Headache/Temporal Arteritis Case File

Headache/Temporal Arteritis Case File

Eugene C. Toy, MD, Gabriel M. Aisenberg, MD

Case 38

A 59-year-old woman comes to your clinic because she is concerned that she might have a brain tumor. She reports a severe headache for the last 3 weeks that she rates as an 8 on a scale of 0-10. She localizes the pain to the right side of her head and reports that it is constant, occasionally throbbing, but mostly a dull ache. She thinks the pain is worse at night, especially when she lies with that side of her head on the pillow. She also has some pain with chewing her food. She denies any nausea, vomiting, photophobia, or other visual disturbances. She has had headaches before, but they were mostly occipital and frontal, relieved by acetaminophen, and she attributed them to “stress.” Her medical history is significant for hypertension, which is controlled with hydrochlorothiazide, and “arthritis” of her neck, shoulders, and hips, for which she takes ibuprofen for when she feels stiff and achy. On physical examination, her temperature is 100.4 °F, heart rate is 88 beats per minute (bpm), blood pressure is 126/75 mm Hg, and respiratory rate is 12 breaths per minute. Her visual acuity is normal, visual fields are intact, and her fundoscopic examination is significant for arteriolar narrowing but no papilledema or hemorrhage. She has moderate tenderness over the right side of her head but no obvious scalp lesions. Her chest is clear, and her heart rhythm is regular, with normal S1 and S2 but an S4 gallop. Abdominal examination is benign. She has no focal deficits on neurologic examination. She has no joint swelling or deformity but is tender to palpation over her shoulders, hips, and thighs.

▶ What is the most likely diagnosis?

▶ What is the best next step to confirm the diagnosis?

ANSWERS TO CASE 38:

Headache/Temporal Arteritis

Summary: A 59-year-old woman presents with

• A 3-week history of severe right-sided headaches that are worse at night, especially when she lies with that side of her head on the pillow
• History of hypertension and “arthritis” of her neck, shoulders, and hips
• Temperature of 100.4 °F and normal neurologic and eye examinations
• Moderate tenderness over the right side of her head but no obvious scalp lesions
• Pain with chewing food

Most likely diagnosis: Giant cell (temporal) arteritis (GCA).

Best next diagnostic step: Erythrocyte sedimentation rate (ESR).

ANALYSIS

Objectives

1. Describe the clinical features that help to distinguish a benign headache from one representing a serious underlying illness. (EPA 2, 10)
2. Describe the clinical features and diagnostic tests for GCA. (EPA 1, 3)
3. Describe the clinical features of subarachnoid hemorrhage, migraine headache, cluster headache, and tension headache. (EPA 1, 10)

Considerations

Although headaches are a very common complaint, this patient has features that are of greater concern: older age of onset, abrupt onset, severe intensity, and dissimilarity to previous milder headaches. The mnemonic SNOOP can be used as a reminder of red flags of significant underlying pathology and is outlined in Table 38–1. This patient is very concerned that the headaches may indicate a brain tumor. She has no meningeal signs, and her neurologic examination is nonfocal. She has stiffness and achiness of the shoulder and hip girdles. Together, these factors make the diagnosis of GCA a strong possibility. GCA usually has its onset in patients age 50 or older and occurs in females more often than males. GCA involves inflammation of the medium- or large-size vessels. Her low-grade fever and generalized body aches may represent polymyalgia rheumatica, which is often associated with GCA. Other associated symptoms include jaw claudication and transient vision loss. Although GCA is not a common cause of headache, untreated patients can develop permanent vision loss resulting from ophthalmic artery involvement. Thus, a low index of suspicion is sufficient to begin investigation. The diagnosis could be suggested by an elevated ESR but can only be confirmed by temporal artery biopsy (ESR is a nonspecific marker of inflammation). However, a negative biopsy does not entirely rule out the diagnosis of GCA. Based on the sampled area of the biopsy and due to the discontinuous involvement of arterial pathology, multiple biopsies may be necessary to adequately assess the artery for disease. Indeed, if clinical suspicion is high, high-dose corticosteroids should be administered before the diagnosis is established to prevent irreversible complications.

APPROACH TO:

Headache

DEFINITIONS

BERRY ANEURYSM: A small, thin-walled protrusion from the intracranial arteries that has a “berry” appearance on angiographic imaging. They classically occur at the junction where a cerebral artery departs from the circle of Willis. Over time, increased pressure at the aneurysm can compromise the endothelial lining and predispose a rupture to occur, leading to subarachnoid hemorrhage.

C-REACTIVE PROTEIN (CRP): An acute-phase protein of hepatic origin whose concentrations in blood plasma increase in response to inflammation.

ERYTHROCYTE SEDIMENTATION RATE (ESR): The rate at which erythrocytes suspended in plasma fall when placed in a vertical tube. It is an indirect measure of acute-phase response (ie, inflammation).

