Neuro-Ophthalmologic Manifestations of Nonorganic Disease
Patients who have physical signs and symptoms for which no adequate organic cause can be found may receive any one of a large range of diagnostic labels, including functional illness, functional overlay, hysteria, hysterical overlay, conversion reaction, psychophysiologic reaction, somatization reaction, hypochondriasis, invalid reaction, neurasthenia, psychogenic reaction, psychosomatic illness, malingering, and Munchausen syndrome. This plethora of labels highlights the confusion that occurs when one tries to fit patients with nonorganic disease into a formal classification.
General Considerations
The nature of the symptom and the manner of its communication are crucial in the understanding of a nonorganic disorder. The patient may be stoic and restrained, or histrionic and dramatizing. The symptom may take the form of a physical dysfunction (e.g., strabismus) that is displayed with a minimum of verbal description, or the symptom may be described verbally during the examination. Thus, one must also determine the nature of the physical dysfunction and the degree of disability. It is important to determine why the symptoms have focused in a particular area (e.g., the visual system).
Another consideration is the amount of time a patient spends thinking about his or her symptoms and the precise nature of the thoughts in phenomenologic terms. This concept is called ideation.
A patient’s affect may tell the physician much about the patient’s complaints. Some patients clearly are depressed, whereas others are truly indifferent, and still others are anxious. Of particular interest is a patient’s attitude toward those involved in the diagnosis and treatment of his or her condition. These are persons with whom one would expect the patient to cooperate in an attempt to get well. Is the patient hostile, suspicious, fearful, flirtatious, pleading, aloof, or excessively cooperative and agreeable?
Understanding a patient’s motivation or incentive for achieving the “sick role” and the degree to which he or she is conscious of it may be the most difficult part of the diagnostic process, but it may be the most crucial. The nature of motivation may range from an unconscious seeking of the dependency-gratifying and guilt-allaying aspects of the “sick role” to the overtly conscious attempt to obtain attention, sympathy, material gain, or a combination of these. Hence, the presentation needs to be contextualized to the patient’s overall state and circumstances.
Mere observation of the patient in waiting room or examination room can be very rewarding. For example, individuals with a claim of profound visual loss may be limited in their capacity to navigate, use his/her phone, or perform detailed tasks. However, many patients with nonorganic disease perform well on these types of near-habitual tasks, without realizing the inconsistency. It is also important to remember that “real” disease, at least to some extent, often accompanies a nonorganic component. Hence, the examiner must be thorough and not assume that a patient presenting with nonorganic visual loss must have “normal” vision.
Terminology
Most nonorganic disturbances are categorized by three types: (a) malingering; (b) Munchausen syndrome; and (c) psychogenic.
Malingering
Patients whose symptoms are consciously and voluntarily produced are said to be malingering. Malingering can be divided into several different categories, including simulation of nonexistent disease, elaboration of pre-existing disease, and attribution of a disability to a different cause. The most common settings in which malingering occurs are potential compensation after a real or feigned injury, avoidance of a particular task, such as military service or a simple school examination for which the patient is unprepared, or an attempt to seek special attention from family or friends.
Munchausen Syndrome
Malingering must be differentiated from factitious disorder with physical symptoms, also called the Munchausen syndrome. Patients with this condition intentionally produce physical symptoms and signs, some of which may be ocular. Manifestations might include swelling and redness of the conjunctiva simulating an orbital cellulitis, scarring of the eyelids and conjunctiva, and even chorioretinal scarring, all of which are then presented to members of the medical profession. Patients with Munchausen syndrome are thought to harbor a psychological internal need to adopt the role of a sick person.
Psychogenic Disturbance
Patients whose symptoms seem truly independent of volition are said to have a somatoform disorder or psychogenic disturbance. Examples of psychogenic disturbances include body dysmorphic disorder, conversion disorder (hysteria, conversion reaction), hypochondriasis, and somatization disorder.
A body dysmorphic disorder is characterized by a patient’s perception of a single physical defect, most often in the facial region, including the eye. The patient is preoccupied with this sign, even though it is minimal (e.g., a mild ptosis or anisocoria) or is not present at all.
A conversion disorder is diagnosed if alterations or a loss of physical functioning are present that seem to express a psychological conflict or need rather than indicating organic illness. This disorder comprises the clinical syndromes that were previously classified as “hysteria” or “conversion neurosis.” Patients in whom such disorders occur may subconsciously obtain both primary gain (e.g., protection from trauma or reduction of stress) and secondary gain (e.g., increased attention).
