1538 - Ophthalmologic Considerations in Incomplete Locked-In Syndrome

Background:

Incomplete LIS, characterized by quadriplegia and inability to speak while retaining consciousness and cognitive function, can affect voluntary eye movements crucial for communication. Ophthalmologic complications of LIS such as gaze palsy, exposure keratitis, and nystagmus can significantly impact quality of life and rehabilitation.

Patient Summary:

A 64-year-old woman with no significant medical history presented to an Inpatient Rehabilitation Facility (IRF) following an acute pontine stroke and intraventricular hemorrhage. Post-stroke complications included respiratory failure, dysphagia, and vision impairments like disconjugate gaze, horizontal gaze palsy, and vertical nystagmus. Vision therapy and neuro-optometry evaluations recommended fixation and tracking exercises. Ophthalmology evaluated the patient and found a complete right gaze palsy, nystagmus, lagophthalmos due to cranial nerve 7 palsy, and exposure keratopathy, and recommended aggressive lubrication and a moisture chamber for exposure keratitis, with consideration for pharmacologic management of the opsoclonus.

Discussions: Pharmacologic management of opsoclonus-myoclonus syndrome is limited. Baclofen has been shown to reduce involuntary eye movements by acting on GABA receptors in the central nervous system, which may help in stabilizing vision.  A small study demonstrated improvements in opsoclonus symptoms in the setting of LIS with the use of gabapentin, which was well-tolerated within the study group, and suggested that gabapentin may regulate saccadic circuits. These improvements translated to improved voluntary eye control which allowed for improved eye gaze communication.

Conclusions:

For patients with incomplete LIS, early management of vision/ophthalmic complications is recommended. Early vision therapy and referrals to neuro-optometry or ophthalmology are crucial for optimizing rehabilitation outcomes.