Differential changes in the spinal segmental locomotor output in Hereditary Spastic Paraplegia

Publication date: March 2018
Source:Clinical Neurophysiology, Volume 129, Issue 3
Author(s): G. Martino, Y. Ivanenko, M. Serrao, A. Ranavolo, F. Draicchio, M. Rinaldi, C. Casali, F. Lacquaniti
ObjectiveA comprehensive treatment of Hereditary Spastic Paraplegia (HSP) should consider the specific pathophysiological changes in the spinal cord. Here we reported a detailed characterization of the spinal motoneuronal output in HSP during locomotion.MethodsWe recorded kinematics and electromyographic (EMG) activity of 12 leg muscles in 29 patients with pure forms of HSP and compared them with 30 controls while walking at matched speeds. We assessed the spinal locomotor output by evaluating EMG patterns and by mapping them onto the rostrocaudal location of the spinal motoneuron pools.ResultsThe activity profiles of muscles innervated from the sacral segments were significantly wider in patients. Similarly, spinal maps revealed a tendency for spreading the main loci of activation, involving initially the sacral segments and, at more severe stages, the lumbar segments.ConclusionsThe degeneration of the corticospinal tract in HSP is associated with a widening of spinal locomotor output spreading from caudal to rostral segments.SignificanceThe findings highlight pathophysiologically relevant differential changes in the spinal locomotor output in HSP related to the specific innervation of muscles in the spinal cord, and might be helpful for developing future therapeutic strategies and identifying physiological markers of the disease.



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