Headache (Case 56)

Headache (Case 56)

Michelle Fabian MD and Jennifer Elbaum MD

Case: The patient is a 42-year-old woman with a history of hypertension and polycystic ovarian syndrome who presented to the ED complaining of headache. The headache was severe enough that she called 911 and was brought in by ambulance. She described the pain as pulsating and located in the right frontal region, extending down into her neck. She vomited twice in the ambulance. She denied fever, chills, rash, weakness, numbness, changes in vision, or recent trauma. Her medications include oral contraceptives, a “blood pressure pill,” and multivitamins. She reported a prior history of headaches but none so severe as to require medical advice. Her mother and aunt also have headaches, and there are no known neurologic diseases in her family.

Differential Diagnosis



Mass lesion

Ruptured aneurysm/SAH

Pseudotumor cerebri

Cluster headache

Temporal arteritis


Speaking Intelligently

Headache is the most prevalent neurologic symptom, and most people not do see a doctor for their headaches because usually they are mild and infrequent. Fortunately, most headaches are benign conditions and fall into the category of primary headache disorders, such as tension, migraine, and cluster headaches. However, for the patients who experience these headaches on a regular basis, even “benign” headaches can be extremely disabling. Chronic headache sufferers experience a decline in their quality of life, with absences from work and the inability to participate in social activities; comorbid depression and anxiety are common. A small percentage of patients will experience “transformed” headaches that manifest as chronic daily headaches that are very difficult to treat. The approach to headache must begin by differentiating benign headaches from those that reflect underlying pathology that may be life-threatening.


Clinical Thinking

• Since there is a very large differential diagnosis for headaches, it is helpful to think of headaches in three broad categories: acute, subacute, and chronic.

• New acute-onset headaches are concerning, as they suggest an acute event, such as an SAH or meningitis.

• Patients who report that they rarely have headaches but develop a sudden-onset severe headache should be evaluated emergently.

• Headache in the presence of neurologic signs and symptoms should immediately raise clinical suspicion for an intracranial process.

• Subacute-onset headache implies a slowly progressive process, such as a tumor or a vasculitis.

• Long-standing, recurrent headaches will usually be one of the primary headache disorders, and patients will often report that although the pain may be severe, “this is one of my typical migraines.”


• When taking a headache history, a good approach is to start with the PQRST method of pain assessment:

P = Provocation and palliation. What makes the pain better or worse? You will want to specifically inquire about sensitivity to light and sounds. What medications has the patient already tried at home?

Q = Quality. What type of pain is the patient experiencing?

R = Region and radiation. Where is the pain, and where does it travel?

S = Severity. It is helpful to have the patient use a rating scale of 1 to 10 so that you can later assess how well treatments are working.

T = Timing. When did the pain start? Did it begin suddenly or gradually? How often does it recur?

• In addition to taking a thorough history of the current headache, it is important to get a general headache history from the patient to determine if this headache is secondary to a new disease process or a manifestation of a chronic primary headache disorder.

Does the patient frequently have headaches?

Has he or she seen a physician for a headache in the past?

Is this headache similar or different in quality from his or her usual headaches?

• It is important to know the patient’s medical history as it may predispose him or her to neurologic involvement that can present as headaches. Think about patients who have a collagen vascular disease, malignancy, or immunocompromised state, as these patients are particularly vulnerable to having a potentially serious cause of the headaches.

• Some specific areas to discuss in the reviewing systems that relate to headaches include the following: neck pain/stiffness, aura, visual changes, fever/rash, focal neurologic complaints, and recent head or neck trauma.

• In patients with chronic headache, dietary and lifestyle issues should be addressed, including caffeine intake, sleep habits, menses, and consumption of foods such as red wine and chocolate.

Physical Examination

• A neurologic examination should be performed on all patients complaining of headache.

• Note whether the patient is in significant distress or whether he or she appears comfortable. Also pay attention to the level of consciousness. Patients with meningoencephalitis or SAH will often have an altered level of consciousness.

• Check for manifestations of increased intracranial pressure (ICP) by examining the fundi for papilledema.

• Assess for meningismus by bending the patient’s neck forward toward the chest and noting whether there is stiffness or resistance.

• Assess for the presence of Brudzinski sign by bending the patient’s neck forward to see if the knees bend reflexively. Similarly, check for Kernig sign by flexing the hip at 90 degrees and hyperextending the knee to see if the patient reflexively bends his or her neck forward. Both these maneuvers reflect the presence of meningeal irritation but are found in only 5% of adult patients with bacterial meningitis.

