Room tilt illusion. A central otolith dysfunction

BACKGROUND: We report a sudden 90 degrees room tilt illusion (RTI) following vestibular stimulation in 3 patients with persistent skew deviation caused by a brain stem lesion. Room tilt illusion is a transient tilt perception of the visual surrounding, on its side or even upside down, that is often reported with brain stem lesions. Although its pathophysiologic cause is not well known, the RTI suggests an impairment of otolith pathways, as reported in skew deviation. METHODS: The 3 patients with brain stem lesions were reexamined as part of a follow-up of patients with signs of otolith dysfunction. A registration of vestibular function was performed with a rotatory chair, including earth-vertical axis rotation for canal stimulation and off-vertical axis rotation (OVAR) for otolith stimulation. Measurement of the subjective visual vertical (SVV) was also performed. RESULTS: The otolith-ocular reflex registered by OVAR was impaired in the 3 patients with skew deviation and the SVV in 2 patients. After each direction of OVAR stimulation, the 3 patients reported an RTI as the room was illuminated. CONCLUSIONS: The coexistence of otolith oculomotor (skew deviation and impaired otolith-ocular reflex) and perceptual (tilt of SVV and RTI) disorders suggests a common otolith dysfunction. However, an RTI occurred specifically after vestibular stimulation and when the room was illuminated. We thus suggest that RTI reflects a dynamic visuo-otolith mismatch.     

Partial ocular tilt reaction due to unilateral cerebellar lesion

We report on two patients each with tonic, contraversive partial ocular tilt reactions due to unilateral cerebellar lesions: one patient had had a caudal cerebellar hemorrhage, the other a posterior inferior cerebellar artery territory infarct. Both patients had tonic contraversive conjugate ocular torsion; one had skew deviation; neither had a head tilt. One patient had no specific neurologic deficit apart from the conjugate ocular torsion, which was first suspected because of a deviation of the subjective visual horizontal. These observations imply that the ocular tilt reaction (OTR), a brainstem otolith-ocular reflex of probable utricular origin, is under the inhibitory control of the ipsilateral caudal cerebellum, possibly the nodulus, and that a patient with a cerebellar infarct can present with imbalance as the only neurologic symptom and with conjugate ocular torsion as the only specific neurologic sign.     

Three-dimensional modeling of static vestibulo-ocular brain stem syndromes

Static vestibulo-ocular brain stem syndromes characterized by skew deviation, a vertical disconjugacy of the eyes, and ocular torsion are the result of a vestibular tone imbalance in the frontal (roll) plane. Similar physiological changes in static eye position, ocular counter-roll and conjugated deviations of vertical eye position, are caused by the influence of gravity mediated by the utricles. These observations prompted our approach with the model described here: based on the known deviations of static eye position, we devised a three-dimensional mathematical model of otolith-ocular function including detailed brain stem anatomy. This model is able to explain and predict the differential effects of unilateral and bilateral peripheral or central vestibular lesions on static eye position in roll, pitch, and yaw planes.     

Epileptic skew deviation

We describe a 43-year-old neurologically intact patient who reported episodes of diplopia and oscillopsia associated with a right-beating nystagmus and a skew deviation. These symptoms and signs were related to a left posterior epileptic EEG discharge. We suggest that these ocular motor signs derived from an ictal activation of the vestibular cortex, which in turn activated descending projections to the vestibular nuclei, leading to both a dynamic (right-beating nystagmus) and a static (skew deviation) vestibular imbalance.     

Peripheral vision in children

A comparison of peripheral vision in adults and children showed that children had reduced sensitivity in a standard visual field examination. The constriction of the visual field amounted to 0.4 log units at 30 degrees excentricity in a static perimetric test. This loss was maintained in a spatial forced choice experiment without foreknowledge of target location. However, in a temporal forced choice experiment with foreknowledge of target position the difference between children and adults was reduced to 0.1 log units. It is thought, therefore, that the former visual loss in children is due to the nature of the task rather than to deficiencies in the visual mechanisms.     

