Patterns of neural activity and clinical outcomes in a juvenile Huntington’s disease patient undergoing STN DBS
Kaymak A, Vissani M, Lenge M, Melani F, Fino E, Cappelletto P
Deep Brain Stimulation · 2023 · doi:10.1016/j.jdbs.2023.03.001
Huntington's disease (HD) is a hereditary neurodegenerative disease leading to cognitive and motor impairment. HD depends on basal ganglia dysfunctions, but the role of subthalamic nucleus (STN) neurons is not completely known. Drug-resistant motor symptoms of HD can be alleviated by neuromodulation of the basal ganglia through Deep Brain Stimulation (DBS) of STN. DBS target selection is supported by intra-operative microelectrode recordings (MER). MER have been previously used to characterize neural dynamics of STN in several movement disorders and can provide information on firing patterns underlying HD. We analyzed MER data acquired during bilateral DBS of STN in a juvenile HD female patient with hypokinetic motor symptoms (generalized dystonia, stiffness, and severe gait impairment). Firing patterns of STN in HD were characterized by isolating single neuron activities (n=23) and measuring their regularity, bursting, and oscillatory behavior. Multi-unit activity recordings spectrum was used to estimate the presence of network oscillations, characterizing the subthalamic firing signatures of this rare juvenile Huntington's disease case and relating them to the patient's clinical picture.