Hypochondriasis is the fear of, or strong belief in, specific serious physical conditions accompanied by excessive self-observation and the reporting of numerous physical signs and symptoms. It differs from body dysmorphic disorder in that it includes both symptoms and signs from multiple organ systems throughout the body.
A somatization disorder features recurrent and multiple somatic complaints. As in hypochondriasis, multiple organ systems may be mentioned, but the patient’s descriptions are vague, and anxiety or depression usually is present.
Unfortunately, there remains a large group of patients in whom a clear distinction between malingering, Munchausen syndrome, and psychogenic or somatoform disturbances simply cannot be made. In such cases, the physician must recognize that there is no organic basis for the patient’s symptoms and signs and manage the patient accordingly.
Specific Nonorganic Neuro-Ophthalmologic Disorders
From a neuro-ophthalmologic standpoint, there are five areas that may be affected by nonorganic disease.
Vision, including visual acuity and visual field
Ocular motility and alignment
Pupillary size and reactivity
Eyelid position and function; and
Corneal and facial sensation.
The physician faced with a patient complaining of decreased vision or some other disturbance related to the afferent or efferent visual systems for which there is no apparent biologic explanation has two responsibilities. First, the physician must ascertain that an organic disorder is not present. Second, the physician must determine if the patient can see or do something that would not be possible if the condition were organic in nature. To best achieve these goals, the physician must adopt an empathetic attitude toward the patient regardless of the patient’s history, attitude of the patient toward the physician or the disease, or the clinical findings. Keeping an open mind will be helpful for the physician to not miss something important and will likely gain more cooperation from the patient than if a confrontational or dismissive tone is taken.
Nonorganic Disease Affecting the Afferent Visual Pathway
Nonorganic disease that affects the afferent visual system is extremely common. It may occur as monocular or binocular decreased visual acuity, abnormal visual fields, or both. Color vision often is abnormal in such
patients (depending on the manner in which it is tested), but abnormal color vision is rarely a primary complaint.
patients (depending on the manner in which it is tested), but abnormal color vision is rarely a primary complaint.
 Figure 23.1 Use of the optokinetic drum to detect nonorganic bilateral blindness. The patient is asked to look straight ahead with both eyes open, while the drum is rotated, first in one direction, then in the other. |
Decreased Visual Acuity
Decreased visual acuity is probably the most common nonorganic disturbance in ophthalmology. It occurs most often in children and young adults, but it may be observed in patients 60 years of age and older. It may be psychogenic or caused by malingering. Nonorganic visual loss that is psychogenic seems to be more common in children, with females being affected much more often than males. Malingerers are most often adult males, perhaps because men are more often involved in motor vehicle and work-related accidents than are women.
Patients with nonorganic loss of visual acuity complain of a variable loss of vision in one or both eyes that is not accompanied by a refractive error, a disturbance of the ocular media, or other evidence of retinal or optic nerve dysfunction. Abnormal color perception, an abnormal visual field, or both, may accompany the visual loss.
In many cases, the physician may suspect that the patient’s visual loss is nonorganic during the medical history, as noted above, is perhaps the most crucial aspect of the evaluation. In addition, the way the patient acts during the history taking may be helpful. Patients who are truly blind in both eyes tend to look directly at the person with whom they are speaking, whereas patients with nonorganic blindness, particularly patients who are malingering, often look in some other direction. Similarly, patients claiming complete or nearly complete blindness often wear sunglasses, even though they do not have photophobia, and the external appearance of the eyes is perfectly normal. In any event, the physician who suspects a nonorganic visual process may be able to orient the examination in a way to bring out the nonorganic nature of the visual disturbance.
If a patient claims no perception of light, light perception only, or perception of hand motions by one or both eyes, one can use a rotating optokinetic drum or horizontally moving tape to produce a horizontal jerk nystagmus that indicates intact vision of at least 20/400 (Fig. 23.1). It is important in this regard that the images on the tape or drum be sufficiently large, so that the patient is not able to look around them. When testing a patient who claims complete loss of vision in one eye only, the test is begun by rotating the drum or moving the tape in front of the patient, who has both eyes open. Once good optokinetic nystagmus is elicited, the unaffected eye is suddenly covered with the palm of the examiner’s hand or a handheld occluder (Fig. 23.2).
Patients with nonorganic loss of vision in one eye will continue to show a jerk nystagmus.
