Ocular Abnormalities in Amyotrophic Lateral Sclerosis

Slow saccades in bulbar-onset motor neurone disease

“…Shaunak et al had found reflexive saccades to be normal, however, earlier reports in the literature had found slowed saccades [23,34]. No study to date has looked specifically at saccades in bulbar-onset patients.…”

“…The decline in velocity gain and the proportion of time spent in smooth pursuit with increasing speeds occurred at a faster rate in MND patients compared to controls. Shaunak et al [42] in 1995 found smooth pursuit to be normal while four earlier studies [23,25,29,34] smooth pursuit gain was found to be reduced. In another study abnormal smooth pursuit was observed due to the breakdown of the smooth tracking by saccadic interruptions or intrusions [19].…”

“…Numerous abnormalities, however, have been reported in the literature including slow saccades [6,17,23,34,35], saccadic interruption of smooth pursuit [23,36], gaze palsies [36,41], horizontal gaze nystagmus [41], difficulties in maintaining fixation [37] and reduced smooth pursuit gain [2,17,23,34]. Many of these studies, however, were very small, lacked controls and pre-dated the El Escorial criteria (EEC) [8].…”

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;.…”

Motor neuron disease presenting with slow saccades and dementia

“…4 Classically, ocular motility is spared in ALS; however, ocular motility abnormalities have been reported. [5][6][7][8][9][10][11][12][13][14][15] Our patient presented with both dementia and abnormal ocular motility.…”