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Acuity Swinging flashlight test Anterior segment Fundus Diagnosing visual loss of uncertain origin - see Chap. 2 Homonymous visual field defects or normal Nonphysiological only with justified suspicion j _ Simulation tests The where system occipitoparietal Disorders of visual orientation Flow diagram. Diagnostic procedures in the case of suspected central disturbances of vision The how or what system occipitotemporal Cerebral achromatopsia Table 13.1. Summary of commonly described symptoms in...
T Pearl 1
Presentation of an acutely tonic pupil in patients over 50 years of age should lead to testing of the erythrocyte sedimentation rate or of C-reactive protein levels, since giant cell arteritis can present in this way. In rare cases, there have been possibly coincidental associations with malignant disorders. Being so very uncommon, this possibility does not require diagnostic testing in most cases. It does help to be aware of the risk, however, and to plan accordingly. Patients with a history...
Diagnosis of Pupillary Disorders
Pupillary testing serves two purposes, first, to find disorders of pupillary function itself, and second, to detect disorders of the afferent visual system and the autonomic innervation of the eye. A systematic approach will help significantly with interpretation of the findings. The examination should be done in a logical order, since the pupillary system responds in a logically predictable way. Tests that yield no useful information can only create confusion. Anatomic and Physiologic...
Clinoid and Sphenoid Wing Meningiomas
Twenty-five percent of all meningiomas arise near the anterior clinoid processes or along the sphenoid wings. Most affected are women 66 aged 30 to 50 years. Exophthalmos is caused in 50 of cases, and optic disc swelling occurs often bilaterally in half of all meningiomas of this category. Growth through the superior orbital fissure and into the orbit is common, and is usually associated with prominent hyperostosis of the sphenoid wing, as seen on CT scans. Typical visual field findings include...
Eog 1
Diffuse and regional choroidal degenerations Choroidal atrophy central areolar, peripapillary, helical Diffuse and regional photoreceptor dystrophies Retinitis pigmentosa rod-cone dystrophy Depressed or extinguished scotopic ERG Depressed or extinguished photopic ERG and prolonged implicit times Normal or depressed implicit times often prolonged Stargardt's disease -fundus flavimaculatus
Info Qlt
Fig. 19.10. a Aicardi syndrome. Circumpapillary chorioretinal lacunae in a young girl. The disease is lethal in males. b Aicardi syndrome. Hypoplasia of the corpus callosum arrow and cortical heterotopia Proper diagnostic classification of congenital or neonatal nystagmus is challenging. A practical schema is illustrated in the flow diagram of Fig. 19.11 see also Chap. 11 . Sensory defect nystagmus SDN , or ocular nystagmus that arises from a congenital defect in retinal and or optic nerve...
Note Ghr
Children with newly discovered optic disc colobomas should be carefully studied to rule out an associated renal insufficiency. In Aicardi syndrome chorioretinal lacunas result in a characteristic appearance Fig. 19.10 . This disorder results in such severe malformations in males, that with few exceptions, none are born living. In affected girls, malformations of the CNS present, such as agenesis of the corpus callo-sum, schizencephaly a variably pronounced cleft formation of the cerebrum , and...
Cavernous Sinus Disease Affecting the Orbit
As a rule, lesions within the cavernous sinus produce complex disorders of ocular motility, simultaneously affecting more than one cranial nerve. The abducens nerve and the postganglionic fibers of the sympathetic pathway are more severely affected. In contradistinction to the cranial nerves that are protected within the lateral wall of the cavernous sinus third, fourth, and fifth the sixth nerve is suspended within the sinus, where it can be damaged most easily. An o abducens palsy that is...
Pearl Jee
The strategy does not attempt to degrade the patient, but is rather a means to demonstrate the actual presence of vision where it is claimed to be absent. Table 15.2. General principles of examination for when functional or feigned loss of vision is suspected 1. Testing for the reproducibility of responses Testing of reflexes such as pupillary light responses, optokinetic nystagmus Deception testing unrelated functions that the patient assumes are vision related Measuring a single function with...
Info Mdp
ERG electroretinogram, VEP visually evoked potentials, OKN optokinetic nystagmus 206 ERG electroretinogram, VEP visually evoked potentials, OKN optokinetic nystagmus 206 Observations of Behavior and Triggering of Reflexes For those complaining of complete blindness, behavioral observation provides telling information. When greeted for the first time, does the patient reach for your hand, bump into obstacles, or have trouble finding the examination chair On passage down a flight of stairs, those...
