Ординатура / Офтальмология / Английские материалы / Neuro-Ophthalmology_Kidd, Newman, Biousse_2008
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Figure 11–5 Left-sided Horner’s syndrome: There is pupillary miosis, ptosis of the upper lid, and elevation of the lower lid when compared with the normal side.
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Figure 11–6 Unilateral postganglionic Horner’s syndrome in a patient presenting with headache. The affected pupil (dashed line) has a smaller resting diameter in the dark and shows redilatation lag (arrow) compared with the unaffected fellow eye (solid line). The cause in this case was unknown.
an intact sympathetic supply to the eye is required for normal melanization of the iris during development, sympathetic lesions that are congenital or occurred within the first few years of life lead to iris hypochromia (heterochromia iridis in unilateral cases).
The sympathetic nerves that supply the eye and face include vasomotor, sudomotor, and vasodilator fibers as well as pupillary fibers. Involvement of some or all of these fibers in Horner’s syndrome may give rise to a number of nonpupillary signs, including ptosis of the upper lid and elevation of the lower lid (because of weakness of Mueller’s muscles), congestion of the conjunctival vessels and lowered intraocular pressure (vasomotor fibers), and anhydrosis (sudomotor fibers) either of the entire ipsilateral face (for preganglionic lesions) or of a small patch of supraorbital skin above the affected eye (for postganglionic lesions). In some cases, damage to sympathetic vasodilator fibers in the face and neck gives rise to ipsilateral failure to flush on heat, on exercise, or in response to curries and spicy food (harlequin syndrome14). The pattern of sympathetic deficits seen in each patient with Horner’s syndrome varies considerably depending
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on the location, nature, and extent of the lesion, but in practice it is difficult to localize an isolated Horner’s on the basis of clinical examination alone.
The causes of Horner’s syndrome are legion, reflecting the long course of the peripheral sympathetic pathway from hypothalamus to eye. Central lesions associated with brainstem (Wallenberg’s syndrome, tumors) or spinal cord (syringomyelia, cervical spondylosis) pathology usually but not invariably produce long tract and other neurologic signs. A rare but easily missed sign is contralateral superior oblique paresis in an apparently isolated central Horner’s, which indicates a midbrain lesion of the ipsilateral trochlear nucleus through which the sympathetic fibers pass. Preganglionic lesions are most commonly associated with trauma to the chest or neck (including insertion of chest tubes, central lines, and pacemakers). However, occult malignancy must always be considered: Tumors at the apex of the lung (usually lung or breast) cause wasting of the small hand muscles and Horner’s syndrome (Pancoast’s syndrome), and neck tumors, both benign and malignant, may also interrupt the cervical sympathetic chain. The proportion of preganglionic Horner’s syndrome caused by malignancy has been estimated at up to 25%15 but in my experience the neuro-ophthalmic signs are rarely the presenting feature. Postganglionic lesions most often arise in the context of carotid, skull base, and cavernous pathology and may occur in association with visual loss, ophthalmoplegia, or accompanied only by trigeminal pain. The signs of a Horner’s syndrome may be masked if there is also an oculomotor nerve palsy. An important condition in the differential diagnosis of an isolated painful postganglionic Horner’s is internal carotid artery dissection: The eye signs may be the only abnormal examination finding, but if the diagnosis is missed there is a significant risk of subsequent stroke.
Bilateral Horner’s syndrome is difficult to diagnose and rarely suspected by patient or clinician. There may be little or no anisocoria, the lid and other extrapupillary signs are symmetrical, and pharmacologic tests are insensitive when there is no “control” eye for comparison (Fig. 11–7). The only reliable way to detect bilateral Horner’s syndrome is by measuring redilatation lag using formal pupillographic techniques.16 Bilateral Horner’s syndrome is most commonly seen in diabetes mellitus but is also found in other conditions associated with widespread autonomic neuropathy, for example, amyloidosis, pure autonomic failure, and the hereditary sensory and autonomic neuropathies.17
When the cause of an isolated Horner’s syndrome is not apparent, hydroxyamphetamine (or pholedrine, tyramine; see previous discussion) should be
Figure 11–7 Bilateral Horner’s syndrome. Both pupils are small with no anisocoria, and there is ptosis of both upper lids. Neither the patient nor the referring clinician were aware of the oculosympathetic paresis because the signs are bilateral and symmetrical. In the absence of a “control” eye, drug tests have poor sensitivity, and the diagnosis is best made using pupillography to demonstrate bilateral redilatation lag.
