Faculty Bibliography

BACKGROUND: Sports-related concussion has received increasing awareness due to short- and long-term neurologic sequelae seen among athletes. The King-Devick (K-D) test captures impairment of eye movements and other correlates of suboptimal brain function. We investigated the K-D test as a screening for concussion when administered by layperson sports parents in a cohort of amateur boxers. METHODS: The K-D test was administered pre-fight and post-fight by laypersons masked to the head trauma status of each athlete. Matches were watched over by a ringside physician and boxing trainer. Athletes with suspected head trauma received testing with the Military Acute Concussion Evaluation (MACE) by the ringside physician to determine concussion status. Athletes sustaining concussion were compared to the athletes screened using the K-D test. RESULTS: Post-fight K-D scores were lower (better) than the best baseline score (41 vs. 39.3 s, P=0.34, Wilcoxon signed-rank test), in the absence of concussion. One boxer sustained a concussion as determined by the ringside physician. This boxer was accurately identified by the layperson K-D testers due to a worsening in K-D test compared to baseline (3.2 seconds) and an increased number of errors. High levels of test-retest reliability were observed (intraclass correlation coefficient 0.90 [95% CI 0.84-0.97]). Additionally, 6 boxers who participated in multiple bouts showed no worsening of their K-D times further supporting that scores are not affected by the fatigue associated with sparring. CONCLUSION: The K-D test is a rapid sideline screening tool for concussion that can be effectively administered by non-medically trained laypersons.

BACKGROUND: The King-Devick (KD) test measures the speed of rapid number naming, and is postulated to require fast eye movements, attention, language, and possibly other aspects of cognitive functions. While used in multiple sports concussion studies, it has not been applied to the field of movement disorders. METHODS: Forty-five Parkinson's disease (PD), 23 essential tremor (ET), and 65 control subjects were studied. Subjects performed two trials of reading out loud single-digit numbers separated by varying spacing on three test cards that were of different formats. The sum time of the faster trial was designated the KD score and compared across the three groups. RESULTS: PD patients had higher (worse) KD scores, with longer reading times compared to ET and control subjects (66 s vs. 49 s vs. 52 s, p < 0.001, adjusting for age and gender). No significant difference was found between ET and control (Delta = -3 s, 95% CI: -10 to 4). CONCLUSIONS: This is the first study of the King-Devick Test in Parkinson's disease. PD patients were found to have a slower rapid number naming speed compared to controls. This test may be a simple and rapid bedside tool for quantifying correlates of visual and cognitive function in Parkinson's disease.

BACKGROUND: The decision between conservative management and invasive treatment of juxtaorbital intracranial vascular anomalies can be challenging. Whereas arteriovenous malformations (AVMs) can lead to vision loss and are potentially life threatening if they rupture, invasive endovascular and surgical procedures also carry risks. CASE DESCRIPTIONS: Two patients developed blinding orbital congestion soon after successful treatment of cerebral vascular anomalies. The first patient, a 36 year-old man, underwent partial embolization followed by resection of a congenital frontal lobe AVM. The second patient, a 62 year-old woman, underwent embolization of a peri-orbital/skull base dural arteriovenous malformation. Following intervention, both patients developed unilateral vision loss proptosis, chemosis, complete ophthalmoplegia, and elevated intraocular pressure. The first patient suffered from acute orbital compartment syndrome in the absence of any acute localized hemorrhage or thrombosis. The second patient experienced refractory acute glaucoma from orbital congestion, secondary hyphema and angle closure due to superior ophthalmic vein and cavernous sinus thromboses. CONCULSIONS: These cases highlight the potential orbital congestion to worsen acutely after invasive treatment of juxtaorbital cerebral vascular anomalies, and suggest diverse mechanisms of resultant visual and orbital compromise. The first case represents the first report of orbital compartment syndrome following resection of a congenital AVM.

OBJECTIVE:A double-blind, randomized, controlled study was undertaken to determine whether combined use of interferon β-1a (IFN) 30 μg intramuscularly weekly and glatiramer acetate (GA) 20 mg daily is more efficacious than either agent alone in relapsing-remitting multiple sclerosis. METHODS:A total of 1,008 participants were randomized and followed until the last participant enrolled completed 3 years. The primary endpoint was reduction in annualized relapse rate utilizing a strict definition of relapse. Secondary outcomes included time to confirmed disability, Multiple Sclerosis Functional Composite (MSFC) score, and magnetic resonance imaging (MRI) metrics. RESULTS:Combination IFN+GA was not superior to the better of the single agents (GA) in risk of relapse. Both the combination therapy and GA were significantly better than IFN in reducing the risk of relapse. The combination was not better than either agent alone in lessening confirmed Expanded Disability Status Scale progression or change in MSFC over 36 months. The combination was superior to either agent alone in reducing new lesion activity and accumulation of total lesion volumes. In a post hoc analysis, combination therapy resulted in a higher proportion of participants attaining disease activity-free status (DAFS) compared to either single arm, driven by the MRI results. INTERPRETATION/CONCLUSIONS:Combining the 2 most commonly prescribed therapies for multiple sclerosis did not produce a significant clinical benefit over 3 years. An effect was seen on some MRI metrics. In a test of comparative efficacy, GA was superior to IFN in reducing the risk of exacerbation. The extension phase for CombiRx will address whether the observed differences in MRI and DAFS findings predict later clinical differences.