Mri
High-dose corticosteroids or no therapy Surgical decompression, radiation, or no intervention Benign tumors, visual recovery possible Fig. 8.15. At the acute onset of optic neuritis the optic disc can appear completely normal left or swollen Pain on eye movement is very characteristic of optic neuritis and is reported by more than 90 of affected patients. Fig. 8.15. At the acute onset of optic neuritis the optic disc can appear completely normal left or swollen 3. Age in a majority of cases, 18...
O
One examined retinal location with two threshold reversals DLS Differential luminance sensitivity decibel Several presentations only for assessment of depth Fig. 4.8. Testing strategies used with automated static perimetry. Left The principle of threshold static perimetry, in which the threshold for perception of a stimulus is determined by sequentially presenting test objects with stimulus values that lie just above or below a putative value at a given location, and threshold is defined as a...
Headache from a NeuroOphthalmic Point of View
Headache is the most frequent cause for cooperative interaction between neurologists and ophthalmologists. Frequently, patients are seen first by their primary care physicians, and are then referred to ophthalmologists for further testing and management, chiefly to rule out ocular sources of pain. From there, they are commonly referred to neurologists, to be sure that something serious will not be missed. The diagnostic classification of headache is at first glance extraordinarily confusing or...
Ocular Myasthenia
Ocular myasthenia should be considered when there is a history of ocular or general problems with fatigue, frequent variations in the size of a strabismus, an earlier episode of diplopia or ptosis, or signs of ocular muscle fatigue. A normal pupillary response to light and accommodation, a positive edrophonium test, and no restrictions found when testing forced ductions are all clues to a myasthenic process. Antibodies to acetylcholine receptors in the serum establish the diagnosis...
Tuberculum Sellae Meningiomas
Meningiomas often arise in the suprasellar region. Typically, they cause a rounded elevation of the planum sphe-noidale. Less commonly, there can be an associated hyper-prolactinemia and or hypopituitarism both being late developments . Growth of these masses is usually asymmetric, causing an initially monocular, fluctuating loss of vision with a central scotoma, and frequently an anterior junction syndrome of the chiasm see Chap. 3 . Tuberculum sellae meningiomas constitute 3 to 10 of all...
Meningiomas
Several forms of meningioma are recognized meningothe-lial, fibrous, and transitional are the most common subtypes. They arise ubiquitously. The growth can be nodular or en plaque a laminar spread along the surface of the dura , and there are extracerebral manifestations. Forty percent are basal meningiomas, 50 are tumors of the convexities, and 10 are meningiomas of the posterior cranial fossa. In up to 16 of cases, meningiomas arise multi-focally. Typically, there is a long, slow growth that...
Hereditary Disorders of NeuroOphthalmic Relevance
Hereditary disorders of importance for neuro-ophthalmology include genetically inherited disorders of the posterior segment and afferent visual pathway that characteristically present as visual field defects, visual acuity loss, strabismus, or even complete blindness. These disorders have significant social and economic importance with lifelong consequences for the afflicted patient. The prevalence of inherited retinal disorders is approximately 20 cases per 100,000 people. Decoding of the...
Info Ljc
Adapted from E. Zrenrier, Farbsinnprufungeri Eds. O.-E. Lund, Th. Waubke Enke, Stuttgart, 1985 Fig. 6.7. These formes serve as standard documents for recording the results of color vision testing. During tests with the anomaloscope, scale values for the mixture control 0 to 73 and the patient's answer to the question, Can the yellow field be made to match the mixture field in both brightness and color are recorded. If the answer to the question is yes, the scale value is recorded. The breadth...
Optic Atrophy after Papilledema Definition
damage to the optic nerve caused by chronic papillede-ma. If elevated intracranial pressure goes undetected, bilateral loss of vision can develop, because chronic papilledema often leads to gliosis and atrophy of the optic nerve. The same problem can develop if the cause of the elevated pressure cannot be corrected. The pathogenic mechanism is not well understood, but ischemia is thought to play an important role. The time needed to develop this complication is variable and is not predictable...
Tests of the VOR
The patient is asked to read small characters while turning his her head back and forth with increasing frequency. For examination of the vertical VOR, the patient is asked to read while nodding the head up and down. There is a threshold frequency beyond which the VOR of a healthy visual system cannot keep up, and illusory movements of the external world intrude. Such illusory movement is called oscillopsia. For a comparative assess ment, it is sufficient for the examiner to compare the...