• Check the pupils for symmetry and reactivity to light. A dilated or minimally reactive pupil is an ominous sign for impending brain herniation or the presence of an aneurysm compressing the third nerve.

• Evaluate for focal signs such as weakness, numbness, and cranial nerve abnormalities.

Tests for Consideration

Lab tests: An elevated WBC count can support a suspicion of an infectious etiology. Platelet counts and coagulation studies are essential when considering the possibility of an intracerebral hemorrhage. An erythrocyte sedimentation rate (ESR) is very useful when temporal arteritis or other vasculitides are of concern.

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LP: An LP is essential when considering meningitis. The test should be performed as soon as possible, ideally before the initiation of antibiotic treatment. The CSF should be sent for cell count, protein, glucose, Gram stain, and cultures. The spectrum of the CSF results can help guide you to the etiology of the infection (bacterial, viral, or fungal) to direct proper treatment. An opening pressure will be elevated in the diagnosis of pseudotumor cerebri. An LP should also be done when there is a high suspicion of SAH, even if the CT scan is normal; in about 5% of cases, the CT scan will appear normal, but the CSF will reveal the presence of RBCs with xanthochromia.



→ CT of head: A CT of the head is the initial test of choice for diagnosing a hemorrhage. It will also be useful in detecting large structural lesions such as a tumor. Keep in mind, though, that acute strokes and small lesions may not be apparent on a CT scan.


→ Brain MRI: An MRI with contrast can be helpful for visualizing enhancement of the leptomeninges from infection or neoplasm. It will also reveal small lesions that can be missed on CT scan, including foci of metastatic disease and infection. It can also further define any lesion seen on CT scan.


→ Angiogram: Visualizing the vasculature is important when considering a vasculitis, an aneurysm, or a venous sinus thrombosis. The gold-standard conventional angiogram is often not the test of choice, as it is invasive with potential procedural complications. Instead, a CT angiogram, MR angiogram, or MR venography (MRV) will usually be sufficient with minimal risk of adverse events.

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Clinical Entities Medical Knowledge

Subarachnoid Hemorrhage

Rupture of a cerebral artery aneurysm leads to a sudden, dramatic increase in ICP. The subarachnoid space rapidly fills with blood. In severe hemorrhages, this blood may also leak into the intraventricular space or into the parenchyma. Both genetic and environmental factors predispose patients to formation of saccular aneurysms, the most common form of aneurysm. Saccular aneurysms tend to occur at arterial branch points, most commonly in the anterior communicating artery. Less common forms of aneurysms include mycotic aneurysms (formed by septic or neoplastic emboli that lodge in the arterial wall) and fusiform aneurysms.


Classically a patient with subarachnoid headache will experience an explosive, generalized headache that will often be described as “the worst headache of my life.” Associated symptoms can include neck stiffness (usually), vomiting, photophobia, confusion, and loss of consciousness. Depending on whether there is parenchymal hemorrhage, the patient may also exhibit focal motor or sensory signs. Before aneurysmal rupture, patients are usually asymptomatic. However, they may present with a third-nerve palsy, which involves the pupil; the pupil will be asymmetrically enlarged and poorly reactive to light. This is caused by direct pressure from the aneurysm, most often from the posterior communicating artery, on the third-nerve fibers. This is an important syndrome not to be missed by the clinician because identification can be lifesaving.


A noncontrast head CT scan has >90% sensitivity in SAH in the first 24 hours. If the head CT scan is negative but there is still a high suspicion for SAH, LP must be performed. LP typically reveals a high opening pressure, is grossly bloody with a very high RBC count, and is positive for xanthochromia—a yellow appearance to the supernatant.


Initially, the most important treatment is to control blood pressure to decrease the chance of repeat bleeding. Antiepileptic drugs are administered to prevent seizures. At the same time, it is imperative to quickly identify the aneurysm that caused the hemorrhage. Once the aneurysm is identified and secured by either surgical or endovascular treatment, then traditionally triple-H therapy (hypertension, hypervolemia, hemodilution) is employed to prevent vasospasm. In addition, many patients require ventriculoperitoneal (VP) shunts if hydrocephalus develops. See Cecil Essentials 119, 124.


Acute Meningitis and Encephalitis

Meningitis is an infection of the arachnoid, the pia, and the subarachnoid space. Encephalitis is an infection of the brain parenchyma. When there are symptoms that seem to indicate infection of both meninges and brain, this is termed meningoencephalitis. CNS infections produce an intense inflammatory response. This response is often more detrimental than the pathogen itself and may lead to acute and chronic complications.


