysfunction of elements of CNS such as the thalamus, hypothalamus, brainstem, and neocortex.8 Changes of cerebral vasculature are not central to migraine pathophysiology.8 Headache is not due to extracranial or intracranial vasodilation.9 Diagnostic features of migraine, as defined in the ICHD3β,1 are presented in Box 27-1.
This patient has severe headache with movement sensitivity, nausea, photophobia, and phonophobia. The ictus lasted hours. Several episodes had occurred in the past. There is no other alternative diagnosis suggested by this presentation. The patient therefore fulfills the diagnostic criteria for migraine outlined in Box 27-1.
Box 27-1. Diagnostic features of migraine, as defined in the International Classification of Headache Disorders 3β
At least five attacks fulfilling criteria B–D
Headache attacks lasting 4–72 hours (untreated or unsuccessfully treated)
Headache has at least 2 of the following four characteristics:
unilateral location
pulsating quality
moderate or severe pain intensity
aggravation by or causing avoidance of routine physical activity (eg, walking or climbing stairs)
During headache at least 1 of the following:
nausea and/or vomiting
photophobia and phonophobia
Not better accounted for by another ICHD-3 diagnosis
Data from Headache Classification Committee of the International Headache Society (IHS). The International Classification of Headache Disorders, 3rd ed. (beta version), Cephalalgia 2013;33(9):629–808.
Premonitory symptoms can occur several hours prior to the onset of headache. They can last throughout the attack and be present for hours to days subsequent to an attack, otherwise known as the postdrome period. Sometimes these symptoms can be more bothersome and intrusive than the headache itself. Premonitory symptoms are almost ubiquitous in both children and adults migraineurs.10 Pathophysiology may relate to hypothalamic and brainstem dysfunction.11,12 The patient presented in Case 2 suffered the premonitory symptoms of fatigue, yawning, and neck discomfort hours prior to the onset of his headache.
Mood changes. Some people experience an elevation in mood prior to a migraine attack akin to euphoria, while many other patients become quick to anger, or develop low mood or anxiety.
Food cravings, particularly for sweets and carbohydrates.
Polyuria and/or polydipsia.
Yawning may occur irrespective of “sleepiness.”
Transient alteration in bowel habits, such as diarrhea.
Difficulties with attention.
This patient awoke with premonitory symptoms. It was only after the game that the headache with accompanying migrainous symptoms became manifest. As such, while there is a temporal link between the physical activity and migraine, there is unlikely to be a causal association here.
The analysis can be brought to bear on other commonly endorsed triggers, such as chocolate ingestion and exposure to bright lights. In the case of the former, it is likely the premonitory symptom of sweet craving precedes the migraine, while increased photosensitivity leads to ambient and high-contrast lights being perceived as bright and intrusive.13,14
Aura occurs in around one third of migraineurs, and is defined as migraine with aura.1 Aura symptoms typically last less than 60 minutes, and are classified as prolonged if they persist beyond this timeframe. They are defined as a focal neurological disturbance.1 Symptoms tend to precede the headache phase of migraine but this is a variable observation.
Aura manifesting as a visual percept occurs almost universally among patients with migraine with aura.15 It is dynamic and consists of both negative and positive perceptions. Scotoma and, less commonly, constriction of peripheral vision or even complete loss of vision are examples of the former. Teichopsia, also known as fortification spectra, flashing lights, and scintillations are examples of the latter. The symptoms of difficulty in focusing, prolonged blurring of vision, and transient scintillations on straining or standing, commonly encountered among migraineurs, do not represent aura.
Sensory aura occurs in around one third of patients with migraine with aura, as in the patient in Case 1. It consists of a spread over minutes of positive or negative sensory symptoms. This tempo distinguishes migraine from causes of abrupt-onset sensory change, such as stroke. Symptoms tend to proceed in a distal to proximal direction; propagation in the opposite direction is also common. Variability exists not just between people but also between attacks in an individual. Common examples include a cheiro-oral wave of paresthesia and numbness that begins from the fingers on one side of the body and travels proximally up the arm over minutes ultimately leading to ipsilateral facial sensory changes. Real-life examples do not always correlate with contiguous parts of the sensory homunculus represented at different organizational levels of the brain.