Traumatic Optic Neuropathy Definition
A traumatic optic neuropathy is one caused by trauma to the optic nerve, most frequently in the setting of a traffic accident with cranial and or midface fractures. Traumatic optic neuropathy results primarily from indirect injury, rather than by direct crushing or tearing mechanisms. A direct blow to the eye can cause an avulsion of the optic nerve more properly called an expulsion . The mechanism appears to be one of a sudden, explosive increase in intraocular pressure with rupture of the...
Compression Syndromes
The development of a compression syndrome of the pregeniculate visual pathway should always suggest the possibility of an intracranial tumor, no matter which segment of the path is involved nerve, chiasm, tract or at what rate it has developed acute, subacute, or slowly progressive . Compression syndromes damage the afferent path by the mass effect of the pressure they exert on the involved segment. This can be caused by neoplasms, hemorrhages or obstruction to CSF flow e.g., aqueductal...
Functional Visual Loss and Malingering
Malingering is an intentionally deceptive mimicry of a nonexistent disorder, and augmentation is an intentionally exaggerated account of an existing disorder. Functional visual loss is a subjectively described visual disorder without an objectively observed abnormality. It is an unconscious, often subconscious, simulation of a nonexistent disease. Synonyms include psychogenic visual loss, conversion, and hysterical visual loss . The related group of psychogenic ocular disorders includes...
Pearl Nro
Meningiomas make up to 20 of all intracranial tumors. They are rare among children and adolescents a patient age of 50 years is typical, with women being most frequently affected. Eighty-five percent of all meningiomas arise in women 40 to 60 years old. Of primary ophthalmic importance are 1 optic nerve sheath meningiomas, 2 meningiomas of the tuberculum sellae, 3 meningiomas of the anterior clinoid process, and 4 sphenoid wing meningiomas. Intrasellar meningi-omas are uncommon, rarely cause...
T Pearl Jvh
The association of myelinated nerve fibers with cutaneous nevi is known as Gorlin's syndrome, and also as basal cell nevus syndrome, which needs to be identified early in life, so as to minimize the risk of developing cutaneous melanomas. Melanocytomas of the optic disc are benign and require only regular follow-up examinations. They are strikingly black in color, prominent, and commonly extend across the margin of the disc or are surrounded by a choroidal nevus. The have a soft, feather-like...
T Pearl Rcl
Those with the rather common congenital dyschroma-topsias, classified as dichromacy or anomalous trichro-macy, are able to correctly differentiate hues and shades within some parts of the hue circle. They are not colorblind, but rather have a diminished capacity to discriminate among some hues that are adjacent to one another in some part of the normal gamut of color perception. Hereditary Disorders of Color Perception Congenital X-linked dyschromatopsias are common Table 6.3 . About 8 of men...
Symptom Of Pelopsia
Fig. 13.3. Schematic diagram of the most common pseudo hallucinations. Upside down inverted objects. Macropsia microp-sia objects seen as too large or small. Telopsia pelopsia objects seen as too far or near. Palinopsia objects seen in correct relation to others e.g., exclusively situated on horizontal planes and thus in multiple locations a subtype of polyopsia . Metamorphopsia objects misshapen. Polyopsia multiple images of a single object a failure of the extinguishing center . Platyopsia...
Signs and Symptoms of the Arteritic Form of AION j Note
It is particularly important to consider the possibility that an acute case of ischemic optic neuropathy might be arteritic in nature, for if the problem is indeed caused by arteritis, it should be considered as an emergent situation requiring immediate intervention with highdose corticosteroid therapy. The average age at the onset of anterior ischemic optic neuropathy in patients with temporal arteritis AAION is about 75 years. Arteritis can also present with branch retinal vessel occlusions,...
Central Vestibular Nystagmus
Central vestibular nystagmus is caused by damage to the vestibular nuclei or to their connections with the ocular motor nuclei. Since the vestibular, optokinetic, and pursuit systems all converge on a common final pathway through the vestibular nuclei, they are all impaired. Due to the loss of the optokinetic and pursuit systems, the sufferer is unable to stop the nystagmus-driven movement of images across the retina. Consequently, central vestibular nystagmus in contradistinction to peripheral...
Definition Yuv
An internuclear ophthalmoplegia INO is present if an eye cannot be adducted when changing gaze direction, but will adduct during accommodative convergence Fig. 11.7 . To detect this diagnostically important superiority of adduction during accommodative convergence, even in subtle lesions of the MLF, the following procedure is advisable. At first, the involved eye is stimulated to adduct as far as possible via a conjugated version by letting the patient follow a distant target to the relevant...
