The effects of auditory cues and weighted pens on handwriting in individuals with Parkinson's disease.

BACKGROUND: Micrographia, or small handwriting, is a common symptom of Parkinson's disease (PD). Weighted pens have previously been recommended to improve handwriting, but there is limited research supporting their effectiveness. Additionally, previous research has demonstrated that music as an auditory cue can reduce variability in fine motor movements, but its effect on handwriting in people with PD remains unknown. PURPOSE: This study explored potential handwriting interventions for people with PD by evaluating the effectiveness of weighted pens and auditory cues on handwriting. STUDY DESIGN: This was a pilot cohort study. METHODS: Eight older adults with PD used a standard pen and a weighted pen to write continuous cursive "l"s on 1.5-cm-lined paper for a total of 10 seconds while listening to auditory cues in 4 conditions: control (silence), metronome, activating music, and relaxing music. Kinematic data were measured with sensors attached to the tip of each pen, and muscle activity was measured with electromyography sensors adhered to the extensor digitorum communis and first dorsal interosseous. RESULTS: When writing with the standard pen, peak-to-peak time was reduced in the metronome (control = 0.807 ± 0.121 seconds, metronome = 0.701 ± 0.100 seconds, p = 0.024) and activating (control = 0.807 ± 0.121 seconds, activating = 0.691 ± 0.113 seconds, p = 0.009) conditions compared to the control condition. Furthermore, the weighted pen increased the variability of distance between letter peaks (standard = 0.187 ± 0.010, weighted = 0.482 ± 0.065, p = 0.033) and the variability of time needed to complete each letter (standard = 0.176 ± 0.010, weighted = 0.187 ± 0.016, p = 0.042) compared to the standard pen. Finally, area under the curve of the extensor digitorum communis was reduced in the metronome (metronome = 66.03 ± 25.74 mV, control = 88.98 ± 30.40 mV, p = 0.034) and activating music (activating = 66.49 ± 26.02 mV, control = 88.98 ± 30.40 mV, p = 0.012) conditions compared to control when writing with the standard pen. CONCLUSIONS: These results suggest that weighted pens may not improve handwriting in novice users, but auditory cues appear beneficial. This can inform future directions in the research and clinical application of handwriting interventions for persons with PD.

Aberrant age-related alterations in spontaneous cortical activity in participants with cerebral palsy.

Introduction: Cerebral Palsy (CP) is the most common neurodevelopmental motor disability, resulting in life-long sensory, perception and motor impairments. Moreover, these impairments appear to drastically worsen as the population with CP transitions from adolescents to adulthood, although the underlying neurophysiological mechanisms remain poorly understood. Methods: We began to address this knowledge gap by utilizing magnetoencephalographic (MEG) brain imaging to study how the amplitude of spontaneous cortical activity (i.e., resting state) is altered during this transition period in a cohort of 38 individuals with spastic diplegic CP (Age range = 9.80-47.50 years, 20 females) and 67 neurotypical controls (NT) (Age range = 9.08-49.40 years, Females = 27). MEG data from a five-minute eyes closed resting-state paradigm were source imaged, and the power within the delta (2-4 Hz), theta (5-7 Hz), alpha (8-12 Hz), beta (15-29 Hz), and gamma (30-59 Hz) frequency bands were computed. Results: For both groups, the delta and theta spontaneous power decreased in the bilateral temporoparietal and superior parietal regions with age, while alpha, beta, and gamma band spontaneous power increased in temporoparietal, frontoparietal and premotor regions with age. We also found a significant group x age interaction, such that participants with CP demonstrated significantly less age-related increases in the spontaneous beta activity in the bilateral sensorimotor cortices compared to NT controls. Discussion: Overall, these results demonstrate that the spontaneous neural activity in individuals with CP has an altered trajectory when transitioning from adolescents to adulthood. We suggest that these differences in spontaneous cortical activity may play a critical role in the aberrant motor actions seen in this patient group, and may provide a neurophysiological marker for assessing the effectiveness of current treatment strategies that are directed at improving the mobility and sensorimotor impairments seen in individuals with CP.

Pain interference and fatigue in limb-girdle muscular dystrophy R9.

