Effects of retinal image stabilization in acquired nystagmus due to neurologic disease

We studied the effects of variable amounts of artificial retinal image stabilization (RIS) upon oscillopsia and visual acuity in eight patients with acquired nystagmus due to neurologic disease. We measured horizontal and vertical eye movements with the magnetic search coil technique and used these electronic signals to control the position of a visual stimulus on a screen in front of the patient. We also used an optical device to stabilize images of the real world upon the retina. During electronic stabilization, RIS was progressively increased until oscillopsia was abolished; this was achieved in all eight patients and corresponded to retinal image drift of 5 degrees/sec or less. In five patients with downbeat nystagmus, further increases in RIS caused the oscillopsia to reappear, but in the opposite direction. Electronic stabilization also improved visual acuity in four of five patients; the limitation of improvement could be related to coexistent visual system defects. Using electronic feedback, we could measure the range of RIS that any individual required to abolish oscillopsia; from this measurement, the components of the optical device that were best suited to provide a stable field of vision could be calculated.     

Vision and vertigo

This review deals with two syndromes, oscillopsia and visual vertigo. Oscillopsia is the illusion of oscillation of the visual surroundings. For diagnosis purposes one should ask, when does the oscillopsia occur? If oscillopsia is only present during head (or whole body) movements, the likely underlying cause is a bilateral defect in the vestibulo-ocular reflex (VOR). The more common causes are post meningitic vestibular damage, gentamicin ototoxicity or bilateral idiopathic vestibular failure. When oscillopsia develops after specific head positions, it is usually due to a positional nystagmus, usually the result of brainstem-cerebellar disease. When the oscillopsia is largely unrelated to head movements, one should ask, is it fairly constant or is it in attacks (paroxysmal)? If the oscillopsia is constant it is usually due to the presence of a clinically observable nystagmus; the most common is downbeat nystagmus but the most visually disabling is pendular nystagmus. If the oscillopsia comes in brief attacks it is usually due to a paroxysmal nystagmus as observed in irritative VIII nerve and brainstem lesions. However, the most common cause of paroxysmal oscillopsia is a non organic condition called voluntary nystagmus. Treatment of oscillopsia is often pharmacological but disappointing; the best chance of success is carbamazepine for paroxysmal disorders secondary to structural vestibular nerve/nuclear lesions.Visual vertigo should not be confused with oscillopsia. It can be defined as dizziness provoked by visual environments with large size (full field) repetitive or moving visual patterns. Patients with visual vertigo report discomfort in supermarkets and when viewing movement of large visual objects, eg crowds, traffic, clouds or foliage. Visual vertigo is present in many patients with a history of a peripheral vestibular disorder, particularly those who are visually dependent (ie subjects who use vision preferentially for postural and space orientation control). Patients with visual vertigo benefit from the addition to their standard vestibular rehabilitation of optic flow (optokinetic) stimuli and exercises involving visuo-vestibular conflict.     

Oscillopsia without nystagmus caused by head titubation in a patient with multiple sclerosis.

Oscillopsia in patients who have brain stem disorders but not nystagmus is attributed to a failure of the vestibular-ocular reflex (VOR) to compensate for head movements. We report a patient who had marked head titubation and oscillopsia in aggressive multiple sclerosis but no nystagmus. Her severe head titubation precluded our ability to measure a VOR accurately. Because oscillopsia has also been described after rapid voluntary head oscillations in normal subjects, we queried whether the oscillopsia in our patient could be ascribed to the head movement alone. Six normal control subjects did not experience oscillopsia while shaking their heads at the same frequency as the patient's titubation. We conclude that the oscillopsia in our patient was probably the result of an impaired VOR or an alternative compensatory mechanism.     

Both actual and imagined locomotion suppress spontaneous vestibular nystagmus

Acute unilateral vestibulopathy is characterized by a combination of signs and symptoms including spontaneous nystagmus, postural imbalance and gait disturbance. A functional link between these vestibulo-ocular and vestibulo-spinal motor responses has been demonstrated. These responses use common vestibular input and partially overlapping neuronal networks, but it is not known if and to what extent they are linked or operate separately. We found that slow phase velocity of spontaneous vestibular nystagmus in patients with acute vestibulopathy was suppressed by 26% during actual walking (ANOVA p< 0.02, = 6). It was also suppressed by imagined locomotion: by 26% during the imagination of walking and by 42% during the imagination of running ( p< 0.003, = 10). The suppression of vestibular nystagmus might be beneficial for patients, for it alleviates the disturbing impression of movement of the visual scene (oscillopsia) caused by involuntary eye movements.     