GIANT CELL ARTERITIS (GCA): Also known as temporal arteritis; a form of systemic vascular inflammation most commonly affecting patients older than 50 years. Medium- and large-size vessels, especially the superficial temporal artery, are affected.

CLINICAL APPROACH

Pathophysiology

Headache is one of the most common complaints of patients in medical practice. As with many common symptoms, a broad range of conditions, from trivial to life threatening, might be responsible. About 90% of all headaches fall under a few main categories of what are called “primary headaches”: tension, migraine, and cluster headaches. Headache symptoms usually are accompanied by a shortage of associated laboratory findings, leaving the clinician to depend largely on a thorough history with focused neurologic examination as the initial workup. Careful history and physical examination, keeping in mind the red flags of headaches (see Table 38–1), will serve the clinician well. Differentiating serious underlying causes of headache from benign causes may be difficult. Table 38–2 lists some typical features of different causes of headache.

Adapted with permission, from Braunwald E, Fauci AS, Kasper KL, et al. Harrison’s Principles of Internal Medicine. 16th ed. 2005. Copyright © McGraw Hill LLC. All rights reserved.

Subarachnoid Hemorrhage

One of the most catastrophic causes of headache is subarachnoid hemorrhage, usually secondary to a ruptured intracerebral (berry) aneurysm. A study of alert and oriented patients presenting to the emergency center with a sudden-onset headache peaking in severity within 1 hour revealed 6% of patients had a subarachnoid hemorrhage. The initial hemorrhage may be fatal, result in severe neurologic impairment, or produce only minor symptoms such as headache. Neurologic findings may not be present initially, and the patient who will benefit the most from intervention will often have mild symptoms; thus, it is important to diagnose subarachnoid hemorrhage early if it is suspected.

The best first diagnostic study is a noncontrast computed tomographic (CT) scan with thin imaging cuts at the brain base. If done within the first 6 hours of symptom onset, this diagnostic test will have nearly 100% sensitivity for subarachnoid hemorrhage, which will then progressively decline over time. If hemorrhage is suspected but the CT is negative, lumbar puncture should be performed as soon as possible to assess for the presence of red cells or xanthochromia (yellowish discoloration of cerebrospinal fluid [CSF]). This finding indicates the presence of bilirubin in the CSF from the breakdown of hemoglobin released from lysed red blood cells and thus differentiates subarachnoid hemorrhage from a traumatic lumbar puncture.

Giant Cell Arteritis and Polymyalgia Rheumatica

GCA, also known as temporal arteritis, is a chronic vasculitis of large- and medium-size vessels usually involving the cranial branches of the arteries arising from the aortic arch. Suggestive signs and symptoms include new headache, abrupt onset of visual disturbances, jaw claudication, fever, anemia, or elevated ESR and/or CRP in a patient aged 50 years or older. GCA is closely related to polymyalgia rheumatica, an inflammatory condition characterized by bilateral aching and stiffness of the neck, torso, shoulders, or thighs, with a significantly elevated ESR. Both conditions probably are polygenic diseases in which various environmental and genetic factors influence susceptibility and severity. The most worrisome complication of GCA is permanent vision loss, which can occur as an early manifestation in up to 20% of patients. Temporal artery biopsy is recommended in all patients suspected of having GCA. Long segments (1-2 cm) of the artery may require excision to find the typical areas of segmental inflammation.

Corticosteroids are the drugs of choice to treat both polymyalgia rheumatica and GCA, with daily doses of 10 to 20 mg of prednisone for polymyalgia rheumatica and 40 to 60 mg for GCA. Steroids may prevent, but usually do not reverse, visual loss. Steroid dosage should be gradually tapered when discontinuing therapy; however, relapse is common and should be suspected if symptoms return. It is also important to consider complications of corticosteroid therapy, including hyperglycemia, bone loss, and neutropenia.

Migraine Headache

Migraine headache is the most common cause of clinic visits for headache because of its frequency, disabling qualities, and associated symptoms. Migraine headache is more common in women, and there may be a positive family history. Migraine headaches present with a throbbing or pulsatile quality, are usually unilateral (although bilateral presentation does not exclude migraine), and may have a preceding aura. Aura will typically present with temporary visual symptoms like bright shapes but can also present with auditory or somatosensory symptoms. Other associated symptoms include nausea, vomiting, or sensitivity to light or sound. Migraines can also present with tearing or nasal congestion, which may be confused with sinus infection. Treatment of acute episodes involves the initial use of nonsteroidal anti-inflammatory drugs (NSAIDs), followed by triptans if symptoms persist. Preventive therapies include tricyclic antidepressants, beta-blockers, or anticonvulsants such as valproate or topiramate.

Cluster and Tension Headaches

Episodic cluster headache is much less common but is more easily diagnosed by its distinctive pattern. Cluster headaches are more common in men and are characterized by periodic attacks of intense, unilateral, periorbital pain with nasal or ocular watering lasting minutes to hours but recurring daily over several weeks or months. Acute attacks can be treated with oxygen or subcutaneous sumatriptan. Verapamil is used for preventive therapy.