Profile of some risk factors for coronary heart disease in a developing country: Nigeria

We examined visual evoked potentials and pattern electroretinograms in a patient with Tolosa-Hunt syndrome associated with optic nerve involvement. The 82-year-old woman developed unilateral painful ophthalmoplegia and visual loss in the right eye. Magnetic resonance imaging showed an abnormal soft-tissue area in the right cavernous sinus and the right orbital apex. Symptoms responded rapidly to treatment with corticosteroid. Visual evoked potentials to flash and pattern stimuli were both remarkably reduced and delayed in the right eye in the acute stage; however they improved to almost normal after steroid therapy. The pattern electroretinogram recorded in the acute stage was normal bilaterally. These results indicate that optic nerve involvement in Tolosa-Hunt syndrome can be mild and reversible.     

Defective phagocytosis in systemic lupus erythematosus

The authors describe a case of traumatic retinal dialysis with retinal detachment from a water balloon slingshot during a "water balloon war." A 31-year-old woman presented with decreased visual acuity in her right eye 2 days after being hit by a water balloon. The visual acuity in the right eye was counting fingers and fundus examination showed subtotal retinal detachment secondary to a superonasal dialysis. The patient underwent a scleral buckling procedure with external drainage, and at 18 months visual acuity was stable at 20/50 with attached retina. Water balloon eye injuries can result in permanent visual loss. More public awareness needs to be created regarding the potential harmful effects of this commonly used "toy."     

Inflammatory sensory ataxic neuropathy presenting with alternating skew deviation on lateral gaze: a case report

We report a 56-year-old female with chronic progressive sensory ataxic neuropathy presenting with alternating skew deviation on lateral gaze in the clinical course. She initially developed dysesthesias in the hands and feet asymmetrically, then gait disturbance developed over several months, and she was admitted to our hospital. Neurological examinations revealed profound deep sensory loss and mild superficial sensory disturbance with the absence of deep tendon reflexes, but muscular strength was completely preserved. EMG showed no evoked response of sensory nerve velocities and normal motor nerves. Sural nerve biopsy showed moderate demyelination with mild infiltration of inflammatory cells, and no vasculitis or onion bulb formation. CSF examination revealed elevation of cell counts and protein with marked intrathecal IgG synthesis and myelin basic protein, but finding of neurosyphillis. Serological examinations did not show any evidence of collagen disease, paraproteinemia, retrovirus infections or Lyme disease. Serum antiganglioside antibodies and anti-Hu antibody were negative. No evidence of malignancy was seen by radiological examinations and assays of tumor markers. In the weeks after admission, gait ataxia progressively worsened, and then she developed alternating skew deviation on lateral gaze, suggesting that the CNS was involved. No responsible lesion was detected on MRI. Corticosteroid administration improved not only the CSF findings, but also the neurologic symptoms, including the alternating skew deviation on lateral gaze. Although the disease entity was not identified, inflammatory demyelinating processes and immune-mediated mechanisms were considered to play important roles.     

Asymmetrical vertical phorias indicating dissociated vertical deviation in subjects with normal binocular vision

We measured the symmetry of phoria angles in six normal subjects. Subjects were selected on the basis of good visual acuity and stereopsis, normal binocular eye alignment and, apart from mild refraction errors, absence of ocular abnormalities. They were instructed to look at a word on a reading chart at 2 m distance. Each measurement consisted of five subsequent intervals of 5 s duration. During these five intervals viewing was binocular, with the right eye only, binocular, with the left eye only, and binocular, respectively. Each experiment consisted of twelve measurements. Eye movements were measured with scleral coils suited for measuring in horizontal, vertical and torsional directions. Five out of six subjects displayed an asymmetrical vertical phoria; one subject showed an alternating hyperphoria; four displayed a left over right vertical phoria that was largest for left eye occlusion. Only one subject showed a symmetrical vertical phoria. Both the size of the vertical phorias and the size of the asymmetries in these vertical phorias were very small: on average 0.16 +/- 0.01 and 0.17 +/- 0.01 degree, respectively. The direction of the vertical phoria asymmetries (the largest left over right was found with left eye occlusion) and the fact that asymmetries were found more often in vertical than horizontal and torsional phorias suggest that these asymmetries are related to dissociated vertical deviation. These results suggest that dissociated vertical deviation, often observed in subjects with a disruption of binocular vision early in life, reflects the enhancement of a phenomenon that is present in normal subjects as well.     