Ophthalmoplegic Migraine
A small number of migraine patients experience an ipsilat-eral oculomotor paresis in connection with an episode of migraine. Like the migraine attacks, this form of paresis appears during childhood as the initiating event in an episode that includes vomiting, photophobia, abdominal pain, irritability, and less commonly headache. The paresis affects all branches and recovers completely, as a rule, within a month's time. Relapses can repeatedly occur, sometimes after years of inactivity, and such...
Transient Visual Loss
By transient visual loss we mean a drop in visual acuity or a loss of visual field, analogous to the transient ischemic attacks TIAs of neurological disease that last no longer than 24 h. This chapter does not discuss transient visual loss for which primary ophthalmic disorders are evident, such as intermittent angle closure glaucoma, vitreous clouding, retinal venous stasis, or the transient obscurations found in papilledema. Patients complaining of transient loss of vision frequently cause...
Ocular Tilt Reaction
In the petrous pyramid of the temporal bone, where the labyrinthine canals converge, are two bony recesses, the utriculus and the sacculus. They contain ciliated sensory receptors. Adherent to the ciliae are tiny lime concrements called otoliths. Gravitational pull on the otoliths bends the ciliae downward, generating a neural signal that defines the static rotational orientation of the head. Depending on the direction of head tilt, the sensory receptors are stimulated or inhibited, allowing...
Info Ffz
Fig. 8.9. Four examples of papillary drusen. Top left is an example of very characteristic features that are immediately recognizable. Top right and bottom left also are examples of visible drusen. In both cases illustrated in the lower images, one can also see subreti-nal hemorrhages in the circumpapillary zone. The bottom right example is not so easily interpreted, since it lacks the usual appearance. The drusen are buried deep within the disc tissue and can be demonstrated only by...
Info Xjm
All types of movement affected saccadic, pursuit, vestibulo-ocular , exophthalmos possible Certain types of movement more or less affected than others supranuclear disease Exophthalmos and or inflammation of orbital tissues No exophthalmos, no inflammation of orbital tissues Diff Dx chronic progressive external ophthalmoplegia, Graves'disease, myasthenia Diff Dx demyelination, infarction, neoplasm, degeneration e.g., progressive supranuclear paralysis, or PSP Diff Dx aneurysm, tumor, ischemic...
Eog Fkq
Central areolar pigment epithelial CAPE dystrophy Benign concentric annular macular dystrophy Bietti's crystalline fundus dystrophy Congenital stationary night blindness CSNB , Riggs type B-wave reduced, delayed dark adaptation in ERG After prolonged dark adaptation, normal Melanoma-associated retinopathy MAR
Subnormal Photoreceptor Functions
Multiple evanescent white dot syndrome MEWDS Normal or reduced b-wave and oscillatory potentials The EOG is the testing method of choice when the primary damage is thought to lie in the retinal pigment epithelium. The most important indications for the EOG are a suspicion of Best's vitelliform macular dystrophy and the suspicion of a pigment epitheliopathy, especially when assessing visual function during chronic chloroquine therapy. The phototransduction process in the photoreceptor outer...
Prolactinoma
A prolactin-secreting tumor of the pituitary gland can cause a dramatic hyperprolactinemia. One third of all hy-perprolactinemias are caused by hypersecreting tumors of the pituitary, and the prolactin level in serum rises to above 200 ng ml. Typical clinical symptoms are amenorrhea and galactorrhea among women, and loss of libido and erectile function in men, and less commonly, gynecomastia. Pro-lactinomas in women, who are often alarmed by the endo-crinological changes, are usually...
Preface
Yet another textbook of clinical neuro-ophthalmology This text and its digital supplement are meant to be used by comprehensive ophthalmologists and residents in training, and are not meant to be used as one would the larger, almost encyclopedic, reference texts with their detailed citations and case reports. Resident physicians should find the format of this text particularly helpful as a learning tool, including the interactive, digital DVD supplement. The material is sufficiently complete as...
Eog
Scotopic flash electroretinogram ERG Inner plexiform layer and especially amacrine cells Scotopic flash ERG, with special filtering Transient pattern ERG Steady-state pattern ERG Posterior pole, cones, bipolar cells, and amacrine cells Visually evoked potentials VEP pattern VEP, flash VEP Primary visual cortex and the cone dominated portion of the afferent pathway central visual field Fig. 7.1. The electrooculogram EOG . Left The principles of the measure. As measured through cutaneous...
