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ophthalmologists, can be found in the monographs of Leigh and Zee and of Glaser listed with the References Other Forms of Diplopia The red-glass and other similar tests are most useful when a single muscle is responsible for the diplopia If testing suggests that more than one muscle is involved, one must be certain not to overlook myasthenia gravis and thyroid ophthalmopathy, which affect several muscles of ocular motility Monocular diplopia occurs most commonly in relation to diseases of the cornea and lens rather than the retina; usually the images are overlapping or superimposed rather than discrete In most cases, monocular diplopia can be traced to a lenticular distortion or displacement, but in some no abnormality can be found Monocular diplopia has been reported in association with cerebral disease (Safran et al), but this must be a very rare occurrence Occasionally patients with homonymous scotomas due to a lesion of the occipital lobe will see multiple images (polyopia) in the defective eld of vision, particularly when the target is moving Rarely, the acute onset of convergence paralysis may give rise to diplopia and blurred vision at all near points; most cases are due to head injury, some to encephalitis or multiple sclerosis Many cases of convergence paralysis do not have a demonstrable neurologic basis; they are due to hysteria or remain unexplained The acute onset of the ill-de ned entity of divergence paralysis causes diplopia at a distance because of crossing of the visual axes; in such patients images fuse only at a near position This disorder, the pathologic basis of which is unknown, is dif cult to distinguish from mild bilateral sixth nerve palsies and from convergence spasm, which is common in malingerers and hysterics A special type of divergence paralysis is seen not uncommonly in patients with strokes in the rostral midbrain; they display an asymmetrical incompleteness of ocular abduction on both sides ( pseudo-sixth palsy ) Based on scant clinical data, a center for active divergence has been postulated to reside in the rostral midbrain tegmentum Causes of Third, Fourth, and Sixth Nerve Palsies (Table 14-3) Ocular palsies may have a central cause ie, due to a lesion of the nucleus or the intramedullary (fascicular) portion of the cranial nerve or they may be peripheral Weakness of ocular muscles due to a lesion in the brainstem is usually accompanied by involvement of other cranial nerves and by signs referable to the crossed brainstem syndromes of a cranial nerve palsy on one side and a hemiparesis on the opposite side (Table 34-3) Peripheral lesions, which may or may not be solitary, have a great variety of causes Rucker (1958, 1966), who analyzed 2000 cases of paralysis of the oculomotor nerves, found that the most common causes were tumors at the base of the brain (primary, metastatic, meningeal carcinomatosis), head trauma, ischemic infarction of a nerve (generally associated with diabetes), and aneurysms of the circle of Willis, in that order The sixth nerve was affected in about half of the cases; third nerve palsies were about half as common as those of the sixth nerve; and the fourth nerve was involved in less than 10 percent In a series of 1000 unselected cases reported subsequently by Rush and Younge, trauma was a more frequent cause than neoplasm and the frequency of aneurysm-related cases was fewer than in the aforementioned series; otherwise the ndings were little changed from those of the earlier studies Less common causes of paralysis of the oculomotor nerves include variants of GuillainBarre syndrome, herpes zoster, giant-cell arteritis, ophthalmoplegic migraine, carcinomatous or lymphomatous meningitis, and sarcoidosis as well as fungal, tuberculous, syphilitic, and other chronic.

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forms of meningitis Myasthenia gravis must always be considered in cases of ocular muscle palsy, particularly if several muscles are involved and if ptosis is a prominent feature Thyroid ophthalmopathy, discussed further on, presents in the similar fashion but without ptosis and is less common than myasthenia Actually, in the single largest group (20 to 30 percent) in each of the above series, no cause could be assigned, although more cases are now being resolved with magnetic resonance imaging (MRI) and newly available serologic tests of the cerebrospinal uid (CSF) Fortunately, in many cases of undetermined cause, the palsy disappears in a few weeks or months Sixth Nerve Palsy Infarction of the sixth nerve is a common cause of sixth nerve palsy in diabetics, in which case there is usually pain near the outer canthus of the eye An idiopathic form that occurs in the absence of diabetes possibly atherosclerotic, arteritic, or phlebitic is also well known An isolated sixth nerve palsy with global headache, and more speci cally when the palsy is bilateral, as mentioned below, frequently proves to be caused by neoplasm In children, the most common tumor involving the sixth nerve is a pontine glioma; in adults, it is a metastatic tumor arising from the nasopharynx Thus it is essential that the nasopharynx be examined carefully in every case of unexplained sixth nerve palsy, particularly if it is accompanied by sensory symptoms on the side of the face As the abducens nerve passes near the apex of the petrous bone, it is in close relation to the trigeminal nerve Both may be implicated by petrositis, manifest by facial pain and diplopia (Gradenigo syndrome) Fractures at the base of the skull and petrous clivus tumors may have a similar effect, and sometimes head injury alone is the only assignable cause, even in the absence of a fracture (as mentioned, fourth nerve palsy is a more common complication of closed cranial injury) Unilateral or bilateral abducens weakness may be a nonspeci c sign of increased intracranial pressure from any source including brain tumor, meningitis, and pseudotumor cerebri; rarely, it may appear after lumbar puncture, epidural injections, or insertion of a ventricular shunt The type that arises with infarction of the rostral midbrain (pseudo-sixth) was described above Occasionally the nerve is compressed by a congenitally persistent trigeminal artery Fourth Nerve Palsy The fourth nerve is particularly vulnerable to head trauma (this was the cause in 43 percent of 323 cases of trochlear nerve lesions collected by Wray from the literature) The reason for this vulnerability has been speculated to be the long crossed course of the nerves A fair number of cases remain idiopathic even after careful investigation The fourth and sixth nerves are practically never involved by aneurysm This re ects the relative infrequency of carotid artery aneurysms in the infraclinoid portion of the cavernous sinus, where they could impinge on the sixth nerve (In contrast, supraclinoid aneurysms commonly involve the third nerve) Herpes zoster ophthalmicus may affect any of the oculomotor nerves but particularly the trochlear, which shares a common sheath with the ophthalmic division of the trigeminal nerve Diabetic infarction of the fourth nerve occurs, but far less frequently than infarction of the third or sixth nerves Trochlear nerve palsy may also be a false localizing sign in cases of increased intracranial pressure, but again not nearly as often as abducens palsy Entrapment of the superior oblique tendon is a rare cause (Brown syndrome) in which, in addition to diplopia, there is focal pain at the corner of the orbit; hence it may be mistaken for the Tolosa-Hunt syndrome, discussed further on Most cases are idiopathic Trochlear nerve palsies have been described in patients with.

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