The effect of immunotoxins directed against CD80 and CD86 on primary T-cell alloresponses

A 13-year-old boy presented with acute loss of vision in his right eye of 2 weeks' duration. He had a high fever and was ill for several days, then improved but suffered recurrent episodes of sweating and a high fever. Ophthalmoscopy of the right eye showed optic disk edema, mild vitreous cells, and minimal exudates in the macula. Bartonella henselae titers were positive. A diagnosis of optic disk edema with a macular star secondary to cat-scratch disease was made. The patient was treated with doxycycline and made a dramatic improvement to visual acuity of 20/30 with a minimal residual relative central scotoma. The optic disk edema and macular star resolved, and the patient was left with mild optic atrophy in the right eye.     

Diagnosis and therapy of antiphospholipid antibody syndrome

PURPOSE: To report alterations of electrophysiologic tests, including the multifocal electroretinogram, in a case of acute zonal occult outer retinopathy. METHOD: We recorded photopic, scotopic, and single-flash electroretinograms and a multifocal electroretinogram in a 47-year-old woman with acute zonal occult outer retinopathy in the right eye. RESULTS: Her visual acuity was 20/20 in the right eye throughout the follow-up period. The electroretinograms indicated retinal impairment of the right eye, predominantly in the cones. The multifocal electroretinogram showed reduced responses corresponding to the visual field defect of the static perimetry. CONCLUSIONS: In acute zonal occult outer retinopathy, impairment of the retinal area results in a visual field defect. The multifocal electroretinogram can be useful in determining the location of the defect.     

Equivalence in periodontal trials: a description for the clinician

AIMS: To describe the clinical picture and electrophysiological findings in Müller cell sheen dystrophy, a recently reported retinal dystrophy. METHOD: A basic ophthalmological evaluation as well as recording of standard electro-oculography and electroretinography were performed in one patient at the onset of visual loss and after 1 year of follow up. RESULTS: A 61 year old woman presented with visual loss in the right eye. Multiple folds at the level of the internal limiting membrane were seen at the posterior pole in both eyes. Macular oedema was present in the right eye. The visual acuity of the right eye was 6/30 and of the left 6/9. A paracentral scotoma was found in the right eye. Electro-oculographic examination of both eyes gave normal results. Electroretinography (ERG) revealed reduced b-wave and flicker amplitudes in the right eye; these potentials were normal for the left eye. The ON response in the right eye was reduced and delayed; it was normal in the left eye. A further loss of visual function was noted 1 year later in the right eye, but the ophthalmoscopic findings were unchanged. The ERG of the right eye had a negative waveform when dark adapted. Light adapted responses showed an unusual delayed b-wave, broad and delayed ON and OFF responses and a missing flicker response, suggesting a Müller cell dysfunction. Light adapted responses were slightly reduced in the left eye. CONCLUSIONS: Electrophysiological data indicate Müller cell dysfunction as a background of functional loss in Müller cell sheen dystrophy. This is in agreement with previously reported histological findings in this disorder.     

Krypton laser suture lysis

PURPOSE: To analyze indocyanine green angiography (ICGA) features in two cases of sympathetic ophthalmia using a standard angiography protocol for posterior uveitis. METHODS: Report on two patients who suffered from penetrating ocular injuries 45 and 8 years before sympathetic ophthalmia was diagnosed and confirmed by histopathological examination of the enucleated eye. In addition to routine examination and fluorescein angiography, initial and follow-up ICGAs were performed. RESULTS: The first patient, with a phthisic right eye following s shotgun injury, consulted 6 months after cataract extraction in his good left eye for progressive visual loss due to a neovascular membrane in a moderately inflamed eye. The second patient consulted 8 years after a perforating injury of his right eye by a metallic foreign body because of recent visual loss and inflammation in his good left eye. ICGA of both patients showed numerous hypofluorescent dark dots visible at the intermediate phase, some becoming isofluorescent at the late phase and resolving after long-term corticosteroid therapy, others remaining hypofluorescent until the late phase. CONCLUSION: The two patterns of hypofluorescent areas, either persisting throughout angiography or fading in the late phase, were interpreted respectively as cicatricial and active lesions. ICGA gave determining additional information on choroidal involvement and on subsequent evolution of lesions.     