Info Fit
0 10 20 30 40 50 60 70 b Green Red-Green Mixture Setting Red 0 10 20 30 40 50 60 70 b Green Red-Green Mixture Setting Red reading of 40 average normal corresponds to an AQ of 1.0, a reading of 73 to an AQ of 0, and a reading of 10 to an AQ of 7.6. For instruments with scale divisions of 0 to 100, 73 must be replaced by the value 100 in the above formula. A normal trichromat will have an AQ between 0.7 and 1.4. In cases of dichromacy, AQ is undefined, since dichromats will accept all matches AQ...
Id
axons by way of the third cranial nerve to the ipsilateral ciliary ganglion from where postganglionic parasympathetic fibers innervate the eye via the short posterior ciliary nerves, terminating at the pupillary sphincter. This anatomically bilateral sharing of neural input has the important consequence that damage to the afferent visual pathways that lead to the Edinger-Westphal nucleus cannot cause an anisocoria. Thus, anisocoria is never a sign of an afferent disturbance, but is always a...
Fundus Albipunctatus Treatment
For macular diseases, however, the multifocal ERG see below is a particularly valuable diagnostic test. During the flash ERG, the following values are recorded The amplitude of the a-wave, measured from the electrical baseline to the negative peak recorded just before the appearance of the b-wave The amplitude of the b-wave, measured from the valley of the a-wave to the positive peak of the b-wave The implicit times, meaning the time from the flash stimulus to the peak of the corresponding ERG...
Pearl Dmq
Only 30 of patients presenting with pituitary tumors complain of visual problems. The tumor must rise more than 1 cm above the diaphragma sellae before causing a clinically detectable loss of visual field and or acuity. Commonly, this is a hormonally inactive tumor that due to the absence of endocrine signs or symptoms has been very inconspicuous. Pituitary microadenomas are those with a diameter of 10 mm or less. The threshold for radiological detection of a microadenoma is 3 mm or less. A...
Segment of the Afferent Visual Pathway
Tumors of the prechiasmal segment of the afferent visual pathway are typically unilateral and for that reason often unnoticed , cause a fluctuating acuity deficit from day to day, and have a slow rate of progression. Monocular visual deficits that vary with the direction of gaze are frequently a sign of a retrobulbar mass, such as a hemangioma or an optic nerve sheath meningioma. Frequently an associated loss of color saturation, especially for red colors, also escapes the patient's notice....
Signs and Symptoms of Nonarteritic Anterior Ischemic Optic Neuropathy
The average age of patients with acute nonarteritic anterior ischemic optic neuropathy NAION is about 61 years. The visual loss occurs abruptly and without a prodromal warning, usually while the patient is asleep or within a 12-h period, and there is no associated pain or headache. The initial swelling of the optic disc subsides within 2 months, leaving a pale, atrophic appearance, which is often sectoral most commonly located in the superior half of the disc . There is for the most part no or...
TolosaHunt Syndrome
This disease is caused by a nonspecific, granulomatous inflammation in the cavernous sinus similar to idiopathic orbital pseudotumor , leading to variable combinations of extraocular muscle pareses on one side with strong, chronic, periorbital pain. The pupil is frequently spared. The syndrome is not common, and the diagnosis can be made only after neuroradiologic exclusion of other types of painful ophthalmoplegia. Spontaneous remission within a few days, relapses including contralateral...
of NeuroOphthalmic Emergencies
Ninety percent of clinical neuro-ophthalmology is in the taking of a history after W.F. Hoyt . Attentive listening, specific questioning and careful evaluation of the information gained make up the foundation of what is primarily a diagnostic subspecialty. The effort invested in gathering this information saves time and avoids unnecessary, potentially dangerous and or expensive diagnostic procedures. When possible, the previous records of the patient's care should be reviewed prior to beginning...
j Note
Typical cases of recurrent retrobulbar optic neuritis RBON should not be treated with lower doses of orally administered medication, since this is known to double the risk of subsequent relapses of demyelination. In the 1990s, the use of beta-interferon and copolymer 1 proved effective at reducing both the frequency and severity of attacks of demyelination in patients with clinically definite MS meaning two or more separate episodes of demyelination . Another study reported in the year 2000...
Testing OKN
Experimental testing of OKN in a research laboratory has traditionally used a rotating drum marked with alternating black and white stripes. For clinical tests of OKN, the examiner uses a patterned ribbon or band of material, held approximately 20 in. away from the eyes. The band is drawn in the direction to be tested, holding it at a stable location with one hand while pulling it past the stabilized location with the other hand Fig. 11.9 . Fig. 11.9. Testing optokinetic nystagmus OKN . The...