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used to distinguish between preganglionic and postganglionic lesions: The affected pupil fails to dilate when the last order neuron is damaged. Subsequent investigation will depend on the clinical context and may include chest, neck, and head imaging, but the yield is low and the cause in many cases is never established.
Excitation of sympathetic fibers by irritative lesions may lead to mydriasis (“springing pupil”) or, if affecting only one meridian, a “tadpole pupil” (Fig. 11–8).18 These pupil changes are invariably unilateral and transient lasting only a minute or two but often associated with blurring of vision and an odd sensation within the eye. The pupil shows brisk and normal light and near responses both during and between these “attacks” confirming that these changes occur as a result of sympathetic overactivity and not parasympathetic underactivity. It is interesting to note that in many cases pharmacologic and pupillographic tests reveal a degree of coexisting sympathetic block.19 The etiology of both springing pupils and tadpole pupils is unknown but appears to be benign and further investigations are not indicated.
PARASYMPATHETIC LESIONS
In contrast to sympathetic lesions, damage to the parasympathetic supply to the eye produces different clinical signs depending on whether the lesion is preganglionic or postganglionic, and so clinical examination alone is required to make the distinction. The pupil in preganglionic lesions is dilated, with the anisocoria most apparent in the light, and its shape is round with no selective paresis of different meridians of the sphincter muscle. The light reflex is attenuated or absent to both direct and consensual stimuli, and there is no miosis during an accommodative effort although convergence may be preserved. Unaided near acuity will be reduced in nonpresbyopic patients because of the cycloplegia. Within days, the denervated sphincter muscle becomes supersensitive to weak receptor agonists such as 0.1% pilocarpine, and so (contrary to what is written in some older textbooks) this drug test has no localizing value.
Preganglionic parasympathetic blockade usually occurs in the context of oculomotor nerve palsies and much has been written about the significance of pupil involvement or pupil “sparing.” In general, the rule suggested by Rucker20 still holds true: If the oculomotor nerve palsy is incomplete, then pupil involvement implies an extrinsic compressive lesion until proved otherwise
Figure 11–8 Example of a “tadpole” pupil. The pupil has a circular shape most of the time (left) but during an “attack,” it becomes elongated along the 7 o’clock meridian (center). Within 5 to 10 minutes, it has returned to its normal round shape (right). In many cases, a tadpole pupil occurs in the context of an underlying Horner’s syndrome.
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(mostly aneurysms in his large case series). The anatomical studies of Kerr and Hollowell21 help to explain this clinical finding: The pupillomotor fibers travel superficially throughout much of the subarachnoid part of the nerve rendering them more susceptible to compressive lesions but less susceptible to ischemia. Some additional caveats are needed when applying this rule in clinical practice. First, the rule applies only to incomplete third nerve palsies—when all muscles supplied by the oculomotor nerve are denervated the fact there is pupil involvement contributes nothing to the differential diagnosis. Second, pupil sparing may be seen early in the evolution of compressive lesions and so patients with pupil-sparing incomplete third nerve palsies need careful monitoring to ensure that the pupil does not become involved days or weeks after initial presentation with external ophthalmoplegia. Third, any patient with progressive ophthalmoplegia requires neuroimaging regardless of the pupil signs because a surgical cause is very likely. Finally, whereas it is not uncommon to see patients with a pupil-sparing third nerve palsy, the opposite almost never occurs (i.e., internal ophthalmoplegia sparing all the extraocular muscles and levator): This clinical picture is most often seen as a result of receptor blockade, for example after inadvertent exposure to an atropine-like drug (see previous discussion).
