Preserved sensory-motor function despite large-scale morphological alterations in a series of patients with holocord syringomyelia

Abstract

Although the central nervous system has a limited capacity for regeneration after acute brain and spinal cord injuries, it can reveal extensive morphological changes. Occasionally, the formation of an extensive syrinx in the spinal cord can be observed that causes no or only limited signs of functional impairment. This condition creates a unique opportunity to evaluate the mismatch between substantial morphological changes and functional outcomes. We identified seven patients with holocord syringomyelia affecting the cervical cord following chronic traumatic thoracic/lumbar spinal cord injury (19-34 years after injury) or holocord syringomyelia of non-traumatic origin, and anatomical syrinx dimensions (length, cross-sectional area) were determined using sagittal and axial magnetic resonance imaging scans. Motor- and sensory-pathway integrity were evaluated using electrophysiological assessments (i.e., motor, dermatomal sensory, and dermatomal contact-heat [dCHEP] evoked potentials, as well as nerve conduction studies). These were specifically compared to clinical measures of upper-limb strength and grasping performance, including three-dimensional motion analysis. Despite extensive anatomical changes of the cervical cord (on average 26% reduction of residual spinal cord area and intrusion of almost the entire cervical spinal cord), a clinically relevant impairment of upper-limb motor function was absent while only subtle sensory deficits could be detected. dCHEPs revealed the highest sensitivity by disclosing impairments of spinothalamic pathways. Comparable to that of the brain, extensive anatomical changes of the spinal cord can occur with only subtle functional impairment. The time scale of slowly-emerging morphological alterations is essential to permit an enormous capacity for plasticity of the spinal cord.