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Lesions of the macula, retina, or optic nerve cause a scotoma (an island of impaired vision surrounded by normal vision) rather than a defect that extends to the periphery of one visual eld ( eld cut ) Scotomas are named according to their position (central, cecocentral) or their shape (ring, arcuate) A small scotoma that is situated in the macular part of the visual eld may seriously impair visual acuity Scotomas are the main features of optic neuropathy, the causes of which have been mentioned earlier Demyelinative disease (optic neuritis), Leber hereditary optic atrophy, toxins (methyl alcohol, quinine, chloroquine, and certain phenothiazine drugs), nutritional de ciency (so-called tobacco-alcohol amblyopia), and vascular dis-
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CARDINAL MANIFESTATIONS OF NEUROLOGIC DISEASE
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ease (ischemic optic neuropathy or occlusion of a branch of the retinal artery) are the usual ones Orbital or retro-orbital tumors and infectious or granulomatous processes (eg, sarcoid, retinal toxoplasmosis in AIDS) are other common causes Certain toxic and malnutritional states are characterized by more or less symmetrical bilateral central scotomas (involving the xation point) or cecocentral ones (involving both the xation point and the blind spot) The cecocentral scotoma, which tends to have an arcuate border, represents a lesion that is predominantly in the distribution of the papillomacular bundle However, the presence of this visual eld abnormality does not establish whether the primary defect is in the cells of the origin of the bundle, ie, the retinal ganglion cells, or in their bers Demyelinative disease is characterized by unilateral or asymmetrical bilateral scotomas Vascular lesions that take the form of retinal hemorrhages or infarctions of the nerve- ber layer (cotton-wool patches) give rise to unilateral scotomas; occlusion of the central retinal artery or its branches causes infarction of the retina and, as a rule, a loss of central vision As pointed out earlier, anterior ischemic optic neuropathy causes sudden monocular blindness or an altitudinal eld defect Since the optic nerve also contains the afferent bers for the pupillary light re ex, extensive lesions of the nerve will cause a so-called afferent pupillary defect, which has been mentioned earlier and is considered further in Chap 14 With most diseases of the optic nerve, as alluded to above, the optic disc will eventually become pale (atrophic) This usually requires 4 to 6 weeks to develop If the optic nerve degenerates (eg, in multiple sclerosis, Leber hereditary optic atrophy, traumatic transection, tumor of nerve, or syphilitic optic atrophy), the disc becomes chalk-white, with sharp, clean margins If the atrophy is secondary to papillitis or papilledema, the disc margins are indistinct and irregular; the disc has a pallid, yellow-gray appearance, like candle tallow; the vessels are partially obscured; and the adjacent retina is altered because of the outfall of bers As in the case of optic neuritis, visual evoked potentials from the stimulation of one eye may be slowed even if the optic disc appears normal and there is no perimetric abnormality
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Lesions of the Chiasm, Optic Tract, and Geniculocalcarine Pathway
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Hemianopia (hemianopsia) means blindness in half of the visual eld Bitemporal hemianopia indicates a lesion of the decussating bers of the optic chiasm and is caused most often by the suprasellar extension of a tumor of the pituitary gland (Fig 13-2C) It may also be the result of a craniopharyngioma, a saccular aneurysm of the circle of Willis, and a meningioma of the tuberculum sellae; less often, it may be due to sarcoidosis, metastatic carcinoma, ectopic pinealoma or dysgerminoma, Hand-Schuller-Christian dis ease, or hydrocephalus with dilation and downward herniation of the posterior part of the third ventricle (Corbett) In some instances a tumor pushing upward presses the medial parts of the optic nerves, just anterior to the chiasm, against the anterior cerebral arteries Heteronymous eld defects, ie, scotomas or eld defects that differ in the two eyes, are also a sign of involvement of the optic chiasm or the adjoining optic nerves or tracts; they are caused by craniopharyngiomas or other suprasellar tumors and rarely by mucoceles, angiomas, giant carotid aneurysms, and opticochiasmic arachnoiditis The visual eld pattern created by a lesion in the optic nerve as it joins the chiasm typically includes a scotomatous defect on
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the affected side coupled with a contralateral superior quadrantanopia ( junctional eld defect ) As noted previously, the latter is caused by interruption of nasal retinal bers which, after crossing in the chiasm, project into the base of the affected optic nerve (Wilbrand s knee, see Fig 13-2B) A sharply de ned pattern of this type is relatively uncommon Variations in the pattern of visual loss are frequent, in part accounted for by the location of the chiasm in an individual patient a pre xed chiasm making unilateral eye ndings more common Homonymous hemianopia (a loss of vision in corresponding halves of the visual elds) signi es a lesion of the visual pathway behind the chiasm and, if complete, gives no more information than that Incomplete homonymous hemianopia has more localizing value As a general rule, if the eld defects in the two eyes are identical (congruous), the lesion is likely to be in the calcarine cortex and subcortical white matter of the occipital lobe; if they are incongruous, the visual bers in the optic tract or in the parietal or temporal lobe are more likely to be implicated Actually, absolute congruity of eld defects is rare, even with occipital lesions The lower bers of the geniculocalcarine pathway (from the inferior retinas) swing in a wide arc over the temporal horn of the lateral ventricle and then proceed posteriorly to join the upper bers of the pathway on their way to the calcarine cortex (Fig 13-2) This arc of bers is known variously as the Flechsig, Meyer, or Archambault loop, and a lesion that interrupts these bers will produce a superior homonymous quadrantanopia (contralateral upper temporal and ipsilateral upper nasal quadrants; Fig 13-2E) This clinical effect was rst described by Harvey Cushing, so that his name also has been applied to the loop of temporal visual bers Parietal lobe lesions are said to affect the inferior quadrants of the visual elds more than the superior ones, but this is dif cult to document; with a lesion of the right parietal lobe, the patient ignores the left half of space; with a left parietal lesion, the patient is often aphasic As to the localizing value of quadrantic defects, the report of Jacobson is of interest; he found, in reviewing the imaging studies of 41 patients with inferior quadrantanopia and 30 with superior quadrantanopia, that in 76 percent of the former and 83 percent of the latter the lesions were con ned to the occipital lobe If the entire optic tract or calcarine cortex on one side is destroyed, the homonymous hemianopia is complete But often that part of the eld subserved by the macula is spared, ie, there is a 5- to 10-degree island of vision around the xation point on the side of the hemianopia (sparing of xation, or macular sparing) With infarction of the occipital lobe due to occlusion of the posterior cerebral artery, the macular region, represented in the most posterior part of the striate cortex, may be spared by virtue of collateral circulation from branches of the middle cerebral artery Incomplete lesions of the optic tract and radiation usually spare central (macular) vision We have nevertheless observed a lesion of the tip of one occipital lobe that produced central homonymous hemianopic scotomata, bisecting the maculae Lesions of both occipital poles (as in embolization of the posterior cerebral arteries) result in bilateral central scotomas; if all the calcarine cortex or all the subcortical geniculocalcarine bers on both sides are completely destroyed, there is cerebral or cortical blindness (see below and Chap 22) An altitudinal defect is one that is con ned to the upper or lower half of the visual eld but crosses the vertical meridian Homonymous altitudinal hemianopia is usually due to lesions of both occipital lobes below or above the calcarine sulcus and rarely to a
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