Postganglionic parasympathetic blockade occurs as a result of damage to the ciliary ganglion or the short posterior ciliary nerves and leads to a different clinical picture. The pupil is initially large and round showing little reaction to light or near, but with time the pupil becomes progressively smaller—eventually in some cases reversing the anisocoria—and irregular in shape. A magnified view of the pupil margin as seen using slitlamp biomicroscopy reveals “sector palsy” with bunching of the iris collarette in some meridians and stretching in other meridians (Fig. 11–9) leading to “vermiform” movements as light is moved across the pupil margin. The light reflex is slow and attenuated or may be completely absent to both direct and consensual stimuli. In contrast, the pupil shows an exaggerated miosis during accommodative efforts (i.e., there is lightnear dissociation) and moreover the pupillary changes when transferring gaze from far to near and especially from near back to far again are greatly slowed (Fig. 11–10): This pupil behavior is termed “tonic” (the inability to relax following muscle contraction reminding one of myotonia elsewhere) and is pathognomonic of damage to the postganglionic parasympathetic fibers. At least some of these pupil features can be understood in terms of aberrant regeneration: More than 95% of postganglionic fibers emerging from the ciliary ganglion normally terminate in the ciliary muscle, but after injury many of these regenerating
Figure 11–9 Irregular pupil shape in Holmes-Adie syndrome. Sector palsy and aberrant regeneration lead to bunching of the collarette in some meridians but stretching in others. The result is an irregular pupil margin that shows vermiform movement when stimulated with the beam of a slit lamp.
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Figure 11–10 Pupil responses to a light stimulus (left graph) and a near effort (right graph— arrow indicates time when gaze moved from distance to near target). The tonic pupil (dashed line) shows little response to light but a slow, exaggerated response to near. The pupil in the fellow eye shows normal responses to both light and near. The patient had tendon areflexia and Holmes-Adie syndrome.
“accommodation” fibers mistakenly terminate in the sphincter muscle giving rise to exaggerated near but impaired pupil light responses.22
Since Holmes23 and his student Adie24 reported patients with tonic pupils and ipsilateral tendon areflexia, considerable confusion has been generated in the literature as a result of failing to distinguish between the nonspecific pupil signs described previously and their eponymous syndrome. Clarity is restored if the term “Adie’s pupil” is expunged and replaced by the more descriptive term “tonic pupil.” A tonic pupil results from damage to the postganglionic parasympathetic supply to the eye. If unilateral, it may be caused by local processes such as traumatic, neoplastic, or inflammatory orbital disease and even panretinal laser photocoagulation in the eye. If bilateral and symmetrical, the clinician should suspect a generalized autonomic neuropathy such as pure autonomic failure, amyloidosis, Sjo¨gren’s syndrome, or paraneoplastic states.17
In many patients, however, no cause can be identified: In some of these idiopathic cases, the pupil signs are associated with ipsilateral tendon areflexia in otherwise well young adults—this is properly called Holmes-Adie syndrome and occurs most commonly in females. The signs in Holmes-Adie syndrome are unilateral at presentation in 80% to 90%, but the risk of second eye involvement is estimated at 10% per decade thereafter. When bilateral, there is usually marked anisocoria and asymmetry between the eyes, reflecting perhaps the staggered involvement of the eyes over time in contrast to the symmetrical pupil signs found in generalized dysautonomias in which the insults are synchronized. Laboratory testing of autonomic function in patients with a clinical diagnosis of
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Holmes-Adie syndrome reveals a significant prevalence of subclinical abnormalities in both sympathetic and parasympathetic arms of the autonomic nervous system, and the number of these abnormalities increases over time, implying that the unknown pathologic process is not only more widespread than initially thought but also progressive.25,26 However, the only extraocular symptom experienced by a small number of patients with Holmes-Adie syndrome is a patchy disturbance of sweating because of damage to the sympathetic sudomotor fibers (Ross syndrome27); in general, patients with Holmes-Adie syndrome complain only of the cycloplegia and anisocoria and the disease runs a benign course.