Differential diagnosis of tinnitus and vertigo. A review

Tinnitus and vertigo, two common neurological complaints, often challenge the physician's ability with respect to possible etiology. Objective tinnitus can result from an abnormally patent eustachian tube, from tetanic contractions of the muscles of the soft palate, or from vascular abnormalities within the head or neck. Subjective tinnitus refers to lesions involving the external ear canal, tympanic membrane, ossicles, cochlea, auditory nerve, brainstem, and cortex. As many as 50% of patients with tinnitus do not exhibit associated hearing loss; in these patients, the cause of the tinnitus is rarely identified. An illusion of movement is specific for vestibular system disease--a peripheral or central location depending upon associated audiologic and neurologic symptoms, respectively. However, a presyncopal, light-headed sensation is most commonly associated with diffuse cerebral ischemia: in the young patient, this may be caused by a hyperventilation syndrome; in the aged individual, this can result from diffuse atherosclerotic cerebrovascular disease and decreased cardiac output. Postural and gait imbalance associated with acute vertigo indicates a unilateral peripheral vestibular or a central vestibular lesion; if vertigo is absent, either a cerebellar, proprioceptive, or bilateral peripheral vestibular lesion is likely. Transient oscillopsia suggests unilateral peripheral vestibular lesions. Permanent oscillopsia indicates a bilateral peripheral vestibular lesion or--in the absence of severe vertigo--brainstem or cerebellar damage.     

Drug therapy for acquired pendular nystagmus in multiple sclerosis

Acquired pendular nystagmus (APN) is regularly accompanied by oscillopsia and impairment of static visual acuity. Therapeutic approaches to APN remain controversial, and there is no generally accepted therapeutic approach. We tested 14 patients who had suffered from APN caused by multiple sclerosis for several years; 12 patients presented with fixational pendular nystagmus (increasing during fixation) and 2 with spontaneous pendular nystagmus. All 11 patients with fixational pendular nystagmus who were given memantine, a glutamate antagonist, experienced complete cessation of the nystagmus. In contrast, scopolamine caused no (6 of 8) or only a minor (10–50%) reduction of the nystagmus (2 of 8). It was concluded that memantine is a safe treatment option for APN. Received: 29 August 1995 Received in revised form: 6 August 1996 Accepted: 19 August 1996     

Postural sway during retinal image stabilisation.

Posturographic measurements using a piezoelectric platform were made in normal subjects while wearing a combination of spectacle and contact lens providing partial stabilisation of the retinal image (RIS). The amount of postural sway seen while wearing the device at rest is intermediate between the "normal vision" and "eyes closed" conditions, and increases with increasing amounts of RIS. However, when large active head-and-eye movements are performed, postural sway is dramatically increased when using RIS, and is then worse than while performing the same task in the "eyes closed" condition. It is concluded that patients who use the partial-RIS device for the treatment of severe oscillopsia may benefit only when performing tasks in which the head is relatively still, such as reading, writing or watching TV. It is also proposed that the partial-RIS device can serve as a model in normal free-standing subjects for the postural effects of oculomotor disorders.     

“Innere Perilymphfistel” des anterioren Bogengangs Ein neues Krankheitsbild mit rezidivierenden Schwindelattacken

In 1998 Minor et al. described a new variant of perilymphatic fistula: the "superior canal dehiscence syndrome". This syndrome is clinically characterized by recurrent attacks of vertigo and oscillopsia induced by loud noises or stimuli that result in changes in intracranial or middle ear pressure. It is caused by a dehiscence of bone overlying the superior (anterior) semicircular canal. Due to this dehiscence, a third, mobile window (in addition to the round and oval windows) is formed, and changes in pressure are pathologically transduced to the anterior semicircular canal. Although the superior canal dehiscence syndrome is not a rare condition, no other cases have yet been reported. Therefore, we describe a typical patient who suffered for many years from recurrent attacks of vertigo and oscillopsia induced by coughing and Valsalva's maneuvers. High resolution temporal bone CT scan showed a defect in the bone overlying the left anterior semicircular canal. Three-dimensional eye movement recordings using the search coil technique and subsequent vector analysis demonstrated that the eye movements were induced by excitation of the left anterior semicircular canal. We conclude that superior canal dehiscence syndrome is an important differential diagnosis in patients suffering from symptoms of a perilymphatic fistula, especially since it can be successfully treated by "plugging" of the affected semicircular canal. Such patients are thus spared unnecessary surgery of the middle ear.     