Patients with nonorganic loss of vision in one eye will continue to show a jerk nystagmus.
 Figure 23.2 Use of optokinetic drum to detect nonorganic unilateral blindness. A: In a patient claiming unilateral blindness, the drum is first rotated, while the patient is instructed to look straight ahead with both eyes open. B: Once nystagmus is elicited, the examiner continues to rotate the drum and suddenly covers the “normal” eye with the palm of the hand and observes the “blind” eye for continued nystagmus. |
A second test that is helpful in detecting visual function in an eye or eyes that are said to have either no perception of light or light perception only is the “mirror test.” A large mirror is held in front of the patient’s face, and the patient is asked to look directly ahead. The mirror is then rotated and twisted back and forth, causing the images in the mirror to move. Patients with vision better than light perception will show a nystagmoid movement of the eyes since they cannot avoid following the moving reflection in the mirror.
An excellent way to detect nonorganic visual loss in a patient who claims to be unable to see shapes or objects in one or both eyes is to ask the patient to touch the tips of the first fingers of both hands together. If the patient claims loss of vision in one eye only, the opposite eye is patched before the test is performed. The ability to touch the tips of the fingers of two hands together is based not on vision but on proprioception. Thus, patients with organic blindness can easily bring the tips of the first fingers of both hands together, whereas patients with nonorganic blindness, particularly those who are malingering, will often demonstrate an inability to do so (Fig. 23.3). Similarly, a patient with organic blindness can easily sign his or her name without difficulty, whereas patients with blindness caused by malingering may produce an extremely bizarre signature that often runs off the page or very large script.
A variety of tests may be performed in patients who claim vision in the range of 20/40 to hand motions in one or both eyes. None of these tests is invariably reliable, but one or more usually is sufficient to provide convincing evidence that visual acuity loss either is nonexistent or not as severe as the patient claims. Visual acuity may be tested not by starting from the largest letters or numbers and moving progressively to smaller ones, but by beginning with the smallest line (“bottom-up acuity”). Assuming that the patient cannot see this first line after being allowed to concentrate for several minutes, the physician tells the patient that the size of the print is now going to be “doubled” in size, and the patient is shown the next larger line and given several minutes to read it. This process is continued until the patient is able to read the line. This method of testing often produces visual acuity better than that initially claimed by the patient. In addition, some projector slides have several 20/20 lines, and these lines may be shown to the patient in succession as the examiner tells the patient the size of the letters is increasing. The key is to encourage the patient that each subsequent line presented should be “much easier” to see.
Testing of near vision is also important in patients claiming decreased acuity. A discrepancy in the distance
and near-visual acuity that is not attributable to a refractive error or a disturbance of the media, such as an oil drop cataract, usually is evidence of a nonorganic disturbance.
and near-visual acuity that is not attributable to a refractive error or a disturbance of the media, such as an oil drop cataract, usually is evidence of a nonorganic disturbance.
 Figure 23.3 Testing nonorganic visual loss by asking a patient who claims monocular or binocular blindness to touch the tips of the first fingers of each hand together. A and B: A person who is truly blind can easily touch the tips of the fingers together, using proprioception, as the person in these photographs is doing despite having both eyes occluded. C: A man with nonorganic loss of vision in both eyes is unable to touch the tips of the fingers together, even though he should be able to do so. A person with monocular nonorganic visual loss may touch the tips of the fingers together when viewing with the “normal” eye (D), but may claim to be unable to do so when viewing with the “blind” eye (E). |
Patients claiming decreased vision in one eye only may undergo a “refraction” in which the normal eye is fogged with a high plus lens (e.g., a +5.00 or higher sphere), and a lens with minimal power (e.g., a ±0.50 sphere or cylinder) is placed before the worse eye. The patient is then told to read the chart with “both eyes.” A variation of this test is the use of paired cylinders. A plus cylinder and a minus cylinder of the same power (usually 2 to 6 diopters) are placed at parallel axes in front of the “normal” eye in a trial frame. The patient’s normal correction is placed in front of the affected eye. The patient is asked to read, with both eyes open, a line that previously had been read with the normal eye but not with the affected eye. As the patient begins to read, the axis of one of the cylinders is rotated about 10 to 15 degrees. The axes of the two cylinders thus will no longer be parallel, blurring vision in the normal eye. If the patient continues to read the line, or can read it again when asked to do so, he or she must be using the affected eye.
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