Tension headache is the most common type of headache. It is classically described as a bilateral “band-like” headache that is typically nonthrobbing and does not usually have other associated symptoms like photophobia or phonophobia. Treatment includes NSAIDs or acetaminophen and identifying and preventing headache triggers.

CASE CORRELATION

• See also Case 36 (Transient Ischemic Attack), Case 37 (Alzheimer Disease/Dementia), and Case 43 (Meningitis, Bacterial).

COMPREHENSION QUESTIONS

Match the headache type (A-E) to the clinical presentation described in

Questions 38.1 to 38.3.

A. Migraine headache

B. Tension headache

C. Cluster headache

D. Subarachnoid hemorrhage

E. Meningitis

38.1 A 42-year-old man with polycystic kidney disease who complained of a sudden onset of severe headache and then lost consciousness

38.2 A 22-year-old college student with fever, headache, photophobia, and CSF with 25 white blood cells per high-power field, but no red blood cells or xanthochromia

38.3 A 31-year-old woman with a long history of intermittent severe unilateral throbbing headache lasting hours to days associated with nausea and photophobia, but no preceding symptoms and no visual disturbance, occurring once or twice per month

38.4 A 66-year-old man is taken to the emergency department due to new-onset seizures. He reports a severe headache over the past month and morning nausea and vomiting. He also has had some difficulty with walking and balance. On CT imaging, there is a 4-cm mass noted in the right parietal lobe of the brain. Which of the following is the most likely cell type of this brain mass?

A. Glioblastoma multiforme (GBM)

B. Lymphoma

C. Schwannoma

D. Medulloblastoma

ANSWERS

38.1 D. The sudden onset of severe headache with diminution in level of consciousness is classic for subarachnoid hemorrhage. This patient likely had rupture of a cerebral artery aneurysm, which is associated with polycystic kidney disease.

38.2 E. The presence of white blood cells but no red blood cells in the CSF is indicative of meningeal inflammation, likely due to viral or bacterial infection. This patient would likely be admitted for IV antibiotics pending CSF culture results.

38.3 A. The patient’s history is strongly suggestive of migraine, given its unilateral and throbbing character, and the associated symptoms of nausea or photophobia. Most patients with disabling headache have migraine. Tension headache should have none of these features.

38.4 A. The most common primary brain cancer in adults is GBM. The prognosis unfortunately is not favorable, and a majority of individuals affected die within 18 months. Remember that the histology of GBM shows necrosis and pseudopallisading of cells. Most of these lesions are in the cerebrum (as opposed to in children, where primary lesions are usually in the posterior fossa). Answer B (lymphoma) is usually in HIV-positive patients or others with cell-mediated immunodeficiency. Answer C (schwannoma) is a benign tumor usually at the cerebellopontine angle and often affecting cranial nerve VIII: hearing difficulty and tinnitus. Answer D (medulloblastoma) is of the posterior fossa and usually affects children.

CLINICAL PEARLS

▶ Temporal arteritis usually involves one or more branches of the carotid artery and almost always occurs in patients older than 50 years. Diagnosis is suggested by an elevated ESR and confirmed by temporal artery biopsy.

▶ Visual loss is a common complication of temporal arteritis and can be prevented by initiation of high-dose corticosteroids when the diagnosis is suspected.

▶ Subarachnoid hemorrhage typically presents as a sudden onset of severe headache and is diagnosed by visualization of blood on a CT scan or by finding red blood cells or xanthochromic fluid on a lumbar puncture.

▶ Migraine is the most common type of headache for which patients seek medical attention in a clinic setting. It is an episodic headache with associated features such as nausea or photophobia.

REFERENCES

Edlow J, Caplan L. Avoiding pitfalls in the diagnosis of subarachnoid hemorrhage. N Engl J Med. 2000;342:29-36. 

Goadsby PJ, Raskin NH. Migraine and other primary headache disorders. In: Jameson JL, Fauci AS, Hauser SL, et al. Harrison’s Principles of Internal Medicine. 19th ed. New York, NY: McGraw Hill; 2018:2587-2598. 

Kaniecki R. Headache assessment and management. JAMA. 2003;289:1430-1433. 

Perry JJ, Stiell IG, Sivilotti ML, et al. Clinical decision rules to rule out subarachnoid hemorrhage for acute headache. JAMA. 2013;310(12);1248-1255. 

Salvarani C, Cantini F, Boiardi L, et al. Polymyalgia rheumatica and giant cell arteritis. N Engl J Med. 2002;347:261-278. 

Sidman R, Connolly E, Lemke T. Subarachnoid hemorrhage diagnosis: lumbar puncture is still needed when the computed tomography scan is normal. Academic Emerg Med. 1996;3(9):827-831. 

Snow V, Weiss K, Wall E, et al. Pharmacologic management of acute attacks of migraine and prevention of migraine headache. Ann Intern Med. 2002;137:840-852.

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