Brain-Computer Interface Implant for Severe Communication Disability

Who may be able to join

• Age between 22-75 years.
• Clinical diagnosis of chronic Locked-In Syndrome (LIS) certified by an independent board-certified neurologist, defined here as impairments of voluntary motor control that are widespread and severe enough to prevent independent initiation and maintenance of communication, spoken or otherwise, without the assistance of a caregiver. Diagnosis of LIS requires exclusion of cognitive impairments as a cause for impaired communication (see Exclusion Criteria). For inclusion in this study, participants may have one of the following clinical variants of LIS:
• "Classic" Locked-In Syndrome, with the following signs documented on neurological examination i. Quadriplegia ii. Bulbar palsy iii. Anarthria iv. Partial or complete preservation of vertical eye movements and upper eyelid movements, or b. "Incomplete" Locked-In Syndrome, with the aforementioned signs of "Classic" LIS, documented on neurological examination, except that voluntary movements other than upper eyelid and vertical eye movement may be preserved, albeit still severe enough to prevent independent initiation and maintenance of communication, without the assistance of a caregiver. Preserved voluntary movements may include: i. Horizontal eye movements ii. Facial movements iii. Movements of the extremities not sufficient to reliably operate a joystick or mouse
• Clinical diagnosis of, and confirmatory/exclusionary testing consistent with, one of the following irreversible or progressive neurological disorders as the cause for chronic Locked-In Syndrome, as defined above:
• Permanent lesion of corticospinal and corticobulbar tracts in the ventral pons (basis pontis) due to ischemic stroke, hemorrhage, or trauma, based on the following: i. Documented history of acute ischemic or hemorrhagic brainstem stroke or traumatic brainstem injury at onset of LIS ii. Ancillary testing including the following:
• 1. Brain MRI confirming a chronic lesion of the ventral pons, with no change on serial images at least 1 month apart, consistent with findings on neurological examination, involving at least 50% of the cross-sectional area on both sides of the basis pontis on at least one axial section 2. Absence of lesions on brain MRI that could contribute to communication impairment, including lesions of Broca's or Wernicke's areas, or sensorimotor cortex 3. EEG demonstrating posterior basic rhythm reactive to eye opening and closing iii. No clinical evidence of improving communication ability for at least 1 year prior to recruitment, confirming chronic LIS b. Diagnosis of Amyotrophic Lateral Sclerosis (ALS), certified by an independent board-certified neurologist with expertise in neuromuscular disorders, satisfying the following El Escorial World Federation of Neurology diagnostic criteria for probable, laboratory supported, or definite ALS: i. Signs of lower motor neuron (LMN) degeneration by neurological examination (weakness, atrophy, and fasciculations), electrophysiological (electromyographic findings of denervation), or neuropathological determination (muscle biopsy) in bulbar and at least two of three spinal regions (cervical, thoracic, lumbosacral), or in all three spinal regions ii. Signs of upper motor neuron (UMN) degeneration by clinical examination (spasticity, hyperreflexia, pseudobulbar affect, pathological reflexes) in bulbar and at least two of three spinal regions (cervical, thoracic, lumbosacral), or in all three spinal regions iii. Progressive spread of signs within a region or to other regions, together with the absence of electrophysiological or neuroimaging evidence of other disease processes that might explain clinical and electrophysiological signs of LMN and/or UMN degeneration (see below) iv. Confirmatory testing including: