Abstract Details

Title Oculomotor Dysfunction in Motor Neuron Disease

Topic Neuromuscular and Clinical Neurophysiology (EMG)

Presentation(s) P4 - Poster Session 4 (5:30 PM-6:30 PM)

Poster/Presentation
Number 12-006

Objective To characterize oculomotor dysfunction (OD) in motor neuron diseases (MND).

Background

Eye movements were traditionally thought to be spared in MND. However, recent literature suggests that some patients exhibit OD, presumably related to involvement of frontal eye fields or pontine pathways. We hypothesize that patients with advanced amyotrophic lateral sclerosis (ALS), progressive bulbar palsy (PBP), or primary lateral sclerosis (PLS) will demonstrate more OD compared to patients with progressive muscular atrophy (PMA). Characterization of OD may aid in the diagnosis of MND.

Design/Methods This is a prospective observational study of patients with a diagnosis of MND. Patients were examined for clinical OD. Four patients opted for additional quantitative video oculography. The relationship between OD and clinical characteristics was analyzed using Pearson correlation.

Results Of 23 patients, 13 patients had probable or definite ALS by El Escorial criteria, 6 had PMA, 3 PBP, and 1 PLS. 12 patients (52.2%) exhibited OD; most frequent in PLS (100%), then PBP (66.7%), PMA (50%), and ALS (46.2%). The most common OD finding was square wave jerks (83.3%), followed by hypometric saccades (33.3%), saccadic pursuit (25%), nystagmus (16.7%) and ocular dysmetria (16.7%). Presence of OD was negatively correlated with FVC (r=-0.44, p=0.036), and ALSFRS-R (r=-0.43, p=0.041) which is especially evident in the ALS subgroup (r=-0.64, p=0.020), but not with disease duration, MRC sum score, CNS-LS, or NIF. On quantitative video oculography, one ALS patient demonstrated low peak velocity on central-to-lateral gaze saccades of amplitude >20 degrees, indicating abnormality of the abducens motor neurons. Another ALS patient had absent large saccades and OKN to the right and up. Two patients (ALS and PBP) had slowed large vertical saccades. One PMA patient had normal result.

Conclusions

OD may be associated with more advanced MND and upper motor neuron involvement. Recruitment is ongoing.

Authors/Disclosures
Christine Lu, MD PRESENTER	No disclosure on file
Clover Youn, MD (Raymond G. Murphy VA Medical Center)	Dr. Youn has nothing to disclose.
Sergei Yakushin	No disclosure on file
Jonathan Cauchi, MD (University of New Mexico Hospital)	Dr. Cauchi has nothing to disclose.
Daniel J. MacGowan, MD, FAAN (Mount Sinai Department of Neurology)	No disclosure on file
Stephen N. Scelsa, MD (Mount Sinai BETH ISRAEL, Mount Sinai Downtown)	No disclosure on file

��ɫ��

��ɫ��