Dysphasic aura occurs in almost 20% of patients with migraine with aura. It is typically nonfluent, as in Case 1. It must be distinguished from transient word-finding difficulties and occasional brief disfigurement of syntax that migraineurs report.
Hemiplegic aura is somewhat of a misnomer as symptoms are typically better described as hemiparesis. It must be distinguished from the generalized asthenia that migraineurs commonly experience. It occurs in around 5% of patients with migraine with aura, and can occasionally be prolonged even in the absence of any objective imaging finding suggesting stroke or oligemia. Hemiplegic migraine can be sporadic or familial, both with a similar prevalence; the latter is defined by having a first- or second-degree relative affected. Hemiplegic symptoms can recur. Signs of brainstem dysfunction may be present.
Brainstem aura, previously described as basilar-type migraine. This nomenclature has fallen out of favor as basilar artery caliber is not altered during an attack. Symptoms as defined by the International Headache Society include1:
Dysarthria
Vertigo
Tinnitus
Diplopia
Hypoacusis
Ataxia
Decreased level of consciousness
FHM1. Defined as a mutation in the CACNA1A gene, coding for a P/Q voltage-gated calcium channel, on chromosome 19 with the accompanying clinical syndrome and appropriate family history in a first- or second-degree relative. This mutation is responsible for around 50% of FHM cases. Cerebellar ataxia, either progressive orepisodic in nature, is a common accompaniment. Minor head trauma can trigger an attack, and brainstem signs such as coma can occur, often prompting aggressive treatment for epilepsy or encephalitis in patients.
FHM2. There are mutations in the ATP1A2 gene, coding for a K/Na-ATPase, on chromosome 1. This accounts for around 20% of FHM cases. An epilepsy phenotype may occur. This mutation may also be responsible for alternating hemiplegia of childhood.
FHM3. There are mutations in the SCN1A gene, coding for a sodium channel, on chromosome 2.
FHM genetics suggest migraine may be an ionopathy.8
Extensive studies among families with migraine both with and without aura suggest this genetic brain disorder has a complex inheritance, analogous to hypertension or type-2 diabetes mellitus.
Two families with both migraine and advanced sleep phase have demonstrated mutations in the gene encoding casein kinase 1 delta. This protein phosphorylates clock protein Per2 among other brain proteins and therefore has an influence on circadian rhythm.16
Aura symptoms likely correlate with cortical spreading depression. This is an electrochemical wave largely related to modulation of calcium ions that propagates at around 3–6 mm/min. It can be experimentally induced. Surrogate markers of focal hyperemia followed by spreading oligemia have been demonstrated within vivo neuroimaging.8
The relative risk of ischemic stroke in people who had migraine with aura is 2.16 (95% confidence interval 1.53–3.03).
The relative risk of ischemic stroke in people with migraine without aura is 1.23 (0.90–1.69). Note the confidence intervals cross 1.0. As such migraine without aura is not considered a risk for stroke.
The relative risk of ischemic stroke among men with aura was 1.37 (0.89–2.11) compared to women (2.08, 1.13–3.84). Again note the confidence intervals cross 1.0 regarding men with aura.
Among female patients with migraine with aura, the following further increased relative risk:
Age under 45
Use of the estrogen-containing oral contraceptive pill (OCP)
Smoking, especially when used concurrently with the OCP
There is no evidence for using aspirin in primary prevention for ischemic stroke in women with migraine with aura. Any benefit may be outweighed by hemorrhagic risk. Studies are awaited.
Many authorities do not recommend the use of estrogen-containing OCPs in women of child-bearing age due to increased risk of ischemic stroke.
Risks may be lower in OCPs with lower amounts of estrogen. This remains to be studied, but is biologically plausible given exogenous estrogen can create a relatively thrombophilic milleu in a dose-dependent fashion.
