Headache case file

Headache case file

Eugene C. Toy, MD, Barry C. Simon, MD, Terrence H. Liu, MD, MHP, Katrin Y. Takenaka, MD, Adam J. Rosh, MD, MS

Case 44

A 50-year-old woman presents with a severe headache of abrupt onset of 10 hours duration. The pain is diffuse, throbbing, and worsened when she went outside into the sunlight. She denies any recent fever, neck pain, numbness, weakness, vomiting, and any change in vision. She was concerned because she has never had a headache as strong as this before. Her past medical and family histories are unremarkable. She does not take any medications, does not smoke, and only drinks alcohol socially.

On examination, her temperature is 36.9°C (98.4°F), blood pressure 136/72 mm Hg, heart rate 88 beats per minute, and respiratory rate of 16 breaths per minute. She is not in any acute distress but appears to be mildly uncomfortable. Her pupils are equal and reactive bilaterally. There is no evidence of papilledema on fundoscopic examination. Movement of her neck causes her some increased discomfort. Her neurological examination is normal, including cranial nerves, strength, light touch sensation, deep-tendon reflexes, and finger-to-nose. The computed tomography (CT) scan of her head is normal.

⯈ What is the most likely diagnosis?

⯈ What is the next diagnostic step?

ANSWER TO CASE: 44

Headache

Summary: This is a 50-year-old woman with acute onset of “the worst headache of her life.”

• Most likely diagnosis: Subarachnoid hemorrhage.
• Next diagnostic step: CT scan of the head followed by lumbar puncture.

ANALYSIS

Objectives

1. Learn to differentiate emergent, urgent, and less urgent causes of headache.
2. Understand the treatment of various types of headache.

Considerations

This 50-year-old woman has an acute onset of severe headache, described as the “worse headache of her life.” The acute onset and severity of her symptoms are concerning for subarachnoid hemorrhage (SAH). Headache is a very common chief complaint in the emergency department (ED), accounting for up to 2.2% of all ED visits. When evaluating patients with headaches, the clinician’s goals are to identify those with serious or life-threatening conditions and to alleviate pain. The physical examination should screen for non-neurological causes of headache including palpation of the sinuses (looking for tenderness consistent with sinusitis), palpation of the temporal arteries (for tenderness or reduced pulsations suggestive of temporal arteritis). A thorough eye examination is important and should consist of assessment of the pupils, visual acuity, and fundoscopy. A detailed neurological examination should also be performed. This patient’s CT scan of the head is unremarkable, which does not definitively rule out subarachnoid hemorrhage. CT scan is often performed to rule out a cerebral mass before performing lumbar puncture (LP). Erythrocytes in the cerebral spinal fluid (CSF) or a xanthochromic CSF are considered diagnostic for SAH. However, xanthochromia may take up to 12 hours to appear. Therefore, a negative LP in the face of clinical suspicion for subarachnoid hemorrhage may necessitate repeat LP or other neuroimaging such as magnetic resonance imaging (MRI) or angiography.

Approach To:

Headache

CLINICAL APPROACH

Headaches can be caused by many intracranial and extracranial processes. One of the easiest ways to classify them is to separate them into primary and secondary causes. Primary headaches are most common and include migraine, tension-type, and cluster headaches. Secondary headaches are the result of some other disease process (eg, infection, tumor). Headaches can also be subdivided into critical or emergent versus nonemergent causes. Critical and emergent headaches have an etiology that mandates immediate identification and treatment (Table 44–1). In contrast, nonemergent causes are benign and do not present any immediate threat to life. This category includes primary headache syndromes and post lumbar puncture headaches. Less than 1% of patients with headache have a potentially life-threatening etiology, but identification of these patients is paramount.

When evaluating patients with headaches, the history should focus on the nature of the pain (location, severity, character, onset), any associated symptoms, and aggravating or alleviating factors. Past medical history (including history of head trauma, medications) and family history are important to identify risk factors for serious disease. A history of prior headaches and any previous diagnostic studies can also be helpful. Potentially ominous historical findings include a sudden onset, the “worst headache of life,” headaches dramatically different from past episodes, immunocompromised, new onset after age 50 years, and onset with exertion.