The two most common treatable life-threatening types of CNS infections seen are bacterial meningitis and herpes simplex virus (HSV) encephalitis. A patient with acute bacterial meningitis will typically present with severe generalized headache and neck stiffness. Fevers, chills, nausea, and vomiting are common. Level of consciousness may range from normal to confused or comatose. On exam, meningismus is prominent, although this sign may be absent in coma. Kernig and Brudzinski signs may also be present.

HSV encephalitis may have a similar presentation. There is usually fever, headache, and alteration in mental status. Patients can also exhibit prominent symptoms not often appreciated in meningitis, including olfactory hallucinations, aphasia, recurrent partial seizure, hemiparesis, and ataxia.


The diagnosis of meningitis is made by analysis of CSF. Opening pressure will be elevated. CSF will be cloudy upon visual examination. WBC count in fluid usually ranges from 1000 to 10,000 cells/μL with a neutrophilic predominance. Protein will usually be elevated and glucose decreased. Gram stain and culture reveal the pathogen in a large percentage of patients, although the likelihood of identifying the organism depends upon the concentration of organisms in spinal fluid. Blood cultures should be done as well. Importantly, before LP is performed (particularly if there is any sign of increased ICP or focal neurologic deficit), a head CT scan should be done to rule out a mass lesion.

HSV encephalitis is also diagnosed via the CSF. There is a CSF lymphocytic pleocytosis with elevated protein and normal glucose. In some cases there are increased RBCs or xanthochromia, but this sign is not reliable. HSV PCR is highly sensitive and specific, and is used to establish the diagnosis. The most common abnormalities on MRI are characteristic changes in the temporal lobes.


Treatment with broad-spectrum antibiotics should be started immediately when bacterial meningitis is suspected. Importantly, antibiotics should not be delayed even if there is a delay in performing the LP. Once identification of an organism is made, antibiotics can then be tailored to the offending organism. In addition to antibiotics, dexamethasone (given concomitantly with or just before the first antibiotic dose) has been shown to be effective adjunctive treatment in certain subgroups of patients with bacterial meningitis.

If there is any suspicion of HSV encephalitis, acyclovir should also be given empirically until HSV PCR results are obtained. However, in some patients with HSV encephalitis, initial CSF PCR results may be negative; in these patients, a new CSF specimen for HSV PCR should be obtained 3–7 days after therapy has begun. See Cecil Essentials 97.



The etiology of migraine is not completely understood. There is certainly a genetic component, as migraine tends to run in families. Currently it is thought that migraine occurs as the result of excitability in the trigeminal nerve system, which sets off a chain reaction resulting in the release of neurochemical transmitters, including serotonin and calcitonin gene–related peptide (CGRP), resulting in vascular dilatation. At the same time, this hyperexcitability also causes a phenomenon that has been termed “spreading cortical depression” in which decreased neural activity spreads across both hemispheres from the occiput forward in a continuous pattern.


The two types of migraine most commonly encountered are migraine with aura (classic migraine) and migraine without aura (common migraine). In the patient who has migraine with aura, a stereotyped aura will precede the headache by a period of minutes. The aura commonly is a visual phenomenon of an expanding, shimmering blind spot (scintillating scotoma). In some the scotoma may morph into a colored zigzag line (fortification spectra). Interestingly, the progression of the visual aura across the visual field seems to parallel the spreading cortical depression described above. Less commonly, aura can consist of unilateral sensory or motor symptoms. The aura symptoms last between 20 and 25 minutes, and usually resolve before the headache occurs.

The headache in migraine usually increases to a maximum over 30 minutes to 2 hours. Pain is classically unilateral; however, it may also be generalized. It is throbbing in nature. Usually there is photophobia and/or phonophobia. Physical activity will exacerbate the symptoms, and thus the patient will prefer to lie down in a dark, quiet place until the headache improves, usually over hours to days. Neurologic examination should be normal except in rare cases in which focal deficits accompany migraine symptoms.


Migraine is diagnosed by history. In cases in which a person with migraine presents to the ED, it may be necessary to exclude other more dangerous entities with imaging and/or LP.


There are two major forms of migraine treatment: acute and preventative. Acute treatment should begin as soon as the headache is experienced. For mild headaches acetaminophen or an oral nonsteroidal anti-inflammatory drug (NSAID) may be effective. More severe headaches may warrant the use of a triptan. Ergotamines have also been shown to be effective in acute headache treatment. If headaches are occurring frequently, then prophylactic treatment should be considered. β-Blockers, tricyclic antidepressants, calcium channel blockers, and some antiepileptic drugs have all been shown to be effective in preventing migraines. See Cecil Essentials 119.


