Key Clinical Questions
Are there warning signs of a secondary headache that would require further imaging?
Is the headache new or different?
Is the headache brought on by exertion, sexual intercourse, coughing, or sneezing?
Is the onset of the headache sudden or severe?
Has the patient experienced antecedent head or neck trauma?
Does she have any neurologic symptoms other than visual symptoms occurring only at the beginning of the headache syndrome?
The patient described her typical migraine headaches.
A 25-year-old, right-handed woman with a 3-year history of headaches is admitted to the hospital for “pain control.” In the emergency department she had a negative noncontrast head computed tomographic (CT) scan and was prescribed a hydromorphone (Dilaudid) drip. Does the patient have any other medical problems or risk factors for intracranial pathology? Her past medical history and review of systems is otherwise negative. Her family history is positive for migraine. What factors worsen the headaches? Tension and stress triggered her headaches, typically worse 2 or 3 days before her menstrual period begins. Alcohol, chocolates and peanuts may aggravate her headache. She tried stopping the oral contraceptive and noticed no improvement in her headaches. Social history reveals that she is single and disabled from her headaches. What medications has she tried? She has tried many different medications, including analgesics, antidepressants, calcium channel blockers, and ß-blockers. The only medications that help her are sumatriptan taken subcutaneously and narcotics, currently hydrocodone at least one tablet a day. She has been taking alprazolam 10 mg three times a day for a couple of years. She also uses promethazine for nausea. Recently she is beginning to have daily headaches and has to make trips to the emergency department to get shots of meperidine. What has been her work-up to date? She has seen multiple neurologists. She has been treated with biofeedback and has seen a psychologists. She had multiple CT scans and magnetic resonance imaging (MRI) of her head. |
Introduction
Complaints of headache represent a major health problem due to their prevalence, chronicity, and the cost of ruling out life-threatening or serious underlying pathology that may cause significant morbidity and mortality. Up to 4.5% of all emergency department visits may be attributed to symptoms of headache, and headache may be the fifth most common reason for primary care visits (following checkups, upper respiratory illnesses, back pain, and skin rashes). Loss of productivity due to headache is also substantial with an estimated cost of billions of dollars.
The International Headache Society classifies headache as primary and secondary. Primary headaches account for at least 90% of all headaches and have benign outcomes. Primary headaches include migraine with or without aura, tension type headache, and less commonly, cluster headache. Some patients with a history of primary headaches have significant risk factors for developing secondary headaches. This chapter focuses on the diagnostic approach for the patient with headache in the hospital, and the reader is referred to subsequent chapters for specific management.
Headache occurrence in the hospital may be the following:
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Secondary headaches may be either one of the presenting symptoms leading to admission or acquired secondary to a diagnostic or therapeutic intervention. Rarely, the initial presentation of a primary headache syndrome may occur in the hospital. For patients with preexisting headache syndromes, it is important to recognize that there may be significant drug interactions between medications used to treat chronic headache and those used to treat systemic disease, and there are also important contraindications to commonly used migraine medications that may limit the safety of these drugs in hospitalized patients.
Primary Headache
Elucidating the cause of a headache, particularly when severe, requires an understanding of the pathophysiology of the major headache types and recognizing the classic types of pain syndromes (and associated symptoms) they produce. Primary headaches should not cause focal neurologic signs and symptoms, except sometimes briefly during the aura phase of a complex migraine.
In general, migraine causes episodic severe headache pain associated with nausea, photophobia, and photophobia insensitivities to external stimuli. This disorder is typified as much by nausea and photophobia as it is by pain. Because of the severity of pain associated with migraines, it is the most common headache that leads patients to medical attention (even though it is not nearly as common as tension headaches). Environmental or physiologic stimuli trigger recurrent and stereotyped headache spells that may be associated with meningeal symptoms and signs. Many patients will describe an aura or warning beforehand. A history of recurrent headaches, similar in severity and character occurring with weather changes, the menstrual cycle, stress, sleep deprivation, excessive sleep, withdrawal from caffeine, or associated with ingestion of certain types of food are often migrainous headaches (Table 87-1). Migraine runs in families and can be associated with mitral valve prolapse. The precipitating etiology is probably electrical, much like seizures, rather than vasoconstriction followed by vasodilatation as previously thought.
Migraine without aura
Migraine with aura
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In general, the most important question to answer when considering migraine is whether the patient has ever had a headache like this before. The classic question about the severity of headache—the worst headache ever—does not help distinguish between primary and secondary headache because every migraine sufferer will have the worst migraine of her life at some point, and the most severe headaches are more likely to trigger medical consultation. The evolution of symptoms may help distinguish migraine from other causes of neurologic deficit. Classically migraines begin mildly, following an aura, and worsen over minutes to hours to reach a pinnacle of pain. Patients can often predict when they are about to get a headache as they begin to feel ill or have mild photophobia as the symptoms begin to worsen over time. The pain is not maximal at onset.