A complete physical examination with a detailed neurological evaluation can also help to separate the emergent from other causes. Abnormal vital signs can be a harbinger of life-threatening conditions. Other warning signs include altered mental status, abnormal fundi, meningeal signs, focal neurological deficits, and a rash suspicious for meningococcemia. Some types of headache have classic historical or examination findings that will aid in narrowing the differential (Table 44–2).

Because there is no routine workup of headaches, testing must be based on clinical suspicion of serious illness (Table 44–3). Diagnostic imaging of the head should be considered for patients with sudden-onset severe headaches, HIV-positive patients with a new headache, and headaches with new abnormal findings (eg, focal deficit, altered mental status). Management includes stabilizing any life-threatening conditions, controlling pain, and addressing any underlying disease or specific etiologies.

SUBARACHNOID HEMORRHAGE

Subarachnoid hemorrhage (SAH) has an annual incidence of 1 in 10,000 Americans. Most cases occur in patients 40 to 60 years old. The presentation can be very subtle with a normal neurological examination, little or no nuchal rigidity, normal level of

Abbreviations: CT = computed tomography; MRI = magnetic resonance imaging; DHE = dihydroergotamine; LP = lumbar

puncture; NSAIDs = nonsteroidal anti-infl ammatory drugs.

consciousness, and normal vital signs. Nevertheless, the mortality associated with SAH approaches 50%. Thus, the ED physician must maintain a high index of suspicion in a patient with acute onset of severe headache. CT scan is usually the initial imaging test. Although the newer high-resolution CTs have higher sensitivity, no imaging procedure can definitively rule out SAH. Thus, many authorities advocate performing a lumbar puncture on all patients suspected of having SAH, even in the face of a normal CT scan. The lumbar puncture revealing xanthochromic CSF is considered the gold standard of diagnosis. Because xanthochromia may take up to 12 hours to develop, persistently bloody CSF is also worrisome for SAH. Neurosurgical evaluation is important once the diagnosis of SAH is established. Nimodipine may be helpful in decreasing cerebral arterial spasm and subsequent ischemia. Angiographic evaluation is usually undertaken to assess for possible need of surgical intervention of lesions such as berry aneurysms. Prognosis generally correlates with initial neurological status.

CEREBRAL CAUSES

Viral or bacterial meningitis can cause severe headache. Lumbar puncture is the best method of assessing for these infections. Immunocompromised states such as human immunodeficiency virus (HIV) infection may cause more subtle or atypical symptoms (eg, lack of fever or meningismus). Pre-LP CT does not necessarily need to be performed for a patient without risk factors for a mass lesion (such as HIV or cancer) and a normal neurological examination, normal level of consciousness, and absence of papilledema. Stroke or transient ischemic attack (TIA) may present as headache, but usually there is a history of or continued neurological deficit. The classic presentation of a brain tumor (headache associated with nausea or vomiting, sleep disturbances) is uncommon. Thus persistent atypical headache (such as new onset after age 50 years), severe pain, or associated with even subtle cognitive or neurological function should be investigated, usually with CT.

TEMPORAL ARTERITIS

Temporal arteritis (TA) almost always occurs in patients older than age 50 years and is more common in women. It is caused by systemic arteritis, which presents as severe and throbbing headache, located over the frontotemporal region. Often, the temporal artery has a diminished pulse or is tender or is pulsating. Patients are diagnosed with TA if they fulfill three of the following criteria: age older than 50 years, new-onset localized headache, decreased pulse or tenderness over the temporal artery, an erythrocyte sedimentation rate exceeding 50 mm/h, and/or abnormal temporal artery biopsy. Vision loss is a potential complication, and immediate treatment should include prednisone 40 to 60 mg/d and urgent referral.

PRIMARY HEADACHE SYNDROMES

Migraine headaches are common. Onset usually occurs during the teenage years, and women are more often affected than men. Family history is often positive. The most common variety is migraine without aura, which is usually slow in onset, unilateral, and throbbing. Photophobia, phonophobia, nausea, and vomiting frequently accompany the pain. Patients with migraines with aura have a similar type of headache that is preceded by reversible visual phenomena (most common), paresthesias, motor deficits, or language difficulties. Treatment includes intravenous hydration if the patient is dehydrated and placing the patient in a dark, quiet room. Pharmacological options include dihydroergotamine (a nonspecific serotonin agonist), sumatriptan (a selective serotonin agonist), or dopamine antagonists such as metoclopramide, chlorpromazine, or prochlorperazine. In general, opiates are used for patients with refractory pain.