Mass Lesion

Brain, blood, and CSF are the three elements that reside in the finite space of the cranial vault. If a fourth element is introduced into this space, such as an intracranial mass, then the equilibrium between the preexisting elements is altered. Localized headache may be caused by distension of pain-sensitive structures. If the tumor continues to grow, there will be a rise in ICP, which may then cause generalized headache.


Patients with brain tumor will present in a variety of ways, including headache, seizure, or focal symptoms. Headaches from an intracranial mass can range from dull and constant to sharp and intermittent. Some patients will localize their headache to the approximate location of the mass; however, others will complain of generalized headaches. Patients will often describe headaches that awaken them from sleep. This is because lying in the recumbent position causes a transient increase in ICP. In the same manner other maneuvers that transiently increase ICP, such as coughing or straining, can worsen the headache. On exam there may be papilledema or focal signs referring to the mass.


An intracranial mass is most readily identified with MRI. The addition of contrast can aid in elucidating what type of mass is present. If MRI is not available, CT can be used, but it is not as sensitive or informative. Further attempts to identify the mass noninvasively can be made using either MRI–single photon emission CT (MRI-SPECT) or PET scan. However, most often a brain biopsy is necessary for definitive identification of the mass. The differential diagnosis of an intracranial mass should include brain abscess, which can have a similar presentation and appearance on imaging studies.


Treatment of the intracranial mass is dependent on the type of tumor, its location, and the patient’s clinical status. Patients who have clear signs of raised ICP are usually given corticosteroids to attempt to decrease the mass effect of the surrounding edema. In some cases, resection is attempted. Consultation with a neuro-oncologist and neurosurgeon is usually necessary. See Cecil Essentials 127.


Venous Sinus Thrombosis

Venous sinus thrombosis is the occlusion of an intracerebral venous sinus by thrombus. Sagittal, lateral, and cavernous sinuses are the most common sites for thrombosis. The thrombosis is presumably caused by a hypercoagulable state, whether that is from a genetic predisposition, medications such as oral contraceptives, an autoimmune process, the postpartum period, intracranial infection, or malignancy. Dehydration has also been implicated. Venous sinus thrombosis can lead to a host of complications such as venous infarction, seizure, and increased ICP.


Depending on the site of thrombosis, the presentation can vary. The superior sagittal sinus (SSS) is the most commonly affected sinus; patients with SSS thrombosis most often present with headache, which is usually constant and generalized, but can be located in the occipital region. Headache may be exacerbated by coughing, straining, or lying down. Patients with cavernous sinus thrombosis may also present with chemosis, exophthalmos, and ophthalmoplegia.


Head CT both with and without contrast suggests the diagnosis, although it is by no means a sensitive test for this condition. MRV is the initial test of choice when venous sinus thrombosis is suspected. Further information can be gained by the more invasive conventional angiogram, although this is not always necessary. Once the diagnosis is confirmed, the cause of thrombosis should be pursued.


Anticoagulation with either heparin or low-molecular-weight heparin is started when venous sinus thrombosis is diagnosed. Warfarin is usually titrated to therapeutic range for long-term therapy of at least 6 months and possibly longer depending on the cause. See Cecil Essentials 124.


Pseudotumor Cerebri

The cause of pseudotumor cerebri, also known as idiopathic intracranial hypertension (IIH), is not known. Theories include increased rate of CSF production, decreased rate of CSF absorption secondary to venous hypertension, slowed absorption of CSF by arachnoid villi, and increased brain volume due an increase both in intracranial blood volume and extravascular space. Pseudotumor is most commonly associated with obesity and menstrual irregularities. Other risk factors include excessive vitamin A ingestion, endocrine abnormalities, remote head trauma, and venous sinus thrombosis.


The headache in pseudotumor is most often constant and generalized; it may wake the patient up from sleep and worsens upon coughing or straining. There may be associated visual symptoms including blurring of vision, diplopia, or recurrent episodes of transient visual loss. In severe cases there may be complete visual loss. Exam can reveal papilledema and possibly retinal hemorrhage. There may be bilateral cranial nerve VI palsy. Visual fields may be constricted, and/or there may be an enlargement of the physiologic blind spot. More often, however, bedside visual testing will appear normal, and formal visual testing will have to be performed to identify abnormalities. The rest of the neurologic exam is normal.


