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lupus erythematosus and with Sjogren syndrome, but their basic pathology is not known Superior oblique myokymia is an unusual but easily identi able condition, characterized by recurrent episodes of vertical diplopia, monocular blurring of vision, and a tremulous sensation in the affected eye The affected globe is observed to make small arrhythmic torsional movements The problem is usually benign and responds to carbamazepine, but rare instances presage pontine glioma or demyelinating disease Compression of the fourth nerve by a small looped branch of the basilar artery has been suggested as the cause of the idiopathic variety, analogous to several other better documented vascular compression syndromes affecting cranial nerves This notion is supported by ndings of vascular compression on MRI reported by Yousry and colleagues Third Nerve Palsy The third nerve is commonly compressed by aneurysm, tumor, or temporal lobe herniation In a series of 206 cases of third nerve palsy collected by Wray and Taylor, neoplastic diseases accounted for 25 percent and aneurysms for 18 percent Of the neoplasms, 25 percent were parasellar meningiomas and 4 percent pituitary adenomas The palsy is usually chronic, progressive, and painless As emphasized earlier, enlargement of the pupil is a sign of extramedullary third nerve compression because of the peripheral location in the nerve of the pupilloconstrictor bers By contrast, as indicated above, infarction of the nerve in diabetics usually spares the pupil, since the damage is situated in the central portion of the nerve The oculomotor palsy that complicates diabetes (this was the cause in 11 percent of the Wray and Taylor series) develops over a few hours and is accompanied by pain, usually severe, in the forehead and around the eye The prognosis for recovery (as in other nonprogressive lesions of the oculomotor nerves) is usually good because of the potential of the nerve to regenerate Infarction of the third nerve may occur in nondiabetics as well In chronic compressive lesions of the third nerve (distal carotid, basilar, or, most commonly, posterior communicating artery aneurysm; pituitary tumor, meningioma, cholesteatoma) the pupil is almost always affected by way of dilation or reduced light response However, the chronicity of the lesion may permit aberrant nerve regeneration This is manifest by pupillary constriction on adduction of the eye or by retraction of the upper lid on downward gaze or adduction Rarely, children or young adults may have one or more attacks of ocular palsy in conjunction with an otherwise typical migraine (ophthalmoplegic migraine) The muscles (both extrinsic and intrinsic) innervated by the oculomotor or, very rarely, by the abducens nerve are affected Presumably, intense spasm of the vessels supplying these nerves or compression by edematous arteries causes a transitory ischemic paralysis Arteriograms done after the onset of the palsy usually disclose no abnormality The oculomotor palsy of migraine tends to recover; after repeated attacks, however, there may be permanent paresis
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Table 14-4 Causes of painful ophthalmoplegia Vascular Intracavernous carotid artery aneurysm Posterior communicating or posterior cerebral artery aneurysm Cavernous sinus thrombosis (septic and aseptic) Carotid-cavernous stula Diabetic oculomotor mononeuroapthy (may be multiple) Temporal arteritis Ophthalmoplegic migraine Neoplastic Pituitary adenoma Pituitary apoplexy Pericavernous meningioma Metastatic nodules to dura of cavernous sinus Giant-cell tumor of orbital bone Nasopharyngeal tumor invading cavernous sinus or orbit In ammatory and infectious Tolosa-Hunt syndrome Orbital pseudotumor Sinusitis Mucocele Herpes zoster Mucormycosis Sarcoidosis
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Painful Ophthalmoplegia, Tolosa-Hunt Syndrome, and Cavernous Sinus Syndrome (Table 14-4)
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Some of the diseases discussed above are associated with a degree of pain, often at the epicenter of an affected nerve or muscle But the development over days or longer of a painful unilateral ophthalmoplegia constitutes a special syndrome that is traceable to an aneurysm, tumor, or in ammatory and granulomatous process in
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the anterior portion of the cavernous sinus or at the adjacent superior orbital ssure The idiopathic granulomatous condition has been termed Tolosa-Hunt syndrome; a similar but more extensive process is known as orbital pseudotumor Although there is little pathologic material on which to base an understanding of these two diseases, they are both idiopathic orbital in ammations, in the case of Tolosa-Hunt syndrome involving the superior orbital ssure or the anterior cavernous sinus, and in orbital pseudotumor, in ammatory enlargement of the extraocular muscles, which often also encompasses the globe and other orbital contents Orbital pseudotumor is accompanied by injection of the conjunctiva and lid and slight proptosis; the Tolosa-Hunt syndrome lacks these features but is occasionally associated with additional signs of cavernous sinus disease, such as sensory loss in the periorbital branches of the trigeminal nerve In pseudotumor of the orbit, a single muscle or several may be involved and there is a tendency to relapse and later to involve the opposite globe Visual loss from compression of the optic nerve is a rare complication of either condition Associations with connective tissue disease have been reported, but most cases in our experience have occurred in isolation Ultrasound examination or CT scans of the orbit shows enlargement of the orbital contents in pseudotumor, mainly the muscles, similar to the ndings in thyroid ophthalmopathy The in ammatory changes of Tolosa-Hunt syndrome are limited to the superior orbital ssure and can sometimes be detected by MRI; coronal views taken after gadolinium infusion show the lesion to best advantage It should be noted, however, that sarcoidosis, lymphomatous in ltration, and a small meningioma may produce similar radiographic ndings The sedimentation rate in our patients has varied but is generally moderately elevated in reported cases, sometimes accompanied by a leukocytosis at the onset of symptoms Both the Tolosa-Hunt syndrome and orbital pseudotumor
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