Localizing forms of nystagmus: Symptoms, diagnosis, and treatment

Nystagmus is a rhythmic oscillation of the eyes that may be acquired or congenital. The symptoms of acquired nystagmus include blurred vision and oscillopsia. Most patients with congenital nystagmus do not have oscillopsia symptoms. Several forms of nystagmus have localizing value and any neuroimaging should be directed to the topographic localization suggested by the morphology of the nystagmus and any accompanying localizing signs. Several optical, medical, and surgical treatments have been used with some success in specific forms of nystagmus.     

Localizing forms of nystagmus: symptoms, diagnosis, and treatment

Nystagmus is a rhythmic oscillation of the eyes that may be acquired or congenital. The symptoms of acquired nystagmus include blurred vision and oscillopsia. Most patients with congenital nystagmus do not have oscillopsia symptoms. Several forms of nystagmus have localizing value and any neuroimaging should be directed to the topographic localization suggested by the morphology of the nystagmus and any accompanying localizing signs. Several optical, medical, and surgical treatments have been used with some success in specific forms of nystagmus.     

Oscillopsia: pathophysiological mechanisms and treatment

Oscillopsia is an illusion of an unstable visual world. It is associated with poor visual acuity and is a disabling and stressful symptom reported by numerous patients with neurological disorders. The goal of this paper is to review the physiology of the systems subserving stable vision, the various pathophysiological mechanisms of oscillopsia and the different treatments available. Visual stability is conditioned by two factors. First, images of the seen world projected onto the retina have to be stable, a sine qua non condition for foveal discriminative function. Vestibulo-ocular and optokinetic reflexes act to stabilize the retinal images during head displacements; ocular fixation tends to limit the occurrence of micro ocular movements during gazing; a specific system also acts to maintain the eyes stable during eccentric gaze. Second, although we voluntary move our gaze (body, head and eye displacements), the visual world is normally perceived as stable, a phenomenon known as space constancy. Indeed, complex cognitive processes compensate for the two sensory consequences of gaze displacement, namely an oppositely-directed retinal drift and a change in the relationship between retinal and spatial (or subject-centered) coordinates of the visual scene. In patients, oscillopsia most often results from abnormal eye movements which cause excessive motion of images on the retina, such as nystagmus or saccadic intrusions or from an impaired vestibulo-ocular reflex. Understanding the exact mechanisms of impaired eye stability may lead to the different treatment options that have been documented in recent years. Oscillopsia could also result from an impairment of spatial constancy mechanisms that in normal condition compensate for gaze displacements, but clinical data in this case are scarce. However, we suggest that some visuo-perceptive deficits consecutive to temporo-parietal lesions resemble oscillopsia and could result from a deficit in elaborating spatial constancy.     

Novel subtype of idiopathic bilateral vestibulopathy: bilateral absence of vestibular evoked myogenic potentials in the presence of normal caloric responses

To characterize clinical features of those patients who showed an absence of vestibular evoked myogenic potential (VEMP) responses in the presence of normal caloric responses bilaterally, we reviewed clinical records of 1,887 consecutive outpatients who complained of balance problems, and identified three patients, who showed absent VEMPs in the presence of normal caloric responses bilaterally with unknown causes. All three patients had episodes of recurrent vertigo without spontaneous, gaze-evoked, or positional nystagmus at the time of examination. They complained of oscillopsia while moving their body or head and showed positive Romberg’s signs. Drawing on these cases, we underscore the importance of examining the function of the inferior vestibular nerve system, even with no nystagmus and normal caloric findings, in patients complaining of dizziness or oscillopsia during locomotion.     