LESIONS OF THE VISUAL PATHWAY
Use of the pupil to diagnose lesions of the anterior visual pathways has been practiced since the days of Galen of Pergamon in the second century.28 The pupil signs can, however, be subtle: Lesions that interrupt the afferent limb of the pupil light reflex attenuate or abolish the pupil response to light, but in most other respects the pupil examination in these patients appears normal. Both pupils are round, central, and seem to be of normal size even in patients with no perception of light in either eye (it is likely that both pupils have larger resting light diameters after such visual loss, but without premorbid measurements and with a wide range of “normal” diameters, this size increase is rarely apparent). The lesion does not cause anisocoria even when unilateral. Both pupils constrict briskly and normally to an accommodative effort (i.e., light-near dissociation) confirming integrity of the efferent limb of the light reflex. The pupil responds normally in all drug tests.
The visual pathway damage becomes evident when a bright light is directed into the eye and the phasic pupil light response is observed. With unilateral lesions, the direct response is smaller or absent compared with the consensual response, and the swinging flashlight test confirms the presence of a relative afferent pupil defect (RAPD). In broad terms, an RAPD is seen only with lesions of the retina or optic nerve. Media opacities such as corneal scarring, cataract, or vitreous hemorrhage merely scatter the light degrading acuity but preserving the pupillomotor drive from retinal luminance receptors. It should be remembered that the swinging flashlight test is a comparative assessment, and the “good” eye is only shown to be better than the “bad” eye and not necessarily normal. With bilateral symmetrical lesions, both pupils respond poorly to light (reduced amplitude and “sluggish,” i.e., delayed latency and slowed constriction velocity), and there is no RAPD.
Chiasmal lesions, which affect crossing fibers, symmetrically cause no clinically apparent abnormality in the pupil light reflex. Optic tract lesions theoretically should generate an RAPD because the temporal hemifield is larger than the nasal,29 but in practice this is extremely difficult to reproduce convincingly in a patient and the light responses usually look symmetrical. Retrogeniculate lesions were classically thought not to affect the pupil reflex arc,30 and so it should be possible to distinguish between tract and radiation hemianopias on the basis of the pupil reactions to light shone selectively into the blind and seeing hemifields. Pupil perimetry, however, shows that retrogeniculate lesions do cause significant attenuation of the pupil light response in the blind hemifield (pupillary “hemiakinesia”),31,32 presumably by interrupting a centrifugal connection
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between striate cortex and pretectum/midbrain, and so pupil examination is not as useful as disc examination in making this distinction. Modern pupillographic techniques have similarly confirmed that the pupil response in dense amblyopia is mildly attenuated,33 but in clinical practice it is unwise to ever accept amblyopia as a cause of an RAPD. The safest clinical rule is to state that any convincing RAPD must indicate either retinal or optic nerve disease even if the fundus examination is unremarkable.
