One in a Million: A Case Report of Stiff Person Syndrome

Stiff person syndrome (SPS) is a rare autoimmune disease caused by lack of inhibition to excitatory neurotransmitters in the central nervous system (CNS) leading to inappropriate motor unit firing. The pathophysiology is incompletely understood; however, high titers of antiglutamic acid decarboxylase antibody (anti-GAD Ab) are strongly associated with this disease. We present a 50-year-old woman with a history of ongoing gait and balance issues for 5 years with multiple negative workups. She recently had an acute exacerbation which left her bedbound, unable to move her legs or turn from side to side. After a negative workup at an outside hospital, the patient was discharged to a subacute rehabilitation facility. She then presented to our institution due to worsening of her condition and was ultimately diagnosed with SPS which was successfully treated. We review the case presentation and treatment options in the context of a severe disabling disease presentation.

Firing rate trajectories of human motor units during activity-dependent muscle potentiation

During activity-dependent potentiation (ADP) motor unit firing rates (MUFRs) are lower, however, the mechanism for this response is not known. During increasing torque isometric contractions at low contraction intensities, MUFR trajectories initially accelerate and saturate demonstrating a non-linear response due to the activation of persistent inward currents (PICs) at the motoneuron. The purpose was to assess whether PICs are a factor in the reduction of MUFRs during ADP. To assess this, MUFR trajectories were fit with competing functions of linear regression and a rising exponential (i.e., acceleration and saturation). Using fine-wire electrodes, discrete MU potential trains were recorded in the tibialis anterior during slowly increasing dorsiflexion contractions to 10% of maximal voluntary contraction following both voluntary (post-activation potentiation; PAP) and evoked (post-tetanic potentiation; PTP) contractions. In 8 participants, 25 MUs were recorded across both ADP conditions and compared to the control with no ADP effect. During PAP and PTP, the average MUFRs were 16.4% and 9.2% lower (both P≤ 0.001), respectively. More MUFR trajectories were better fit to the rising exponential during control (16/25) compared to PAP (4/25, P<0.001) and PTP (8/25, P=0.03). The MU samples that had a rising exponential MUFR trajectory during PAP and PTP displayed an ~11% lower initial acceleration compared to control (P<0.05). Thus, synaptic amplification and MUFR saturation due to PIC properties are attenuated during ADP regardless of the type of conditioning contraction. This response may contribute to lower MUFRs and likely occurred because synaptic input is reduced when contractile function is enhanced.

Sensing and decoding the neural drive to paralyzed muscles during attempted movements of a person with tetraplegia using a sleeve array

Motor neurons convey information about motor intent that can be extracted and interpreted to control assistive devices. However, most methods for measuring the firing activity of single neurons rely on implanted microelectrodes. Although intracortical brain-computer interfaces (BCIs) have been shown to be safe and effective, the requirement for surgery poses a barrier to widespread use that can be mitigated by instead using noninvasive interfaces. The objective of this study was to evaluate the feasibility of deriving motor control signals from a wearable sensor that can detect residual motor unit activity in paralyzed muscles after chronic cervical spinal cord injury (SCI). Despite generating no observable hand movement, volitional recruitment of motor units below the level of injury was observed across attempted movements of individual fingers and overt wrist and elbow movements. Subgroups of motor units were coactive during flexion or extension phases of the task. Single digit movement intentions were classified offline from the EMG power (RMS) or motor unit firing rates with median classification accuracies >75% in both cases. Simulated online control of a virtual hand was performed with a binary classifier to test feasibility of real-time extraction and decoding of motor units. The online decomposition algorithm extracted motor units in 1.2 ms, and the firing rates predicted the correct digit motion 88 ± 24% of the time. This study provides the first demonstration of a wearable interface for recording and decoding firing rates of motor units below the level of injury in a person with motor complete SCI.

Motor unit firing patterns on increasing force during force and position tasks

Different neurophysiological strategies are used to perform angle adjustments during motor tasks such as car driving and force-control tasks using a fixed-rigid pedal. However, the difference in motor unit behavior in response to an increasing exerted force between tasks is unknown. This study aimed to investigate the difference in motor unit responsiveness on increasing force between force and position tasks. Twelve healthy participants performed ramp and hold contractions during ankle plantarflexion at 20 and 30% of the maximal voluntary contraction using a rigid pedal (force task) and a free pedal with an inertial load (position task). High-density surface electromyograms were recorded of the medial gastrocnemius muscle and decomposed into individual motor unit firing patterns. Ninety and 109 motor units could be tracked between different target torques in each task. The mean firing rate increased and firing rate variability decreased on 10% maximal voluntary contraction force gain during both force and position tasks. There were no significant differences in these responses between the two tasks. Our results suggest that the motor unit firing rate is similarly regulated between force and position tasks in the medial gastrocnemius muscle with an increase in the exerted force.

Anconeus motor unit firing rates during isometric and muscle-shortening contractions comparing young and very old adults

Changes of neural drive to the muscle with adult aging, measured as motor unit firing rates during limb movements, are unknown. Throughout maximal voluntary efforts we found that, in comparison with young adults, firing rates were lower during isometric contraction in older adults but not different during elbow extension movements. Despite the older group being ∼33% weaker across contractions, their muscles can receive neural drive during movements that are similar to that of younger adults.