Motor neuron disease with slow eye movements and vertical gaze palsy

Abstract

Two middle‐aged patients with motor neuron disease showed common eye movement disorders and intellectual impairment in the later stage of the illness. Eye movement disorders were characterized by slow saccades and vertical glaze palsy, which seemed to be supranuclear ophthalmoplegia. Neuropathological examination of one patient revealed degeneration of the substantia nigra without pathological changes in the ocular motor nuclei, in addition to findings compatible with motor neuron disease. These cases appear to raise a possibility of a distinct group of multiple system degenerations.

Slow saccades in bulbar-onset motor neurone disease

“…Lesions in these areas are known to cause slow saccades [22]. These findings might also help to explain case reports in the literature that describe slow saccades and supranuclear gaze palsies in patients with MND [6,21,35,47].…”

“…Many of these studies, however, were very small, lacked controls and pre-dated the El Escorial criteria (EEC) [8]. Several case reports, however, have described slow saccades and supranuclear gaze palsies in patients with MND often associated with cognitive impairment, advanced or bulbar-onset disease [6,21,35,47]. Some of these had supportive neuropathological evidence of MND and one case report was able to show clinico-pathological correlation for slow vertical saccades by the finding of gliosis in the riMLF (rostral interstitial medial longitudinal fasciculus) which houses the burst cell neurons for vertical saccades [6].…”

“…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].…”

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.…”

Introductory Chapter: Introduction to Update in Amyotrophic Lateral Sclerosis and Review of this Condition in Sportsmen

“…In our series of patients, complaints about visual disturbances, ophthalmoplegia, auditory disorders, vestibular dysfunction, olfactory problems, sensory loss or disorder of the autonomic nervous system related to ALS were not identified, although in late stages of the disease patients may develop a supranuclear gaze palsy or oculomotor palsy [7,8], as anecdotic cases. However, cognitive impairment can be seen in nearly a third of the patients with ALS in a pattern consistent with frontotemporal lobar dementia as has been proved [9].…”