Elizabeth M. Runge, MD, PhD
Resident Physician
University of Pittsburgh Medical Center
Pittsburgh, Pennsylvania, United States
Kevin Franzese, DO
Assistant Professor of Physical Medicine & Rehabilitation
University of Pittsburgh Medical Center
Pittsburgh, Pennsylvania, United States
A 56-year-old female with known hemorrhagic pineal mass was admitted from an outside hospital with recurrent pineal hemorrhage with intraventricular extension and obstructive hydrocephalus. Ophthalmologic evaluation was notable for Parinaud syndrome with superimposed right oculomotor palsy. She was initially awake and alert, but following craniotomy for tumor resection was reported to become profoundly encephalopathic and non-interactive with persistent eye closure. Physical Medicine and Rehabilitation (PM&R) Brain Injury Medicine (BIM) service was consulted for neurostimulation for presumed impairment of consciousness. PM&R examination was notable for brisk command following in all extremities but with inability to open the eyes or follow any bulbar motor commands. Trismus and persistent tonic activation of the orbicularis oculi were noted. Within one hour of oral baclofen trial, the patient temporarily regained the ability to volitionally open her eyes and mouth. Participation with therapies greatly improved, and the patient was eventually discharged to an inpatient rehabilitation facility.
Discussions:
Bulbar dysfunction can represent a milder form of cognitive-motor dissociation, and patients with this condition are at risk of misdiagnosis with a disorder of consciousness due to an inability to speak or engage socially with the examiner. Potential explanations for this patient’s deficits include bulbar hypertonia/spasticity due to tumor involvement of descending corticobulbar tracts, orofacial apraxia, and/or neurobehavioral involvement. Alternative proposed mechanisms for baclofen’s efficacy in this patient beyond its classical spasticity-relieving properties are also discussed, including its effects on cortical excitability and dopamine transmission.
Conclusions:
Early involvement of a PM&R BIM consult service can improve identification of patients with bulbar dysfunction who have been misdiagnosed as having impaired consciousness, leading to improved access to neuromodulatory and rehabilitative treatments.
