Phillip D. Kramer

MRI characteristics of the MLF in MS patients with chronic internuclear ophthalmoparesis

Objective: The authors imaged the medial longitudinal fasciculus (MLF) in 58 patients with MS and chronic internuclear ophthalmoparesis (INO) to determine which MRI technique best shows the characteristic lesion associated with this ocular motor syndrome. Methods: Using quantitative infrared oculography, the authors determined the ratios of abduction to adduction for velocity and acceleration, to confirm the presence of INO and to determine the severity of MLF dysfunction in 58 patients with MS and INO. Conventional MRI techniques, including proton density imaging (PDI), T2-weighted imaging, and fluid-attenuated inversion recovery (FLAIR) imaging, were used to ascertain which technique best shows MLF lesions within the brainstem tegmentum. T1-weighted imaging was performed to determine the frequency of brainstem tegmentum hypointensities. Results: All patients studied had evidence of an MLF lesion hyperintensity on PDI, whereas T2-weighted imaging and FLAIR imaging showed these lesions in 88% and 48% of patients, respectively. With PDI, dorsomedial tegmentum lesions were seen in the pons in 93% of patients and in the midbrain of 66% of patients. Lesions were observed at both locations in 59% of patients. One patient had an MLF lesion with a corresponding T1 hypointensity. Conclusions: PDI best shows the MLF lesion in patients with MS and INO.

Quantitative oculographic characterisation of internuclear ophthalmoparesis in multiple sclerosis: the versional dysconjugacy index Z score

Background: There is a poor correlation between multiple sclerosis disease activity, as measured by magnetic resonance imaging, and clinical disability. Objective: To establish oculographic criteria for the diagnosis and severity of internuclear ophthalmoparesis (INO), so that future studies can link the severity of ocular dysconjugacy with neuroradiological abnormalities within the dorsomedial brain stem tegmentum. Methods: The study involved 58 patients with multiple sclerosis and chronic INO and 40 normal subjects. Two dimensional infrared oculography was used to derive the versional dysconjugacy index (VDI)—the ratio of abducting to adducting eye movements for peak velocity and acceleration. Diagnostic criteria for the diagnosis and severity of INO were derived using a Z score and histogram analysis, which allowed comparisons of the VDI from multiple sclerosis patients and from a control population. Results: For a given saccade, the VDI was typically higher for acceleration v velocity, whereas the Z scores for velocity measures were always higher than values derived from comparable acceleration VDI measures; this was related to the greater variability of acceleration measures. Thus velocity was a more reliable measure from which to determine Z scores and thereby the criteria for INO and its level of severity. The mean (SD) value of the VDI velocity derived from 40 control subjects was 0.922 (0.072). The highest VDI for velocity from a normal control subject was 1.09, which was 2.33 SD above the normal control mean VDI. We therefore chose 2 SD beyond this value (that is, a Z score of 4.33) as the minimum criterion for the oculographic confirmation of INO. Of patients thought to have unilateral INO on clinical grounds, 70% (16/23) were found to have bilateral INO on oculographic assessment. Conclusions: INO can be confirmed and characterised by level of severity using Z score analysis of quantitative oculography. Such assessments may be useful for linking the level of severity of a specific clinical disability with neuroradiological measures of brain tissue pathology in multiple sclerosis.

Accuracy of clinical detection of INO in MS Corroboration with quantitative infrared oculography

The authors compared the accuracy of clinical detection (by 279 physician observers) of internuclear ophthalmoparesis (INO) with that of quantitative infrared oculography. For the patients with mild adduction slowing, INO was not identified by 71%. Intermediate dysconjugacy was not detected by 25% of the evaluators. In the most severe cases, INO was not identified by only 6%. Oculographic techniques significantly enhance the precision of INO detection compared to the clinical exam.

Modeling Uhthoff's phenomenon in MS patients with internuclear ophthalmoparesis

Objective: The goal of this investigation was to demonstrate that internuclear ophthalmoparesis (INO) can be utilized to model the effects of body temperature-induced changes on the fidelity of axonal conduction in multiple sclerosis (Uhthoffs phenomenon). Methods: Ocular motor function was measured using infrared oculography at 10-minute intervals in patients with multiple sclerosis (MS) with INO (MS-INO; n = 8), patients with MS without INO (MS-CON; n = 8), and matched healthy controls (CON; n = 8) at normothermic baseline, during whole-body heating (increase in core temperature 0.8°C as measured by an ingestible temperature probe and transabdominal telemetry), and after whole-body cooling. The versional disconjugacy index (velocity-VDI), the ratio of abducting/adducting eye movements for velocity, was calculated to assess changes in interocular disconjugacy. The first pass amplitude (FPA), the position of the adducting eye when the abducting eye achieves a centrifugal fixation target, was also computed. Results: Velocity-VDI and FPA in MS-INO patients was elevated (p < 0.001) following whole body heating with respect to baseline measures, confirming a compromise in axonal electrical impulse transmission properties. Velocity-VDI and FPA in MS-INO patients was then restored to baseline values following whole-body cooling, confirming the reversible and stereotyped nature of this characteristic feature of demyelination. Conclusions: We have developed a neurophysiologic model for objectively understanding temperature-related reversible changes in axonal conduction in multiple sclerosis. Our observations corroborate the hypothesis that changes in core body temperature (heating and cooling) are associated with stereotypic decay and restoration in axonal conduction mechanisms. GLOSSARY: CON = controls; COV = coefficient of variation; FPA = first pass amplitude; INO = internuclear ophthalmoparesis; LED = light emitting diode; MLF = medial longitudinal fasciculus; MS = multiple sclerosis; NC = normal control; VDI = versional disconjugacy index.