The increase in relative risk of stroke can be discussed with individual patients, and considered in light of the low absolute risk of stroke in young women as well as the risks of pregnancy. The risk of stroke can be worked out for an individual using an online calculator utilizing data from the Framingham study.
Some patients may want to persist with the OCP despite the risk as they prefer this form of contraception.
Some patients are using the OCP for conditions such as endometriosis. Discussion with the patients’ OBGYN may be required.
The OCP containing progesterone only—“the mini-pill”—is available. It has some drawbacks compared to the estrogen-containing OCP from an OGYN perspective.
Progesterone depot injection or eluting implant such as Implanon.
An intrauterine device can be used. Some devices can elute estrogen compounds, so consultation with an OBGYN is urged.
Barrier protection.
Vasectomy.
Triptans can be used in patients with common varieties of aura such as visual and sensory aura.
In the past, brainstem symptoms associated with migraine were thought to be due to vasospasm of the basilar artery (described as basilar or basilar-type migraine in previous iterations of the ICHD)
Studies have demonstrated this supposition to be incorrect.
Medication packet inserts and the FDA recommendations suggest triptans should not be used in this context.
This was due to concern regarding possible vasospasm of the basilar artery precipitating ischemic stroke.
Individual use should be considered in the context of the patient’s disability due to migraine and medical comorbidities.
Yes. This tends to occur later in life, and may be alarming to the patient.
CASE 27-3
A 23-year-old woman presents to her general practitioner complaining of frequent headache occurring on average 20 days a month. She finds it interferes with her ability to study at college. Headaches occurred since menarche, and are similar to her mother’s. Pain can occur anywhere in the head and face, and can be dull or throbbing in quality. On further questioning, it is apparent that she always has some cranial discomfort, with moderate worsening that often occur on the weekend. Occasionally she will spend all day in bed, nauseous, unable to socialize or go to class. She usually wears sunglasses as the light always bothers her, and uses earplugs at night as sounds are irritating. She does not like to use a beanie in winter as it sits uncomfortably on her head. Her neurological and general examination is normal.
This is chronic migraine. It is a common presentation to the neurology outpatient clinic, and can be highly disabling.3 It can be diagnosed when migraine symptoms occur on at least 8 days per month, and headaches occur on at least 15 days per month.1 Exacerbations can lead to presentations to the ED as seen in Case 1. Given the high prevalence of migraine these patients are often in the hospital for another reason entirely. Migraine symptoms can lead to a neurological review, where they should be recognized.
This patient has had a typical transformation from episodic to chronic migraine.1 In this setting, there is no evidence that imaging is indicated; the rate of pathological findings is the same as the general community. Imaging may increase morbidity when an incidental finding such as a calcified pineal gland or small arachnoid cyst is discovered and disclosed as abnormal.
Cranial or somatic allodynia can commonly accompany a migraine ictus and can be continuously present in a patient with chronic migraine, as in Case 3. This symptom is a state in which an otherwise innocuous stimulus such as touching one’s face or brushing one’s hair is perceived as noxious. Some patients with migraine, particularly those with frequent attacks, experience discomfort putting their head under the shower, or placing one side of their head on a pillow. It is an indication of dysfunction of higher-order sensory processing.8
Cranial autonomic features are an important clinical aspect of migraine neurology. These occur unilaterally, often ipsilateral to headache, or bilaterally. These symptoms occur in up to 70% of children; patients are often misdiagnosed with sinus headache. In adults, around two thirds of migraineurs will have at least one cranial autonomic symptom, of which roughly half will be strictly unilateral.8
Cranial autonomic symptoms are the manifestation of activation of a reflex arc depicted in Figure 27-1. The afferent limb of this reflex arc consists of fibers in the ophthalmic (first) division of the trigeminal nerve and efferent pathway from cell bodies in the superior salivatory nucleus (SSN), axons of which course through the facial nerve (7th cranial nerve) to synapse in the sphenopalatineganglion (SPG). Subsequently, the efferent limb of the reflex arc projects through the greater superficial petrosal nerve.