The degree of attenuation of the pupil light response shows best correlation with the extent of visual field loss. In unilateral lesions, the RAPD measured using neutral density filters shows moderately good linear correlation with the mean visual defect measured using automated perimetry.34 With bilateral lesions, the RAPD correlates with the difference between the mean visual defects in the two eyes. The threshold for clinical detection of an RAPD appears to be an inter-eye difference in mean visual defects of at least 8 dB.35 In contrast, attenuation of the pupil light reflex shows little correlation with visual acuity (e.g., poor acuity from cataract but normal pupil light response, or sluggish pupil light response because of extensive visual field loss in advanced glaucoma but preserved acuity) or color vision. In some conditions, there appears to be an interesting mismatch between the extent of the visual defect and the degree of attenuation of the pupil light responses. Patients with Leber’s hereditary optic neuropathy36 or dominant optic atrophy37 have very poor vision but a relatively preserved pupil light reflex, whereas patients after recovery from optic neuritis may have an apparently complete recovery of vision but show a persistent RAPD.38 This mismatch has been called pupillovisual dissociation and may reflect the susceptibilities of different fiber subpopulations within the optic nerves to axonal compared with demyelinating pathologies.28
LESIONS IN THE MIDBRAIN
Pretectal lesions, most commonly extrinsic compression from pineal region tumors or hydrocephalus, interrupt the light reflex pathway without affecting the near triad (which originates ventral to the aqueduct) or the geniculostriate projection. These patients therefore have the classic triad of Parinaud’s (or dorsal midbrain) syndrome: absent pupil light responses, preserved (and brisk rather than tonic) near responses, and normal vision. The pupil abnormalities are almost always bilateral but can be asymmetric; the pupils are large in diameter, normal in shape and position, and constrict to a near effort but not to a light stimulus (i.e., light-near dissociation) despite normal sight. Lesions causing Parinaud’s syndrome are usually large and affect a number of other structures in the upper midbrain. They are often associated with a vertical saccadic palsy (through damage to the rostral interstitial nucleus of Cajal) with convergence retraction nystagmus and Collier’s sign (lid retraction on attempted upgaze), and some patients also develop a skew deviation (from interruption to the otolith pathway to the oculomotor nuclear complex). Some patients particularly in the pediatric age range also develop papilledema. These signs are often reversible once surgical decompression of the dorsal midbrain has been achieved.
A rare abnormality nowadays is the Argyll Robertson (AR) pupil seen in tertiary syphilis. First described in the mid-19th century before a cause for tabes dorsalis had been established,39 it rapidly became an ominous clinical sign in
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the preantibiotic era indicating progression to central nervous system disease. AR pupils are small in both the light and the dark and are often misshapen (because of concurrent inflammatory eye disease rather than the central lesion). The light response is diminished or absent, but a brisk near response can be elicited despite the small resting pupil diameters. These pupils will respond normally to topically applied receptor agonists and antagonists unless there is local damage to the iris. Visual function is classically described as normal but may be poor if there is concurrent involvement of the eye or visual pathways in the treponemal infection. It is not known where the abnormality lies in these patients and autopsy evidence has been inconclusive, but it is assumed that the lesion is ventral to the aqueduct in the upper midbrain. The origin of the pupillary miosis is also debated but may be because of interruption to the supranuclear inhibitory pathways that suppress the natural discharge rates of Edinger-Westphal neurons. The advent of penicillin has made this a rare diagnosis, but other conditions may produce pupils that have all the same features including metabolic (chronic alcoholism, diabetes mellitus), neuroinflammatory (multiple sclerosis, sarcoid), neurodegenerative (dementia), and neurogenetic (myotonic dystrophy) disease.
A number of other unusual pupil findings may be found rarely in association with midbrain disease, including “spastic miosis” (small pupils that do not react to light or near) and “inverse-AR” pupils (small pupils that react to light but not to near). The pattern of deficits is determined by which of the light, near, and central inhibitory pathways have been damaged. All of these midbrain syndromes share in common the following features: The pupil abnormalities are bilateral and usually symmetrical, visual function is preserved, and the pupils respond normally to all pharmacologic tests (assuming no concurrent damage to the eye). An increasingly wide spectrum of etiology is now recognized as potentially causing these syndromes, but a good rule of thumb particularly now that syphilis incidence is rising is that any patient with “midbrain pupils” and normal neuroimaging should have their serum and cerebrospinal fluid tested for syphilis.
REFERENCES
1.Warwick R: The ocular parasympathetic nerve supply and its mesencephalic sources. J Anat 1954;88:71–93.
2.Bremner FD, Booth A, Smith SE: Benign alternating anisocoria. Neuro-ophthalmology 2004;28:129–135.
3.Smith SA, Ellis CJ, Smith SE: Inequality of the direct and consensual light reflexes in normal subjects. Br J Ophthalmol 1979;63:523–527.