Migraine
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At the bedside patients will often exhibit significant photophobia and complain of nausea, although not always with vomiting. They will often have some neck stiffness. While it is true that migraines can occasionally present with focal neurological findings (termed complex migraines when such focal findings exist), it is best to assume that patients with severe headache and focal neurologic findings have something more ominous until proven otherwise.
These are usually due to tension or a spasm within the pain-sensitive muscles of the neck or temples. Tension headaches are a muscular pain syndrome similar to a strained muscle in any other portion of the body (Table 87-2).
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Patients with muscle contraction pain due to tight muscles in the neck, tension from grinding the jaw, or chronic stress can have a different history and physical than any other headache type. In general, tension headaches or muscle contraction headaches do not have associated photophobia, phonophobia, nausea, or vomiting. They tend to be unilateral or bilateral aching pain and worsen with stress. They are usually not disabling.
Tension headache
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Cluster headaches abruptly reach maximum intensity on one side of the head, last 1 to 2 hours, and have associated ipsilateral autonomic signs such as tearing, miosis, ptosis, or rhinorrhea. Patients should not have focal neurologic signs or symptoms (Table 87-3).
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Secondary Headache
While the vast majority of headache pain may have benign causes, a small percentage may be “sentinel events” heralding dangerous and life-threatening sequelae. Secondary headaches result from underlying diseases that require further evaluation and treatment on an emergent basis (Table 87-4).
Disorder | Headache | Exam | Comment |
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Aneurysmal SAH ↑ risk factors: autosomal dominant polycystic kidney disease, coarctation of aorta, connective tissue disorders, neurofibromatosis, pituitary tumors, AVM, sympathomimetic drugs including cocaine SAH from stroke ↑ risk factors: stroke risk factors, cocaine 10% of strokes are associated with SAH | Prodrome of headache due to either a small, warning (sentinel) bleed or to aneurismal dilation before rupture; unilateral in 30% Rupture when patient active, unusually severe or atypical H/A, especially if brief LOC, N/V, meningismus, or any focal findings Bleed → acute rise in intracranial pressure | Cushing sign (↑ BP, bradycardia, ↑ ICP); fever; nuchal rigidity, focal neurologic signs including ptosis of one eyelid with dilated, nonreactive pupil and impaired adduction and vertical movement of affected eye Tersen syndrome: preretinal hemorrhages and SAH | ECG changes, elevation of cardiac enzymes due to release of catecholamines; neurogenic pulmonary edema; hyponatremia probably mediated by hypothalamic injury |
Intracerebral hemorrhage Risk factors: HBP, cerebral amyloid, stroke risk factors 10% of all strokes are associated with intracerebral hemorrhage | Usually no prodrome, typically H/A when patient active, maximum deficit minutes to hours | AVM rupture commonly associated with seizure; fever | Typically hemorrhage into subcortical regions (putamen, thalamus, caudate) or if AVM, in that location |
Rupture of bacterial abscess | Severe, sudden pain, LOC | Fever, nuchal rigidity | Acute ventriculitis and meningitis |
Raised ICP of any etiology | Unilateral or bilateral, a boring, aching pain typically without photophobia or phonophobia or environmental triggers; worse lying down; nocturnal awakening | Cushing sign; abducens paresis; papilledema may be present When SAH causes ↑ ICP, blood may extravasate into retina (subhyaloid hemorrhage); can be bilateral; nuchal rigidity; seizures; may have subtle focal signs | CN VI dysfunction “false localizing sign” as refers to diffuse ↑ ICP rather than localized to 6th CN or nucleus pathology |
Meningitis | Often a throbbing component of the headache, hypersensitivity to sound and light, nausea and sometimes vomiting | Fever, photophobia, nuchal rigidity, obtundation | |
Giant cell arteritis | Severe throbbing but also dull, sharp or burning headaches, localized to the temples (50%) or diffuse, acute onset | Usually associated with painful, stiff proximal extremities, myalgias, weight loss, and other systemic symptoms, jaw claudication, and visual disturbances (sudden monocular blinds or stuttering, visual loss, amaurosis fugax, diplopia, or field cut) | Asymmetric temporal arterial pulses, thickened or tender temporal arteries may be a clue |
Trauma: Status post (s/p) neck injuries with muscle spasm Dissection of great vessels Subdural hematoma Intracranial hemorrhage | Tension-type or occipital headache | Neck or upper shoulder pain and palpation of affected muscles will exacerbate the pain syndrome; reduced range of motion of the neck LOC, focal signs should prompt further evaluation | Seemingly minor trauma may however be associated with dissection and subdural hematoma; Elderly patients receiving anticoagulants may suffer intracranial hemorrhage without severe headache |
Carbon monoxide poisoning (or inhaled methyl chlorine) |