Cranial autonomic symptoms are outlined in Box 27-2, and include conjunctival injection and tearing as well as aural fullness. Most of these symptoms represent cranial parasympathetic activation, while meiosis and ptosis relate to focal sympathetic dysfunction.
Somatic autonomic features such as alteration in temperature regulation, a predisposition to presyncopal episodes, and erectile dysfunction.
Figure 27-1
The trigeminal autonomic reflex.19 Afferent traffic from nociceptive endings in the dura mater with cell bodies in the trigeminal (V) ganglion project to trigeminal nucleus caudalis and the dorsal horns of C1 and C2 to form the trigemino-cervical complex. There is a reflex connection to the superior salivatory nucleus in the pons with an outflow through the facial (VIIth) nerve that primarily synapses in the sphenopalatine ganglion.
Box 27-2. Cranial autonomic symptoms commonly seen in migraine and ubiquitous in the TACs, as set out in the International Classification of Headache Disorders 3β
a) conjunctival injection and/or lacrimation
b) nasal congestion and/or rhinorrhea
c) eyelid edema
d) forehead and facial sweating
e) forehead and facial flushing
f) sensation of fullness in the ear
g) miosis and/or ptosis
Data from Headache Classification Committee of the International Headache Society (IHS). The International Classification of Headache Disorders, 3rd ed. (beta version), Cephalalgia 2013; 33(9):629–808.
Anxiety
Depression
Postural orthostatic tachycardia syndrome
Tinnitus
Narcolepsy
Restless legs syndrome
Essential tremor
What comorbidities are more common in chronic migraine patients compared to those with episodic migraine18?
Depression
Anxiety
Chronic pain
Respiratory disorders
Asthma
COPD
Cardiovascular risk factors
Obesity
Hypertension
Insulin resistance
Dyslipidemia
Overuse of particular medications among patients with episodic or chronic migraine can increase the frequency and intensity of headache exacerbations. Migraine preventives are less likely to work in the context of overuse. Barbiturates such as butalbital, still available in the USA, have been demonstrated to have this effect when used as few as 5 days per month. Triptans such as sumatriptan and opioids such as oxycodone, codeine, and tramadol can also have this effect when used for more than 10 days a month.1 As such, opioids and barbiturates should not be used in the management of chronic migraine. Triptan use should be limited to 2 days per week.
CASE 27-4
A 35-year-old man came into the ED complaining of excruciating pain on one side of his head. This had come on for periods of around 45 minutes, during which he walked around agitated. Episodes had woken him from sleep at 1 a.m. for the past 5 days; this time an episode occurred at 8 p.m., prompting his presentation. He had never had a headache in the past, and says the new pain is much worse than when he broke his arm. He had some moderate photophobia in the eye ipsilateral to the infraorbital headache. On inspection, there was epiphora and periorbital edema of the left eye; the remainder of the exam was unremarkable. He was given high-flow oxygen (100% at 15 L/min), and the headache subsided within minutes.
This man has cluster headache. It is the most common of the trigeminal autonomic cephalalgias (TACs), and is more likely to occur in men. While less common than migraine, it is by no means unusual. It occurs in around 1/1000 people.19–21 Pain typically occurs in the ophthalmic division of the trigeminal nerve. Discomfort can come on as a throbbing or sharp pain or more commonly a single stab or a train of stabs. The duration of the attack is 15–180 minutes.1 Between attacks there can be an element of discomfort. Female sufferers describe the discomfort as more severe than childbirth, while men have described it as more excruciating than a long-bone fracture or passing a kidney stone.
Cranial autonomic symptoms
These are outlined in Box 27-2, and include unilateral periorbital edema and tearing as seen in Case 4. At least one of these symptoms or signs must be present ipsilateral to the head pain to make the diagnosis if agitation is absent.
A sense of agitation or restlessness
Patients often pace, sometimes smoking a cigarette or otherwise fiddling.
This symptom contrasts with most people suffering from a migraine attack, in which most people prefer to remain still and quiet.
Unilateral photophobia or phonophobia, ipsilateral to the head pain, can occur. This phenomenon is suggestive of a TAC rather than migraine.
