condition typically present with a history of months to years of chronic orbital pain. Inflammation of the trochlea causes localized pain, swelling, and tenderness, and there may be associated limitation of eye movement. When there is no obvious orbital inflammation, the diagnosis can be made by palpation in the area of the involved trochlea, which elicits pain. The etiology is usually idiopathic, but trochleitis can be associated with systemic autoimmune diseases. This condition primarily occurs in women (90%). Treatment includes local injection of corticosteroids in the region of the trochlea or high doses of NSAIDs.

Friedman DI, Gordon LK, Quiros PA. Headache attributable to disorders of the eye. Curr Pain Headache Rep. 2010;14(1):62– 72.

Harooni H, Golnik KC, Geddie B, Eggenberger ER, Lee AG. Diagnostic yield for neuroimaging in patients with unilateral eye or facial pain. Can J Ophthalmol. 2005;40(6):759–763.

Levin LA, Lessell S. Pain: a neuro-ophthalmic perspective. Arch Ophthalmol. 2003;121(11):1633.

Photophobia

Photophobia occurs most frequently as a result of ocular inflammatory disorders, including keratitis, uveitis (particularly iritis), and less commonly from chorioretinitis, retinal degenerative disorders, and lesions along the course of the anterior or retrochiasmal visual pathway. Photophobia may also occur because of meningeal irritation (eg, meningitis, subarachnoid hemorrhage) or migraine and is commonly described in patients with traumatic brain injury.

Facial Pain

Pain associated with ischemia, such as carotid dissection or microvascular cranial nerve palsy, often localizes around the ipsilateral eye. Pain in the eye area is most often a manifestation of a headache. Occipital neuralgia produces pain and tenderness over the greater occipital nerve that radiates to the ipsilateral eye area.

Patients may refer localized facial pain to the eye. Common sources of facial pain include dental disorders and sinus disease. Other facial pain syndromes include trigeminal neuralgia, glossopharyngeal neuralgia, temporomandibular joint (TMJ) syndrome, carotidynia, and herpes zoster neuralgia. The onset of facial pain in an elderly patient raises the possibility of giant cell arteritis. Facial pain is occasionally a sign of nasopharyngeal carcinoma or metastatic carcinoma affecting the trigeminal nerve or the dura at the base of the brain. In some patients with constant facial pain that is typically deep and boring, no etiology may be identified (sometimes referred to as atypical facial pain). Treatment may be difficult and usually requires use of a combination of anticonvulsant and antidepressant drugs.

Trigeminal Neuralgia

Trigeminal neuralgia, also known as tic douloureux, typically occurs during middle age or later. In 80%–90% of cases, it is caused by vascular compression of CN V, although a few reports describe trigeminal neuralgia from demyelinating disease or a posterior fossa mass lesion. The pain is almost always unilateral (95%) and usually involves the maxillary or mandibular distribution of CN V; involvement of the ophthalmic division alone is rare (<5%). Chewing, tooth brushing, or a cold wind

may precipitate paroxysmal burning or electric shock–like jabs, lasting seconds to minutes. There may be periods of remission. Sensory function in the face should be normal on testing; any abnormality increases likelihood of a neoplasm. All patients should have neuroimaging of the posterior fossa, preferably with MRI. Treatment options include use of the medications gabapentin, pregabalin, carbamazepine, phenytoin, baclofen, clonazepam, and valproic acid; selective destruction of trigeminal fibers (rhizotomy); or surgical decompression of CN V in the posterior fossa.

Glossopharyngeal Neuralgia

In glossopharyngeal neuralgia, paroxysmal pain occurs unilaterally in the region of the larynx, tongue, tonsil, and ear. Hoarseness and coughing may be present. Pain can be triggered by swallowing and pungent flavors. This condition is treated with the same medications used for treating trigeminal neuralgia and may also be alleviated by microvascular decompression. Carotidynia—pain arising from the cervical carotid artery—is typically neck pain that radiates to the ipsilateral face and ear. Carotid dissection must be excluded.

Occipital Neuralgia

Paroxysmal stabbing pain in the distribution of the greater or lesser occipital nerves may be confused with other causes of head and facial pain. Tenderness may be elicited with pressure over the affected nerve. Injection of local anesthetic drugs relieves the pain and is helpful in confirming the diagnosis.

Temporomandibular Disease

Pain from the temporomandibular area may arise from either the joint or the muscle. Joint pain exacerbated by chewing or talking suggests joint disease. A click or pop with limited jaw opening may be present. Pain from the muscle is more difficult to diagnose, as it may be referred to the ear, preauricular area, or neck. Jaw pain or claudication in an elderly patient may be an early symptom of giant cell arteritis.