In a patient with headache and papilledema, neuroimaging with CT or MRI must be done initially to rule out a structural cause such as a mass lesion. Depending on the clinical scenario, MRV may also be appropriate. If neuroimaging proves unrevealing, LP is performed. Normal opening pressure is <180 mm H2O in nonobese patients and <250 mm H2O in obese patients. Thus, an opening pressure above these levels is diagnostic; however, these minimum thresholds for diagnosis may well be exceeded.


On initial LP, when opening pressure is found to be elevated, a large-volume tap is performed in an attempt to decrease ICP. Acetazolamide, a carbonic anhydrase inhibitor that reduces CSF production, is typically administered. If symptoms persist, repeat LPs are performed. If this still proves unsuccessful (the patient still has symptoms or visual field testing shows worsening), either lumbar peritoneal shunt or optic nerve fenestration can be considered as surgical options for treatment. At the same time, it is imperative that the physician encourage aggressive attempts at weight loss, as this in itself may confer an improved prognosis. See Cecil Essentials 119.


Cluster Headache

The etiology of cluster headache is unknown. Some believe it is secondary to abnormal parasympathetic activity, mediated through the superior petrosal nerve and the sphenopalatine ganglion. Others have implicated the hypothalamus and its pathways because of the daily rhythmicity of attacks and their relationship to the circadian rhythm.


The headaches typically occur in “clusters,” every day at the same time for a 4- to 6-week period, before remitting for months to years, when the cycle repeats. The pain, usually localized over one eye, is deep, stabbing, and unbearable. Prominent symptoms associated with cluster headache include ipsilateral ptosis, conjunctival injection and tearing, nasal stuffiness, and facial flushing. In contrast to the migraneur who prefers to lie in a quiet, dark environment, a cluster headache patient will often pace about until the pain stops. The headache will last between 15 minutes and 2 hours. Alcohol can trigger a headache in 70% of patients; however, if the patient is not in the midst of a cycle, alcohol has no effect.


The diagnosis is made by a careful history and, if possible, observation during an attack.


During an attack, cluster headache can be treated with 100% oxygen given by non-rebreather facial mask. With this treatment, the headache will usually resolve in 15 minutes. Other acute treatments include sumatriptan and intranasal lidocaine. Ergotamine can be effective in prevention of a single attack. To halt a cluster cycle, verapamil, lithium, and corticosteroids have all been used successfully. See Cecil Essentials 119.


Temporal Arteritis

Temporal arteritis is an autoimmune vasculitic syndrome of unknown cause that affects the superficial temporal artery exclusively in the majority of cases, and less commonly affects other larger arteries. It is thought that T cells invade the internal elastic lamina initially in response to an unknown antigen. Lymphokines are then released and attract monocytes, which carry out a full-thickness arterial invasion. In foci of inflammation there is frequent granuloma and giant cell formation. With progression of the disease, there is luminal proliferation causing stenosis of the artery and distal ischemia.


Patients with temporal arteritis usually present with increasingly severe, constant unilateral pain centered over the temporal artery. The pain can be dull, throbbing, or stabbing in character. Associated symptoms can include jaw claudication (pain with chewing), fatigue, anemia, and low-grade fever. If myalgias are a prominent feature, there may be coexistent polymyalgia rheumatica. The dreaded complication of temporal arteritis is visual loss due to involvement of the central retinal artery; this can occur with a prodrome of transient episodes of amaurosis fugax, or it can occur suddenly without warning. On exam a tender, nodular, enlarged, and erythematous superficial temporal artery may be present. If vision has been affected, it may manifest in abnormal visual fields, decreased visual acuity, or blindness. Otherwise the neurologic exam is usually normal.


ESR is the initial test for temporal arteritis. While an ESR > 50 mm/hr is diagnostic, it is important to note that a normal ESR is seen in a minority of cases. In these cases, C-reactive protein (CRP) level may be more sensitive and should be checked. Temporal artery biopsy should then be performed for confirmation.


The treatment for patients diagnosed with temporal arteritis is prednisone, usually starting at a dose of 40–60 mg daily. It should be started immediately, even before biopsy, especially in cases where impending visual loss is a concern. Patients may require a very slow taper over months, as symptoms may recur with a decrease in prednisone dose. See Cecil Essentials 86, 119.


Oct 3, 2016 | Posted by in MANUAL THERAPIST | Comments Off on Headache (Case 56)
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