4.Loewenfeld IE: Pupillary changes related to age. In Thompson HS, Daroff R, Frisen L, et al (eds): Topics in Neuro-Ophthalmology, Baltimore, Williams & Wilkins, 1979, pp 124–150.
5.Levatin P: Pupillary escape in diseases of the retina or optic nerve. Arch Ophthalmol 1959;62:768–779.
6.Wilhelm H, Lu¨dtke H, Wilhelm B: Pupillographic sleepiness testing in hypersomniacs and normals. Graefes Arch Clin Exp Ophthalmol 1998;236:725–729.
7.Salazar-Bookaman MM, Wainer I, Patil PN: Relevance of drug-melanin interactions to ocular pharmacology and toxicology. J Ocul Pharmacol 1994;10:217–239.
8.Kazakos DC, Smith SE, Bron AJ: The pupil response to pilocarpine (0.125%) in dry eye patients. Ophthalmol Res 2001;33(S1):108.
9.Bremner FD, Houlden H, Smith SE: Genotypic and phenotypic heterogeneity in familial microcoria. Br J Ophthalmol 2004;88:469–473.
10.Pourfour du Petit D: Me`moires dans lequel il est de`montre` que les nerfs intercostaux fournissent des rameaux qui portent les esprits aux yeux. Hist Acad Roy Sci (Paris) 1727;1:1–19.
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11.Bernard C: Expe`riences sur les function de la portion ce`phalique du grand sympathique. CR Soc Biol (Paris) 1852;155:168–170.
12.Mitchell SW, Morehouse G, Keen W: Gunshot Wounds and Other Injuries of Nerves, Philadel-
phia, JP Lippincott, 1864, p. 164.
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13. Horner F: Uber eine form von ptosis. Klin Monatsbl Augenheilkd 1869;7:193–198.
14. Lance JW, Drummond PD, Gandevia SC, Morris JG: Harlequin syndrome: The sudden onset of unilateral flushing and sweating. J Neurol Neurosurg Psychiatry 1988;51:635–642.
15. Thompson H, Maxner C, Corbett J: Horner’s syndrome due to damage to the preganglionic neuron of the oculosympathetic pathway. In Huber A (eds): Sympathicus und Auge, Stuttgart, Ferdinand Enke, 1990, pp 99–104.
16. Smith SA, Smith SE: Bilateral Horner’s syndrome: Detection and occurrence. J Neurol Neurosurg Psychiatry 1999;66:48–51.
17. Bremner FD, Smith SE: Pupil findings in a consecutive series of 150 cases of generalised autonomic neuropathy. J Neurol Neurosurg Psychiatry 2006;77:1163–1168.
18. Thompson HS, Zackon DH, Czarnecki JSC: Tadpole-shaped pupils caused by segmental spasm of the iris dilator muscle. Am J Ophthalmol 1983;96:467–477.
19. Balaggan KS, Bremner FD, Hugkulstone CE: Episodic segmental iris dilator muscle spasm—the tadpole-shaped pupil. Arch Ophthalmol 2003;121:744–745.
20. Rucker CW: The causes of paralysis of the third, fourth and sixth cranial nerves. Am J Ophthalmol 1966;61:1293–1298.
21. Kerr FWL, Hollowell OW: Location of pupillomotor and accommodation fibers in the oculomotor nerve: Experimental observations on paralytic mydriasis. J Neurol Neurosurg Psychiatry 1964; 27:473–481.
22. Lowenfeld IE, Thompson HS: The tonic pupil: A reevaluation. Am J Ophthalmol 1967;63:46–87. 23. Holmes G: Partial iridoplegia with symptoms of other diseases of the nervous system. Trans
Ophthalmol Soc UK 1931;51:209–228.
24. Adie WJ: Pseudo-Argyll Robertson pupils with absent tendon reflexes: Benign disorder simulating tabes dorsalis. Br Med J 1931;1:928–930.