Visual fields in neuro-ophthalmology

1. The gathering and interpretation of visual fields is important to a thorough neuro-ophthalmic evaluation. The technique of visual field measurement is essential to the proper characterization of visual field defects and must be tailored to each patient in respect to his or her ability to perform a particular test. 2. The techniques for visual field measurement include Amsler grid, confrontation using fingers or colored objects, tangent screen, manual bowl perimeters, and automated static perimeters. 3. The ability to characterize a visual field defect and interpret its relationship to disease involves a combination of knowledge of the anatomy of the visual pathways and the use of the appropriate visual field examination strategy. Once it has been determined--by simple and expeditious confrontation visual field techniques--whether the defect is present centrally or only in the peripheral visual field, a selection of more formal visual field examination can be made.     

Retention and stability: a review of the literature

The subjective visual horizontal (SVH) was measured by means of a small rotatable luminous line in darkness in the upright body position and at 10, 20 and 30 degrees of body tilt to the right and left prior to, and during a follow-up period after, stapedotomy in 12 patients with otosclerosis. In the acute stage after surgery, SVH in the upright body position was significantly tilted away from the operated side. In addition, the perception of roll tilt towards the operated side (Kop) was significantly increased after stapedotomy, while the perception of roll tilt towards the healthy side (Khe) showed a slight but not significant reduction. After exclusion of two outliers, a statistically significant correlation was found between changes in Kop and in Khe. The slope of the regression line was 1.8:1, probably corresponding to a preference of the utricle for ipsilateral as opposed to contralateral head tilt. In four patients there was a weak ( < 1 degrees/s) spontaneous nystagmus, not systematically related to the side of surgery, while in most cases there were no nystagmus or subjective vertigo symptoms. These specific changes in the subjective horizontal show that the otolithic effects on perception can be dissociated from canal effects. Further, the results are opposite to those for patients with unilateral loss of vestibular function. The tilt of SVH after stapedotomy indicates an increase in resting activity of utricular afferents. In addition, based on recent theories on otolith function, we suggest that an increased activity in saccular afferents is of major importance for the changes in roll-tilt perception because of its interaction with the utricle on the central nervous level.     

Frame-of-reference and hierarchical-organisation effects in the rod-and-frame illusion

Two hypotheses proposed as alternatives by Rock--frame of reference and hierarchical organisation of perception--were tested in a series of experiments with the use of the rod-and-frame illusion. This illusion produces errors in the apparent vertical due to the presence of a tilted frame surrounding the test rod. The apparent vertical is shifted in the direction of the frame tilt. When an upright square was added inside the tilted frame, rod-setting errors varied according to the visual characteristics of the display. In the case of a large display presented in the dark (experiment 1), there continued to be large errors in the direction of the outer-square tilt. This finding supports the frame-of-reference hypothesis, which proposes that the orientation of all objects in the visual field is dominated by the most peripheral reference. In the case of a small display presented in a lit environment (experiments 2 and 3), the direction of errors was the opposite. This latter finding was taken to indicate that the rod was set with reference to the perceived tilt of the inner upright square. Thus, according to a hierarchical-organisation hypothesis, the orientation of an object in the visual field is influenced by objects in the immediate surroundings not by outermost reference. Overall, the results confirm the presence of two qualitatively different classes of orientational phenomena: one is concerned with the definition of egocentric coordinates and one with an object-centred visual representation.     

Retinal ganglion cell layer and visual function in a patient with optic disc drusen

BACKGROUND: To correlate the retinal ganglion cell pattern to visual acuity and visual field data in a patient with bilateral optic disc drusen, a quantitative clinicopathological study was carried out. METHODS: Both retinae of a patient with optic drusen were whole-mounted. Retinal ganglion cell counts were made using a sampling scheme covering the whole retina and compared to the findings in 10 normal retinae. Relative ganglion cell reduction in the drusen retinae was correlated to clinical data. RESULTS: The total retinal ganglion cell count was reduced from 1244858+/-98736 in normal retinae to 305319 on the right and 527571 on the left eye with optic disc drusen. Large ganglion cells had a better chance of survival. Parafoveal ganglion cell loss was 57% for the right and 36% for the left eye, while visual acuity was 0.8 and 1.0 respectively. The mean light sensitivity loss increased from the centre (6.2 dB) to paracentral (9.9 dB), mid-peripheral (13.7 dB) and outer peripheral (15.0 dB) retina, while ganglion cell losses were smallest in outer peripheral retina (21.9%), followed by central (53.0%), mid-peripheral (70.9%) and paracentral retina (87.7%). CONCLUSION: These data validate Frisén's theory on central retinal resolution and provide the structural basis for the clinical rule that low visual acuity should not be attributed to disc drusen. Automated light sense perimetry gives an inadequate picture of retinal damage caused by optic disc drusen.     