Pendular pseudonystagmus arising as a combination of head tremor and vestibular failure.

We describe three patients with spontaneous pendular oscillation of the eye during funduscopy. All patients had blurred, shimmering vision or oscillopsia, exacerbated by concentration, reading, or trivial head movements, and had a history of unsteadiness. Examination revealed a fine head tremor, mild unsteadiness, absent vestibulo-ocular reflex (VOR), and otherwise normal neurologic and ocular motor findings. Rigid immobilization of the head abolished the retinal oscillations. Simultaneous precision recordings of head and eye movements showed that the eye movement was in the compensatory direction to the head tremor but that, in contrast to normal VOR, it was in phase error. We conclude that the essential head tremor was provoking oscillopsia and retinal oscillation because of the absence of VOR. Recognizing the association of head tremor with absent VOR is important since in all these patients the presence of this pendular pseudonystagmus on ophthalmoscopy raised the diagnostic possibility of brainstem disease.     

Hardware Failures in Spinal Cord Stimulation for Failed Back Surgery Syndrome

Spinal cord stimulation (SCS) is an efficient means for treatment of the postsurgical lumbar spine condition known as failed back surgery syndrome (FBSS). Although the devices and the implantation techniques are well established and the technology is sophisticated, there are some complications caused by hardware failures. This study was aimed at identifying the most frequent types of hardware failures and their causes in FBSS patients treated with SCS. In a retrospective analysis, a group of 42 FBSS patients using single lead SCS for 6–74 months was evaluated. Only hardware failures were considered in the analysis, and parameters such as occurrence of failure after primary implantation of the device, frequency and site of failure, stimulation time to failure (TF), and overall time of SCS usage were recorded. In the patient group studied, 12 surgical corrections of the hardware were carried out in 10 patients. In eight patients there was a single corrective procedure, in two additional cases there were two surgically corrected hardware failures each. The most often encountered type of hardware failure was lead breakage or disruption of insulation (percutaneously placed Quad leads only) leading to short circuiting and dysfunction (n= 8). Second in frequency were receiver (model 3470) failures due to insulation leakage at the plug connection site (n= 2). In one case, extension cable breakage caused dysfunction of the system, and another dysfunction was caused by distal extension cable disconnection. In conclusion, SCS is a low‐complication procedure for treatment of benign low‐back pain, but seems to be prone to lead and insulation failures.     

The characteristics and mechanisms of visual disturbance associated with anticonvulsant therapy

Eight epileptic patients receiving anticonvulsants had recurrent visual disturbances in the form of diplopia and oscillopsia in the horizontal or vertical planes. The symptoms could be ascribed to impaired vergence mechanisms, vertical nystagmus, or abnormalities of the vestibulo-ocular reflex. Other eye movements, such as pursuit and gaze-holding, were also affected, but did not lead to complaints. Episodes of visual disturbance were often preceded by prodromes of ocular or systemic discomfort, after which oscillopsia or diplopia evolve rapidly. The symptomatology was stereotyped but unique for each patient and may reflect idiosyncratic susceptibility to the ocular motor side effects of anticonvulsants. Six of the 8 patients were taking carbamazepine and phenytoin in combination, which have similar effects on the ocular motor system.     

Cardiac uptake of [123I]MIBG separates Parkinson's disease from multiple system atrophy.

OBJECTIVE: To improve the differential diagnosis between patients with multiple system atrophy (MSA) and idiopathic PD (IPD) with autonomic failure. BACKGROUND: Some patients diagnosed with IPD are discovered to have alternative diseases such as MSA, despite the application of stringent diagnostic criteria. This differentiation is particularly difficult if patients with IPD also show symptoms of autonomic failure. In IPD, autonomic failure is caused by damage of the postganglionic part of the autonomic nervous system, whereas in MSA, degeneration of preganglionic and central autonomic neurons is revealed histopathologically. METHODS: Scintigraphy with [123I]metaiodobenzylguanidine (MIBG) enables the quantification of postganglionic sympathetic cardiac innervation. Fifteen patients with IPD and 5 patients with MSA underwent standard autonomic function tests and scintigraphy with MIBG. RESULTS: In all patients, cardiovascular testing showed evidence of autonomic failure of varying severity. In all patients with IPD, the heart-mediastinum (H/M) ratio of MIBG uptake was pathologically impaired, independent of duration and severity of autonomic and parkinsonian symptoms. All patients with MSA had a regular H/M ratio. Each patient could be assigned to the correct diagnostic group based on the results of the MIBG scintigraphy, even if the duration of the disease was only 2 years or less. CONCLUSIONS: This population assessment of the heart-mediastinum ratio of [123I]metaiodobenzylguanidine uptake showed a high sensitivity for the detection of autonomic involvement in patients with idiopathic IPD and also a high specificity for the discrimination between idiopathic PD and MSA.     