Tension headaches are extremely common. They are usually characterized by bilateral, nonpulsating, “band-like” pain around the forehead to the occiput. Nausea and vomiting are uncommon. Treatment includes acetaminophen, nonsteroidal anti inflammatory drugs (NSAIDs), and stress reduction techniques.

Cluster headaches are rarer than other types of primary headache syndromes. They occur more commonly in men and usually start after 20 years of age. Patients typically present with unilateral, severe, orbital or temporal pain, often associated with ipsilateral lacrimation, nasal congestion, rhinorrhea, miosis, and/or ptosis. The headaches tend to occur in “clusters” for several weeks and then remit for months or years. High-flow oxygen is usually effective. DHE and sumatriptan can also help relieve symptoms.

COMPREHENSION QUESTIONS

44.1 Several patients have been brought into the ED with a chief complaint of headache. Which of the following patients should be seen fi rst (ie, which is most likely to have a potentially life-threatening condition)?

A. A 52-year-old man with headache of 8-hour duration, and blood pressure of 210/120 mm Hg.

B. A 32-year-old woman with severe throbbing headache involving the right side of her head.

C. A 32-year-old woman who underwent an outpatient bilateral tubal ligation under spinal anesthesia and now complains of severe bilateral headache, especially with sitting up.

D. A 35-year-old woman with severe headache and a diagnosis given to her of pseudotumor cerebri.

44.2 A 22-year-old woman complains of headache of 2 hours duration that is described as unilateral and throbbing with nausea, photophobia, and phonophobia. Which of the following is the most likely diagnosis?

A. Cluster headache

B. Brain tumor

C. Migraine headache

D. Tension-type headache

44.3 A 34-year-old woman is brought into the ED for “the worst headache of her life.” She has some lethargy, photophobia, and nuchal rigidity. A lumbar puncture is performed after examining her eye grounds. Which of the following findings in cerebrospinal fluid is most concerning for subarachnoid hemorrhage?

A. Red blood cells

B. White blood cells

C. Elevated opening pressure

D. Xanthochromia

ANSWERS

44.1 A. The first patient is most likely to have a potentially life-threatening condition (hypertensive crisis).

44.2 C. Migraine headaches are described as unilateral and throbbing with nausea, photophobia, and phonophobia.

44.3 D. Xanthochromia in cerebrospinal fluid is most concerning for subarachnoid hemorrhage. Because it results from hemoglobin metabolism, xanthochromia may take up to 12 hours to develop.

CLINICAL PEARLS

⯈ Potentially ominous historical findings include sudden onset, “the worst headache of life,” headaches dramatically different from past episodes, immunocompromised, new onset after age 50 years, and onset with exertion.

⯈ Diagnostic testing must be based on clinical suspicion. For example, if there is a concern for a subarachnoid hemorrhage, a CT scan of the head and lumbar puncture (if CT is negative) are warranted.

⯈ In general, management includes stabilizing any life-threatening conditions, controlling pain, and addressing any underlying disease or specific etiologies.

References

American College of Emergency Physicians. Clinical policy: critical issues in the evaluation and management of adult patients presenting to the emergency department with acute headache. Ann Emerg Med. 2008;52:407-436. 

Godwin SA, Villa J. Acute headache in the ED: evidence-based evaluation and treatment options. Emerg Med Pract. 2001;3(6):1-32. 

Goldstein JN, Camargo CA Jr, Pelletier AJ, et al. Headache in United States emergency departments: demographics, work-up and frequency of pathological diagnoses. Cephalalgia. 2006;26:684-690. 

Hamilton GC, Sanders AB, Strange GR, Trott AT, eds. Emergency Medicine: An Approach to Clinical Problem-Solving. Philadelphia, PA: WB Saunders; 2003:535-551. 

Marx JA, Hockberger RS, Walls RM, eds. Rosen’s Emergency Medicine: Concepts and Clinical Practice. 7th ed. Philadelphia, PA: Mosby Elsevier; 2009. 

Mick NW, Peters JR, Silvers SM. Blueprints in Emergency Medicine. Malden, MA: Blackwell Publishing; 2002:139-142. 

Tintinalli JE, Kelen GD, Stapczynski JS, eds. Emergency Medicine: A Comprehensive Study Guide. 7th ed. New York, NY: McGraw-Hill; 2011.

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