Carotid Dissection

Carotid dissection typically produces pain localized to the face (see Chapter 14). It is often accompanied by sympathetic dysfunction (Horner syndrome) due to involvement of the sympathetic fibers in the wall of the carotid artery (see Chapter 10, Fig 10-4).

Herpes Zoster Ophthalmicus

When herpes zoster involves the trigeminal dermatomes, pain may arise in the affected region days before a vesicular eruption appears (Fig 12-3). The pain is described as aching or burning. Occasionally, no vesicles are apparent (zoster sine herpete). Acutely, the pain may be exacerbated by concomitant iritis. The pain may persist long after resolution of the acute infection (postherpetic neuralgia), and it can be extremely discomforting and difficult to treat. Pregabalin, gabapentin, tricyclic antidepressants, and topical lidocaine patch 5% may be effective for some patients. There is

evidence that treatment with antiviral drugs during the acute phase may decrease the risk of severe postherpetic neuralgia. The zoster vaccine, offered to immunocompetent persons aged 60 years and older, significantly reduces the incidence of herpes zoster and markedly decreases the incidence and morbidity of postherpetic neuralgia.

Figure 12-3 Herpes zoster ophthalmicus. This 63-year-old woman developed left-sided scalp pain and a rash in the V1

distribution on the left side. (Courtesy of Rod Foroozan, MD.)

Oxman MN, Levin MJ, Johnson GR, et al; Shingles Prevention Study Group. A vaccine to prevent herpes zoster and postherpetic neuralgia in older adults. N Engl J Med. 2005;352(22):2271–2284.

Neoplastic Processes

Pain associated with facial numbness raises the possibility of pathologic involvement of the trigeminal nerve, such as neoplastic processes that affect the nerve in the area of the cavernous sinus and the Meckel cave. Rapidly growing tumors, such as aggressive undifferentiated malignancies, may produce pain in a large percentage of patients. Facial cutaneous malignancy, the prior treatment of which is often not recalled by the patient because it occurred years previously, may be associated with perineural invasion and cause progressive pain, numbness, and multiple cranial nerve palsies.

Mental Nerve Neuropathy

An additional form of facial pain associated with numbness is the syndrome of the “numb chin,” which most frequently accompanies an inflammatory or a neoplastic process. Sarcoidosis has been associated with a painfully numb chin. The most common neoplastic processes are lymphoma and

metastatic breast carcinoma. Rarer causes include osteosarcoma, fibrosarcoma, plasmacytoma, metastatic lung and prostate carcinoma, collagen vascular diseases, trauma, periodontal disease, and sickle cell disease.

CHAPTER 13

The Patient With Nonorganic

Ophthalmic Disorders

Complaints of visual symptoms that have no physiologic or organic basis are called nonorganic disorders. There are 4 categories of involvement:

1.the afferent visual pathway (visual acuity, visual field)

2.ocular motility and alignment

3.pupils and accommodation

4.eyelid position and function

Malingering is willful feigning or exaggeration of symptoms. Litigation involving monetary compensation or disability status is frequently an issue with these patients. Secondary psychological gain is often the underlying basis of Münchausen syndrome, in which patients intentionally induce physical damage. Hysteria is a subconscious expression of nonorganic signs or symptoms, and patients with hysteria are often unconcerned about their incapacitating symptoms (exhibiting “la belle indifference”). Malingering cannot always be clearly differentiated from hysteria; both are tested using the same techniques to determine the validity of the symptoms. The terms functional, nonphysiologic, and nonorganic are preferable in describing this disease spectrum. It is essential to remember that a substantial proportion of patients with actual organic disease may also exhibit superimposed nonorganic behavior or may exaggerate an organic vision disturbance (socalled nonorganic overlay). These patients may be very challenging and time-consuming, as the clinician must ensure that the organic portion of disease is properly identified and treated. This chapter discusses the most frequently used clinical techniques for assessing and identifying nonorganic loss of vision.

The first step in identifying the patient with nonorganic complaints is to have a high index of suspicion when the pattern of vision loss does not fit the common sequence of known diseases. For example, trivial external trauma to the eye should not cause long-term disabling vision loss. Potential secondary gain factors may become evident during the history. Some patients may be more focused on impending litigation or disability determination than on the diagnosis or treatment of their complaint. Other patients who are naive, worried, and eager to convince the physician of their vision deficit tend to have a “positive” review of ophthalmic symptoms and often are suggestible during the history-