Purpose: The King-Devick (K-D) test, a rapid number naming test, captures impaired eye movements and saccades, findings correlated with suboptimal brain function. This test has been used to screen athletes for concussions, with worse time scores in athletes following concussion, consistent with widely distributed visual pathways. We examined the K-D test as a measure of vision and eye movements in multiple sclerosis (MS) and determined the relation of K-D scores to visual function, vision-specific quality of life (QoL), work disability, and history of optic neuritis (ON).
Method(s): Patients with MS and disease-free controls completed the test at a single visit. Scores represent time needed to read single-digit numbers on 3 test cards. Patients had testing of monocular and binocular low-contrast acuity (2.5%, 1.25%), high-contrast acuity (VA), spectral-domain OCT, NEI-VFQ-25, 10-Item Neuro-Ophthalmic Supplement, and MS Functional Composite.
Result(s): In the MS cohort (n=81), K-D scores were worse compared to controls (54.7+/-15.7 vs. 41.2+/-7.2 seconds, p=0.003, least squares means, adjusting for age). Higher scores in MS were associated with worse scores for vision-specific QoL (p<0.001 for NEI-VFQ-25, p<0.001 for 10-Item Supplement), binocular low-contrast acuity at 2.5%, 1.25% (p<0.001), binocular VA (p=0.003), timed 25-foot walk (p<0.001), 9-hole peg test (p=0.001), and 3- second PASAT3 (p=0.03, linear regression). Patients with history of acute ON (p=0.003) or binocular low-contrast acuities below the control group average (p=0.009 for 2.5%, logistic regression) had worse K-D scores. Correspondingly, monocular vision (p=0.001-0.009) and RNFL thickness (p=0.001) were reduced in eyes of patients with worse K-D (adjusting for age and within-patient, inter-eye correlations). Disabled patients (receiving disability pension) did worse on the K-D test compared to those working full-time, accounting for age (p<0.001).
Conclusion(s): The K-D test captures visual dysfunction, vision-specific quality of life and neurologic impairment in MS. Scores reffect work disability as well as structural changes as measured by OCT. History of ON and abnormal binocular acuities were associated with worse scores, suggesting that the K-D captures both a0erent and e0erent components of vision. The K-D test should be considered for future MS trials as a rapid global visual performance measure

Studies suggest that a lack of standardized knowledge may lead to underreporting and undertreatment of sports-related concussion. However, there has been little work done to establish how this knowledge may affect athletes' behaviors toward reporting their concussions and removing themselves from play. We conducted an anonymous online survey to assess athletes' knowledge of signs and symptoms of concussion, and also sought to estimate the potential frequency of underreporting in a collegiate athlete cohort. Among 262 athletes who responded to the survey, 43% of those with a history of concussion reported that they had knowingly hidden symptoms of a concussion to stay in a game, and 22% of athletes overall indicated that they would be unlikely or very unlikely to report concussion symptoms to a coach or athletic trainer in the future. These data suggest that there may be a substantial degree of underreporting of concussion among collegiate athletes, despite most acknowledging that they have been formally educated about the risks of concussion.

The diagnosis of pseudotumor cerebri, or idiopathic intracranial hypertension (IIH), is most confidently established in the typical patient with evidence of papilledema, imaging that does not suggest a structural lesion, and a CSF examination that shows both normal composition and elevated intracranial pressure (ICP). Prompted by an increasing number of reports over the past decade, Friedman et al.1 propose a revised set of diagnostic criteria for IIH, taking into account the most recent observations from neuroimaging studies. Although the patient with IIH is often a young woman who is above ideal body weight or obese, it is well-recognized that the disorder may also occur in obese men and in children, who are less likely to be obese than their adult counterparts. Several advances in the field prompted the expert authors to provide new guidance. First, a large study of children has redefined normal CSF opening pressure for children.2 In the obese or sedated child, an opening pressure of 280 mm H2O has been suggested as the requirement to claim confidently that the ICP is increased. Otherwise, the diagnostic criteria for children and adults continue to rely on a CSF lumbar opening pressure of 250 mm H2O or greater.

OBJECTIVE: To employ a novel stimulation paradigm in order to elicit multifocal electroretinography (mfERG)-induced optic nerve head component (ONHC) responses, believed to be contingent upon the transformation in electrical transmission properties of retinal ganglion cell axons from membrane to saltatory conduction mechanisms, as they traverse the lamina cribrosa and obtain oligodendrocyte myelin. We further sought to characterize abnormalities in ONHC responses in eyes from patients with multiple sclerosis (MS). METHODS: In 10 normal subjects and 7 patients with MS (including eyes with and without a history of acute optic neuritis), we utilized a novel mfERG stimulation paradigm that included interleaved global flashes in order to elicit the ONHC responses from 103 retinal patches of pattern-reversal stimulation. RESULTS: The number of abnormal or absent ONHC responses was significantly increased in MS patient eyes compared to normal subject eyes (p < 0.001, by general estimating equation modeling, and accounting for age and within-subject, intereye correlations). CONCLUSION: Studying the relationship between ONHC abnormalities and alterations in validated structural and functional measures of the visual system may facilitate the ability to dissect and characterize the pathobiological mechanisms that contribute to tissue damage in MS, and may have utility to detect and monitor neuroprotective or restorative effects of novel therapies.