An eye movement disorder in amyotrophic lateral sclerosis

Abstract

Defective pursuit eye movements were recorded by electrooculography (EOG) in 11 of 18 patients (61%) with amyotrophic lateral sclerosis. Pursuit defects consisted of a breakdown of smooth tracking into saccadic motions that were grossly in excess (frequencies and amplitudes) of saccadic interruptions of pursuit in normal subjects. In nine patients, defective pursuits cogwheeling) were obvious by visual inspection as well as by EOG; in two, this abnormality was seen only by EOG. In eight patients, the pursuit defect was the only abnormality of oculomotor function; in three, there were also saccadic defects (optokinetic nystagmus or conjugate gaze) discerned by EOG. Autopsy revealed neuronal degeneration in substantia nigra and demyelination in integral capsule in one patient with the pursuit defect but not in another patient without the defect. The pursuit defect may be a sign of extrapyramidal or supratentorial pyramidal involvement in ALS.

Eye Movements in Amyotrophic Lateral Sclerosis

“…These findings are similar to those reported by Jacobs et al 6 Our methods differed from theirs in that our smooth pursuit targets were projected moving targets, whereas Jacobs et al relied primarily on closely spaced light bulbs flashed in a continual sequence for targets. These could con- ceivably stimulate saccades if the spacing between bulbs was large enough.…”

Ocular fixation instabilities in motor neurone disease

“…With the lack of quantifiable objective measures of disease in MND, characteristic eye movement abnormalities could be of great use both diagnostically and in research. Although eye movements are classically spared in MND some patients have been reported with a range of ocular motor disorders including nystagmus, saccadic hypometria [28], slowed saccades [8,33], increased saccadic latencies [25], decreased smooth pursuit gain [3,23,25, 32] and saccadic interruptions of smooth pursuit [20] although these studies were small and often predated the El Escorial criteria. The most comprehensive study to date [37], however, found increased antisaccadic error rates and latencies with relative preservation of reflexive saccades suggesting frontal lobe dysfunction.…”

An auditory oddball (P300) spelling system for brain‐computer interfaces

“…Although sparing of neurodegeneration of nerves, which subserve eye movement, has been reported (Hayashi & Kato, 1989;Whitehouse, Wamsley, Zarbin, Price, & Kuhar, 1985), there are now numerous reports about impaired eye movement and slowing of saccades in ALS (Averbuch-Heller, Helmchen, Horn, Leigh, & Buttner-Ennerver, 1998;Jacobs, Bozian, Heffner, & Barron, 1981;Leveille, Kiernan, Goodwin, & Antel, 1982;Ohki et al, 1994;Palmowski et al, 1995a;Palmowski et al, 1995b;Szmidt-Salkowska & Rowinska-Marcinska, 2005). Although not commonly seen in neurological practice (because patients usually die before entering the complete locked-in state), eye muscles may become totally paralyzed in ALS rendering the patients completely locked in (complete locked-in state 5 CLIS) (Cohen & Caroscio, 1983;Harvey, Torack, & Rosenbaum, 1979;Kushner et al, 1984;Palmowski et al, 1995a); our own experience confirms these reports (Hill et al, 2006;Hinterberger, Birbaumer, & Flor, 2005;.…”