Benign paroxysmal positioning vertigo in multiple sclerosis: diagnosis, pathophysiology and therapeutic techniques

Objective: To report on the most common causes of vertigo in patients with multiple sclerosis (MS) and emphasize appro priate diagnostic techniques and treatment interventions. Background: True vertigo is estimated to occur in about 20% of MS patients. Lesions within the vestibular nuclei and in the root entry zone of cranial nerve VIII represent the most common locations where demyelinating activity can provoke vertigo in patients with MS. However, other causes of vertigo should be explored in MS patients in order to avoid unnecessar y treatment with corticosteroids and vestibular suppressants. Recently, we reviewed our four-year experience with new onset vertigo in our university-based MS population and found that benign paroxysmal positioning vertigo (BPPV) to be the most common cause. A ll patients diagnosed with BPPV were treated successfully with particle repositioning maneuvers. The remaining patients were treated with conventional therapies appro priate for the specific diagnosis. Conclusions: Empiric treatments with corticosteroids and/or vestibular suppressants should not be employed until all MS patients undergo a careful bedside examination, which includes diagnostic positional and, if indicated, particle repositioning maneuvers. Here we emphasize the pathophysiology of BPPV and illustrate the proper techniques for the diagnostic and therapeutic maneuvers.

Ocular contrapulsion in multiple sclerosis: clinical features and pathophysiological mechanisms

The objective was to describe in multiple sclerosis, a cerebellar eye movement syndrome that resulted from an acute episode of inflammatory demyelination. Contrapulsion is an ocular motor disturbance characterised by a triad of (1) hypermetric saccadic eye movements in a direction opposite from a precisely localised lesion within a specific white matter pathway, the uncinate fasciculus, at the level of the superior cerebellar peduncle (SCP); (2) hypometric saccades towards the side of the lesion; (3) oblique saccades directed away from the side of the lesion on attempted vertical saccades.  Infrared oculography was used to demonstrate the characteristic features of contrapulsion in two patients with multiple sclerosis.  Brain MRI showed lesions within the region of the uncinate fasciculus and superior cerebellar peduncle in both patients. Eye movement recordings showed saccadic hypermetria away from the side of the lesion and saccadic hypometria towards the side of the lesion. The hypometria decomposed into a series of stepwise movements as the eye approached the target. Oblique saccades directed away from the side of the lesion were seen on attempted vertical saccades. In conclusion, ocular contrapulsion can be seen in patients with multiple sclerosis and results from a lesion in the region of the SCP, involving the uncinate fasciculus.

SHORT-TERM VESTIBULO-OCULAR ADAPTATION : INFLUENCE OF CONTEXT

A number of mechanisms and strategies are used to help an individual compensate for loss of labyrinthine function. One important example is the ability to produce a preplanned motor response that anticipates the motion of the head and so compensates for it. Closely tied to this phenomenon is the gating, in or out, of a learned response on the basis of the context in which it must occur. This issue is particularly relevant to designing programs of physical therapy that optimize performance for natural behavior. Here we discuss a model of short-term vestibulo-ocular adaptation—adjustment of vestibulo-ocular phase (timing)—and how it can be used to study context-dependent vestibulo-ocular learning. We will show how vestibulo-ocular phase can be adjusted by selectively altering the common velocity-to-position ocular motor neural integrator for one type of eye movement (vestibular) and not for another (saccades), or for one type of head movement (sinusoidal) and not for another (step). These results are another example of the remarkable flexibility of the vestibulo-ocular adaptive mechanism and further show that the fundamental process of integration for eye movements can be modified according to the pattern of afferent information. (Otolaryngol Head Neck Surg 1998; 119:60-4.)

Dix–Hallpike maneuver results are not influenced by the time of day of the test

Conclusion The result of the DH maneuver does not appear to be affected by the time of day at which it is performed. Objective To determine whether the time of day at which the Dix–Hallpike (DH) maneuver is performed influences the result. Material and methods This was a retrospective study. We reviewed the records of all electronystagmagrams (n=1220) performed at our facility between January 2001 and January 2003, looking at the results of the DH maneuver, the medical history of the patients and the time of day of the test. The distribution of the time of day at which the DH maneuver was performed and induced a positive response was compared to the distribution of the time of day at which all the tests were performed. Results The distribution of the times during the day when the DH maneuver was positive was not statistically different from that when the DH maneuver was negative.

The Effects of Horizontal Head Position (Yaw axis) and Step Velocity on the Vestibulo-Ocular Reflex

PHILLIP KRAMER,a ELLIOT M. FROHMAN,b DANIELE NUTI, AND DAVID S. ZEEd aThe New Jersey Neuroscience Institute, Edison, New Jersey, USA bDepartment of Neurology and Ophthalmology, University of Texas Southwestern Medical Center, Dallas, Texas, USA cDepartment of Otolaryngology, University of Siena, Siena, Italy dDepartment of Neurology, Neurosciences, Otolaryngology and Ophthalmology, The Johns Hopkins Hospital, Baltimore, Maryland, USA