Bilateral loss of vestibular function: clinical findings in 53 patients

The clinical presentations and aetiologies of a series of 53 cases of bilateral vestibular failure (BVF) seen by the authors over a decade were evaluated by retrospective review of the medical records. Thirty-nine per cent of patients had associated neurological disease; 13% had a progressive cerebellar syndrome with disabling gait ataxia, abnormal eye movements and cerebellar atrophy on neuro-imaging. BVF was usually unsuspected. Nine per cent had cranial or peripheral neuropathies and in this group there was no abnormality of brain stem/cerebellar oculomotor function, but hearing loss was common. Eleven per cent revealed BVF and hearing loss secondary to meningitis, and 6% had other neurological disorders. Idiopathic BVF was found in 21% of cases, characterised by paroxysmal vertigo and/or oscillopsia, but no abnormal clinical signs. Gentamicin ototoxicity accounted for a further 17%, while autoimmune disease was present in 9% of patients. Otological or neoplastic disease was diagnosed in the remaining 13% of patients. It was concluded that neurological, audiological and ocular motor assessments allow the probable cause of BVF to be defined in approximately 80% of cases. A group of BVF related to autoimmune pathologies is reported for the first time, indicating the need for immunological screening. Idiopathic BVF may present with only minor visual or vestibular symptoms, while in patients with cerebellar degeneration, BVF may be unsuspected and, thus, underdiagnosed. Accepted: 8 December 1997 Received: 31 January 1997 Received in revised form: 25 November 1997     

Enhanced smooth pursuit eye movements in patients with bilateral vestibular deficits

Patients with bilateral vestibular deficits experience unsteady gait and oscillopsia that can reduce the quality of life, though many patients adapt remarkably well and lead mostly normal lives. One source of adaptation could be the ability of sensory-motor systems to compensate for the vestibular loss by adaptive enhancement of their performance. We studied smooth-pursuit eye movements in five patients and six healthy control subjects using a step-ramp paradigm. Eye movements were measured with scleral search coils. Patients showed open- and closed-loop pursuit gains that were about 9% higher than controls. We suggest that the challenge of living with a deficient vestibular system caused an enhancement in the pursuit system, which contributes to the patient's overall compensation.     

Monocular visual activation patterns in albinism as revealed by functional magnetic resonance imaging

Human albinism is characterized by a disturbance of the chiasmatic projection system leading to predominant representation of just one eye in the contralateral hemisphere. Patients show congenital nystagmus without perceiving oscillopsia. The purpose of the present study was to demonstrate the consequences of atypical chiasmatic crossing with monocular visual stimulation using functional magnetic resonance imaging (fMRI). Sixteen patients with albinism and fifteen normally pigmented controls were stimulated with a monocular visual activation paradigm using flickering checkerboards. In patients, we observed contralaterally dominated activation of visual cortices correlating to clinical albinism parameters. This confirms albinism as a continuous range of hypopigmentation disorders. Additionally, albinos showed activation of the superior colliculus and of visual motion areas although the stimulus was stationary. Activation of visual motion areas is due probably to congenital nystagmus without a conscious correlate like oscillopsia.     

Vertical oscillopsia in bilateral superior canal dehiscence syndrome

A patient sought treatment for vertical oscillopsia and impaired vision during locomotion, and unsteadiness of gait. Positive fistula tests and CT of the temporal bones confirmed a diagnosis of bilateral superior canal dehiscence. An impairment of the superior canal vestibulo-ocular reflex, documented by three-dimensional search coil eye movement recordings for oblique (single) and downward pitch head motion (bilateral canal testing), is proposed to induce vertical rather than torsional-vertical oscillopsia during locomotion.