Headache and the Eye

Ula Jurkunas, MD; John W. Gittinger, JR, MD

Disclosures

Compr Ophthalmol Update. 2004;5(4) 

In This Article

Facial Pain, Oculosympathetic Pathways, Cranial Nerve V

Headache may be associated with an ipsilateral defect in oculosympathetic pathways—Horner's syndrome (HS) characterized by ptosis, miosis, and anhidrosis. The anisocoria is more apparent in dim light. The abnormal pupil is smaller, and pupillary reactions to light remain intact. Conventional pupillophar macological testing with cocaine and hydroxyamphetamine may be problematic because the ophthalmic solutions are difficult to obtain and the results are hard to interpret.[15,16,17] A few recent reports suggest that reversal of anisocoria with alpha-adrenergic agonists may be diagnostic of HS.[18] If this is shown to be a reliable test, it will greatly simplify pharmacologic diagnosis.

Causes of headache and third-order HS are listed in Table 1 . Because it can lead to stroke, internal carotid artery dissection (CAD) should always be considered in acute HS.[19,20,21] The classic presentation is cervical or facial pain radiating to the ipsilateral eye, but in 10% of cases the HS is the only sign.[22,26] Other manifestations of CAD are lightheadedness, bruits, syncope, amaurosis fugax, dysgeusia (foul taste), and cranial nerve palsies (VI, IX, XI, XII).[23] Marfan's syndrome and fibromuscular dysplasia predispose to spontaneous CAD, which is also caused by direct trauma, whiplash injury, or chiropractic cervical manipulation.[24,25] Consideration of CAD should prompt an urgent evaluation with magnetic resonance imaging and magnetic resonance angiography and a neurological consultation for possible admission for anticoagulation (Figure 1).[23,24,25] While CAD is usually benign, it may cause life-threatening thromboembolic events. Even after the resolution of CAD by imaging, the HS may persist.[22]

 Top: Magnetic resonance angiography of right carotid artery dissection 2 cm from bifurcation (on left side). Bottom: Magnetic resonance angiography of right carotid artery dissection with clot in the wall (on left side). Dissection stops below skull base. Images contributed by Philip Kousoubris, MD.

Another headache plus HS entity is cluster headache (CH)—sudden, severe, lancinating pain that often awakens the patient at night.[27,32] Cluster headache characteristically attacks men between 20 and 40 years of age, is unilateral, and when recurrent affects the same side (85%) ( Table 2 ).[1] Orbital myositis, cavernous sinus lesions, and arteriovenous malformations have been confused with CH, leading some to advocate imaging.[28,29,30]

Acute treatment consists of tapering dose of prednisone, methysergide (Sansert®, Novartis, East Hanover, NJ), sumatriptan, or dihydroergotamine. Calcium channel blockers, such as verapamil, are used for prophylaxis. Avoidance of precipitants, such as alcohol and vasodilators, is recommended. A variant of CH that is distinguished by its dramatic response to indomethacin prophylaxis is chronic paroxysmal hemicrania.[31] Facial pain syndromes not associated with HS include trigeminal neuralgia, atypical facial pain syndrome, and temporomandibular joint syndrome ( Table 3 ).[32,33,34]

Idiopathic stabbing headache resembles trigeminal neuralgia and consists of icepick-like pains in the temple and orbit and may be associated with migraine, cluster, and tension headaches. This jabs or jolts syndrome consists of transient knife-like pains lasting less than 1 minute without any structural abnormalities.

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