Rostral fastigial nucleus activity in the alert monkey during three-dimensional passive head movements

The fastigial nucleus (FN) receives vestibular information predominantly from Purkinje cells of the vermis. FN in the monkey can be divided in a rostral part, related to spinal mechanisms, and a caudal part with oculomotor functions. To understand the role of FN during movements in space, single-unit activity in alert monkeys was recorded during passive three-dimensional head movements from rostral FN. Seated monkeys were rotated sinusoidally around a horizontal earth-fixed axis (vertical stimulation) at different orientations 15 degrees apart (including roll, pitch, vertical canal plane and intermediate planes). In addition, sinusoidal rotations around an earth-vertical axis (yaw stimulus) included different roll and pitch positions (+/-10 degrees, +/-20 degrees). The latter positions were also used for static stimulation. One hundred fifty-eight neurons in two monkeys were modulated during the sinusoidal vertical search stimulation. The vast majority showed a uniform response pattern: a maximum at a specific head orientation (response vector orientation) and a null response 90 degrees apart. Detailed analysis was obtained from 111 neurons. On the basis of their phase relation during dynamic stimulation and their response to static tilt, these neurons were classified as vertical semicircular canal related (n = 79, 71.2%) or otolith related (n = 25; 22.5%). Only seven neurons did not follow the usual response pattern and were classified as complex neurons. For the vertical canal-related neurons (n = 79) all eight major response vector orientations (ipsilateral or contralateral anterior canal, posterior canal, roll, and nose-down and nose-up pitch) were found in Fn on one side. Neurons with ipsilateral orientations were more numerous and on average more sensitive than those with contralateral orientations. Twenty-eight percent of the vertical canal-related neurons also responded to horizontal canal stimulation. None of the vertical canal-related neurons responded to static tilt. Otolith-related neurons (n = 25) had a phase relation close to head position and were considerably less numerous than canal-related neurons. Except for pitch, all other response vector orientations were found. Seventy percent of these neurons responding during dynamic stimulation also responded during static tilt. The sensitivity during dynamic stimulation was always higher than during static stimulation. Sixty-one percent of the otolith-related neurons responded also to horizontal canal stimulation. These results show that in FN, robust vestibular signals are abundant. Canal-related responses are much more common than otolith-related responses. Although for many canal neurons the responses can be related to single canal planes, convergence between vertical canals but also with horizontal canals is common.     

Acute visual change

Changes in vision or loss of vision are common complaints for patients presenting to the emergency department. Such complaints may represent a simple problem related to recent trauma, early evidence of a systemic disease, or may be a vision-threatening lesion. A logical and organized approach to the history and the physical examination of the patient with eye complaints is key to the diagnosis and treatment of the more significant causes of these complaints. This article reviews an organized approach to the patient with complaint of visual loss and considers the wide differential diagnosis. It focuses specifically on the evaluation of the patient complaining of visual changes resulting from central retinal artery occlusion, central retinal vein occlusion, retinal detachment, acute angle-closure glaucoma, giant cell temporal arteritis, and retrobulbar hemorrhage.     

A case of malignant lymphoma with a metastasis to the lateral rectus muscle

We reported a 75-year-old woman with malignant lymphoma who had a metastasis to the right lateral rectus muscle. She was well until two months earlier, when a tumor in the left thigh began to enlarge. Ten days before admission, she noticed medial deviation of the right eyeball. Neurological examination showed the right esotropia with isolated paralysis of the right lateral gaze. She denied double vision. MR imaging demonstrated a swelling of the right lateral rectus muscle. Gallium scanning revealed abnormal accumulation in the right orbit and the left thigh. The tumor in the left thigh was histologically diagnosed as non-Hodgkin's lymphoma, diffuse large cell type. Discrete extraocular muscle metastasis is rare and unreported for malignant lymphoma. Reported cases of breast and thyroid cancers metastatic to the extraocular muscles did not develop diplopia similar to our case. The rapid growth of metastases to the extraocular muscles produces a large visual axes deviation, therefore no diplopia may be elicited.