Attacks occur in bouts typically lasting weeks.
Bouts can occur at the same time every year in some patients.
Within an individual a pattern of attacks every other day to 8 attacks per day may be established.
Attacks often occur at a particular time of the day or night. Two hours after going to sleep is a common example.
Some patients never have periods of relief from their cluster attacks lasting longer than 1 month. These patients are considered to have chronic cluster headache, a condition much less common than episodic cluster headache.
The clear response to high-flow oxygen in Case 4 is typical of cluster headache.
Sumatriptan 6 mg (SC) or 20 mg (IN), and zolmitriptan 5 mg (IN) can be effective in aborting a cluster attack. Oral triptans have less favorable pharmacokinetics and pharmacodynamics and tend to be unhelpful in this condition.
A trial of indomethacin is always unrewarding in cluster headache. In contrast, patients with paroxysmal hemicrania (PH) experience a complete response—this is diagnostic (see Box 27-3). PH is characterized by more frequent attacks of shorter durations, often without the striking circadian rhythmicity seen in cluster headache.
Box 27-3. The TACs
| Cluster Headache | Paroxysmal Hemicrania | SUNCT/SUNA | Hemicrania Continua | |
|---|---|---|---|---|
| Sex | 3 M to 1 F | M = F | 1.5 M to 1 F | 1 M to 1.6 F |
Pain Quality Severity Distribution |
Sharp/stab/throb Very severe V1>C2>V2>V3 |
Sharp/stab/throb Very severe V1>C2>V2>V3 |
Sharp/stab/throb Severe V1>C2>V2>V3 |
Throbbing/sharp/constant Moderate to severe V1>C2>V2>V3 |
Attacks Frequency (per day) Length (minutes) |
1–8 15–180 |
11 2–30 |
100 1–10 |
Continuous with exacerbations — |
| Cutaneous trigger | Absent | Absent | Present | Absent |
| Agitation/restlessness | 90% | 80% | 65% | 69% |
| Episodic versus chronic | 90:10 | 35:65 | 10:90 | 15:85 |
| Periodicity | Present | Absent | Absent | Absent |
Treatment effects Oxygen Sumatriptan (6 mg) Indomethacin |
70% 90% No effect |
No effect 20% 100% |
No effect <10% No effect |
No effect No effect 100% |
Migraine features with attacks Nausea Phonophobia/photophobia |
50% 65% |
40% 65% |
25% 25% |
53% 79% |
TACs
Paroxysmal hemicrania can have similar semiology and can be difficult to distinguish from cluster headache, particularly when attacks are prolonged (see Box 27-3). As such, a trial of indomethacin should be carried out if there is diagnostic uncertainty. This consists of a single 100 or 150 mg intramuscular dose of indomethacin carried out with a placebo analogue on a separate occasion. If the parenteral form is unavailable, the following oral regimen can be used:
25 mg TID for 5 days
50 mg TID for 5 days
75 mg TID for 10 days
Some patients will be unable to tolerate the trial due to gastrointestinal side effects. Concurrent use of a proton pump inhibitor may be required. Other dose-limiting side effects can include dizziness and/or confusion.
Other TACs such as SUNCT/SUNA and the indomethacin-responsive hemicrania continua, a differential diagnosis to exclude in prolonged side-locked headache, have a temporal profile distinct from cluster headache (Box 27-3).
Migraine
As seen above, cranial autonomic symptoms are common in migraine. Circadian and seasonal rhythmicity can also occur. Attack duration is prolonged in migraine, however, precluding any confusion with cluster headache for the most part.
Hypnic headache
This rare primary headache, colloquially known as “alarm clock headache,” is characterized by attacks that wake typically older adults from sleep at a set time on a nightly basis. Migrainous symptoms may accompany the ictus. Caffeine prior to sleep can paradoxically be of benefit in sleeping through the night attack free.
Primary stabbing headache
This stabbing discomfort can occur anywhere over the cranium. It tends to occur among migraineurs. It lacks the temporal profile and cranial autonomic features that are so particular to cluster headache.
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