Cross-sectional evaluation of clinical neuro-ophthalmic abnormalities in an amyotrophic lateral sclerosis population

Moss, Heather E.; McCluskey, Leo; Elman, Lauren; Hoskins, Katelin; Talman, Lauren; Grossman, Murray; Balcer, Laura J.; Galetta, Steven L.; Liu, Grant T.

doi:10.1016/j.jns.2011.10.016

« Previous Next »Journal of the Neurological Sciences
Volume 314, Issue 1 , Pages 97-101, 15 March 2012

Cross-sectional evaluation of clinical neuro-ophthalmic abnormalities in an amyotrophic lateral sclerosis population

Heather E. Moss
- Department of Neurology, University of Pennsylvania, Philadelphia, PA, USA
- Department of Ophthalmology & Visual Sciences, University of Illinois, Chicago, IL, USA
- Department of Ophthalmology, University of Pennsylvania, Philadelphia, PA, USA
- Corresponding author at: UIC Department of Ophthalmology & Visual Sciences, 1855 W. Taylor St, MC 648, Chicago, IL 60612, USA. Tel.: +1 312 996 9120; fax: +1 312 413 7895.
Leo McCluskey
- Department of Neurology, University of Pennsylvania, Philadelphia, PA, USA
Lauren Elman
- Department of Neurology, University of Pennsylvania, Philadelphia, PA, USA
Katelin Hoskins
- Department of Neurology, University of Pennsylvania, Philadelphia, PA, USA
Lauren Talman
- Department of Neurology, University of Pennsylvania, Philadelphia, PA, USA
Murray Grossman
- Department of Neurology, University of Pennsylvania, Philadelphia, PA, USA
Laura J. Balcer
- Department of Neurology, University of Pennsylvania, Philadelphia, PA, USA
- Department of Ophthalmology, University of Pennsylvania, Philadelphia, PA, USA
- Department of Epidemiology, University of Pennsylvania, Philadelphia, PA, USA
Steven L. Galetta
- Department of Neurology, University of Pennsylvania, Philadelphia, PA, USA
- Department of Ophthalmology, University of Pennsylvania, Philadelphia, PA, USA
Grant T. Liu
- Department of Neurology, University of Pennsylvania, Philadelphia, PA, USA
- Department of Ophthalmology, University of Pennsylvania, Philadelphia, PA, USA

Received 3 June 2011; received in revised form 12 September 2011; accepted 12 October 2011. published online 23 December 2011.

Abstract

Objective

Ocular motility abnormalities may be a marker of neuro-degeneration beyond motor neurons in amyotrophic lateral sclerosis (ALS). We formally compared clinical neuro-ophthalmic abnormalities in ALS patients and a control population.

Methods

Patients attending a multidisciplinary ALS clinic (n=63, age 60.8+/−16.4years) and their caregivers serving as controls (n=37, ages 55.0+/−12.7years) participated in this cross-sectional study. Visual acuity was assessed. Video recordings of a standardized ocular motility exam including gaze fixation, voluntary saccades, reflex saccades, smooth pursuit, eyelid opening and Bell's phenomenon were rated by two senior neuro-ophthalmologists who were masked to subject group.

Results

Visual acuity was lower in ALS patients versus control subjects (OR 0.81 (0.71–0.93), p=0.003, logistic regression). Inter- and intra-rater reliability for ocular motility examination ratings were good (Cohen's Kappa>0.6). Findings observed only in ALS subjects included gaze impersistence (14%, p=0.01), moderately or severely restricted voluntary upgaze (13%, p=0.01), and moderate or severe eyelid opening apraxia (27%, p=0.0002). Accounting for age, moderately or severely saccadic horizontal smooth pursuits distinguished ALS from control subjects (OR 3.6 (1.2–10.9), p=0.02, logistic regression).

Conclusions

Clinical findings of decreased visual acuity, gaze impersistence, voluntary upgaze restriction, eyelid opening apraxia, and saccadic horizontal smooth pursuits are more frequent in patients with ALS than in similar-aged controls. These findings are potential clinical markers of neurodegeneration beyond upper and lower motor neuron disease in ALS. Further study is warranted regarding their application to disease categorization and outcomes assessment.

Keywords: Prevalence study, Eye movements, Vision, Amyotrophic lateral sclerosis