25. Bacon PJ, Smith SE: Cardiovascular and sweating dysfunction in patients with Holmes-Adie syndrome. J Neurol Neurosurg Psychiatry 1993;56:1096–1102.
26. Jacobson DM, Hiner BC: Asymptomatic autonomic and sweat dysfunction in patients with Adie’s syndrome. J Neuro-Ophthalmol 1998;18:143–147.
27. Ross AT: Progressive selective sudomotor denervation. A case with coexisting Adie’s syndrome. Neurology 1958;8:809–817.
28. Bremner FD: Pupil assessment in optic nerve disorders. Eye 2004;18:1175–1181.
29. Bell RA, Thompson HS: Relative afferent pupillary defect in optic tract hemianopias. Am J Ophthalmol 1978;85:538–540.
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30. Wernicke G: Uber hemianopische pupillenreaction. Fortschr Med 1883;1:49–53.
31. Cibis GW, Campos EC, Aulhorn E: Pupillary hemiakinesia in suprageniculate lesions. Arch Ophthalmol 1975;93:1322–1327.
32. Barbur JL, Ruddock KH, Waterfield VA: Human visual responses in the absence of the geniculocalcarine projection. Brain 1980;103:905–928.
33. Greenwald MJ, Folk ER: Afferent pupillary defects in amblyopia. J Pediatr Ophthalmol Strabis 1983;20:63–67.
34. Kardon RH, Haupert CL, Thompson HS: The relationship between static perimetry and the relative afferent pupillary defect. Am J Ophthalmol 1993;115:351–356.
35. Johnson LN, Hill RA, Bartholomew MJ: Correlation of afferent pupillary defect with visual field loss on automated perimetry. Ophthalmology 1988;95:1649–1655.
36. Bremner FD, Shallo-Hoffmann J, Riordan-Eva P, et al: Comparing pupil function with visual function in patients with Leber’s hereditary optic neuropathy. Invest Ophthalmol Vis Sci 1999;40:2528–2534.
37. Bremner FD, Tomlin EA, Shallo-Hoffmann J, et al: The pupil in dominant optic atrophy. Invest Ophthalmol Vis Sci 2001;42:675–678.
38. Bremner FD, Tomlin EA, Shallo-Hoffmann J, et al: Poor recovery of the pupil light reflex following acute optic neuritis. Neuro-ophthalmology 2001;25:56.
39. Robertson DA: Four cases of spinal miosis: With remarks on the action of light on the pupil. Edinburgh Med J 1869;15:487–493.
12 Papilledema and Idiopathic
Intracranial Hypertension
KATHLEEN DIGRE
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Papilledema |
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What Is Papilledema and How |
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Secondary Intracranial |
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Hypertension |
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Unusual Forms of Papilledema |
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Diagnosing the Underlying |
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Cause of Papilledema |
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Natural History and Visual |
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Complications |
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Treatment of Papilledema |
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Key Points
Papilledema is a term reserved for disc swelling resulting from increased intracranial pressure.
Evaluation for papilledema includes an imaging procedure that adequately excludes intracranial masses and venous thrombosis; if no mass or thrombosis is present, a lumbar puncture with accurate measurement of opening pressure and cerebrospinal fluid contents should be made.
The most common cause of increased intracranial pressure is primary (or idiopathic) intracranial hypertension; it is associated with female gender and obesity.
Visual loss is a disabling feature of papilledema. Careful evaluation of vision, including visual fields, is necessary; when vision is threatened despite maximum medical therapy, a surgical procedure should be considered.
Papilledema
Papilledema is one of the true neuro-ophthalmic emergencies. Not only can the sign signal an underlying brain tumor or acute neurologic process, but swelling of the disc can also mean visual loss for the patient.
WHAT IS PAPILLEDEMA AND HOW DO WE RECOGNIZE IT?
Papilledema is the term used to denote disc swelling related to increased intracranial pressure (ICP). Other terms including “choked disc,” “papillitis,” and “disc
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