Nystagmus in motor neuron disease: Clinicopathological study of two cases

Abstract

Two patients with amyotrophic lateral sclerosis proved postmortem had nystagmus in addition to typical clinical signs of motor neuron disease. The first patient had gaze-evoked rotatory nystagmus that was followed by horizontal nystagmus in the primary position with supranuclear paresis of horizontal gaze and upgaze. The second patient had rotatory nystagmus that was evoked by lateral gaze, with normal range of eye movements. Nystagmus is so rare in motor neuron disease that these observations may imply another disease, but postmortem examination did not provide any other explanation. These two cases add to the increasing evidence that motor neuron disease comprises a heterogeneous group of disorders.

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

“…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;. For such patients, the visually based P300-BCI would no longer provide a reliable communication channel.…”

Eye movements in amyotrophic lateral sclerosis and its mimics: a review with illustrative cases

“…In this case, nystagmus can result from an impaired ability to correct for retinal image drift or disruption of the visual inputs that allow eye movement calibration according to visual demands. Nystagmus is rarely reported in ALS although there are two reported cases of ALS, confirmed post mortem, with nystagmus 30. One of these had gaze evoked nystagmus followed later by supranuclear palsy of horizontal gaze and upgaze, while the other had gaze evoked nystagmus with a normal range of eye movements.…”

Motor neuron disease presenting with slow saccades and dementia

“…6 In 1984, Kushner et al noted gaze-evoked rotary and horizontal nystagmus, limited upgaze, absent convergence and limited abduction in one patient and nystagmus in the primary position in another patient with ALS. 5 An autopsy on the rst patient showed central chromatolysis in the substantia nigra, and reticular formation of the pons and midbrain, with rare involvement of the oculomotor nuclei. The authors noted these pathological changes did not explain the nystagmus and ophthalmoparesis.…”

“…[5][6][7][8][9][10][11][12][13][14][15] Studies have failed to reach a consensus regarding the neuroanatomic structures involved in ALS and the type of saccadic dysfunction present. 6,7,9,11,[13][14][15] In 1979, Harvey, Torack and Rosenbaum reported a patient with ALS and ocular motor dysfunction.…”

“…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.…”