Pain is prevalent in individuals with limb-girdle muscular dystrophy (LGMD) R9, but impact on daily living and correlation with fatigue remain unknown. Patient-Reported Outcomes Measurement Information System (PROMIS) pain interference and fatigue short forms were completed annually by 23 children and 54 adults with biallelic fukutin-related protein (FKRP) variants for up to six years. Concurrent motor and pulmonary function were evaluated. Pain interference T-scores were near the normal mean of 50 by linear mixed model analysis (48.5 in children, 51.6 in adults). 58% of participants experienced pain interference levels greater than the general population on at least one assessment. Fatigue T-scores were elevated in adults but not children (49.0 in children, 55.1 in adults), and 75% had at least one elevated fatigue score. Of participants with at least two visits, serial scores were not consistent across visits, without a clear pattern. Pain interference and fatigue were positively correlated (r = 0.55). Both increased with older age (r = 0.21 and 0.41 respectively). Neither differed by sex or ambulation status. Motor (r=-0.32) and pulmonary (r=-0.25) function correlated with fatigue in adults, not children. Results suggest that pain in those with LGMDR9 is variable and episodic, limiting impact on daily life, while fatigue increases over time.

Aberrant movement-related somatosensory cortical activity mediates the extent of the mobility impairments in persons with cerebral palsy.

There are numerous clinical reports showing that persons with cerebral palsy (CP) have proprioceptive, stereognosis, and tactile discrimination deficits. The current consensus is that these altered perceptions are attributable to aberrant somatosensory cortical activity. It has been inferred from these data that persons with CP do not adequately process ongoing sensory feedback during motor actions, which accentuates the extent of their mobility impairments. However, this hypothesis has yet to be directly tested. We used magnetoencephalographic brain imaging to address this knowledge gap by quantifying the somatosensory dynamics evoked by applying electrical stimulation to the tibial nerve in 22 persons with CP and 25 neurotypical controls at rest and during an ankle plantarflexion isometric force motor task. We also quantified the spatiotemporal gait biomechanics of participants outside the scanner. Consistent with the literature, our results confirmed that the strength of somatosensory cortical activity was weaker in the persons with CP compared to the neurotypical controls. Our results also showed that the strength of the somatosensory cortical responses were significantly weaker during the isometric ankle force task than at rest. Most importantly, our results showed that the strength of somatosensory cortical activity during the ankle plantarflexion force production task mediated the relationship between somatosensory cortical activity at rest and both walking velocity and step length. These results suggest that youth with CP have aberrant somatosensory cortical activity during isometric force generation, which ultimately contributes to the extent of mobility impairments seen in this patient population. KEY POINTS: Persons with cerebral palsy have reduced somatosensory cortical responses at rest and during movement. The somatosensory cortical responses during movement mediate the relationship between the somatosensory cortical responses at rest and mobility. Persons with cerebral palsy may have altered sensorimotor feedback that ultimately contributes to impaired mobility.

Altered spontaneous cortical activity predicts pain perception in individuals with cerebral palsy.

Cerebral palsy is the most common paediatric neurological disorder and results in extensive impairment to the sensorimotor system. However, these individuals also experience increased pain perception, resulting in decreased quality of life. In the present study, we utilized magnetoencephalographic brain imaging to examine whether alterations in spontaneous neural activity predict the level of pain experienced in a cohort of 38 individuals with spastic diplegic cerebral palsy and 67 neurotypical controls. Participants completed 5 min of an eyes closed resting-state paradigm while undergoing a magnetoencephalography recording. The magnetoencephalographic data were then source imaged, and the power within the delta (2-4 Hz), theta (5-7 Hz), alpha (8-12 Hz), beta (15-29 Hz), low gamma (30-59 Hz) and high gamma (60-90 Hz) frequency bands were computed. The resulting power spectral density maps were analysed vertex-wise to identify differences in spontaneous activity between groups. Our findings indicated that spontaneous cortical activity was altered in the participants with cerebral palsy in the delta, alpha, beta, low gamma and high gamma bands across the occipital, frontal and secondary somatosensory cortical areas (all p FWE < 0.05). Furthermore, we also found that the altered beta band spontaneous activity in the secondary somatosensory cortices predicted heightened pain perception in the individuals with cerebral palsy (P = 0.039). Overall, these results demonstrate that spontaneous cortical activity within individuals with cerebral palsy is altered in comparison to their neurotypical peers and may predict increased pain perception in this patient population. Potentially, changes in spontaneous resting-state activity may be utilized to measure the effectiveness of current treatment approaches that are directed at reducing the pain experienced by individuals with cerebral palsy.

Spinal cord microstructural changes are connected with the aberrant sensorimotor cortical oscillatory activity in adults with cerebral palsy.

Previous animal models have illustrated that reduced cortical activity in the developing brain has cascading activity-dependent effects on the microstructural organization of the spinal cord. A limited number of studies have attempted to translate these findings to humans with cerebral palsy (CP). Essentially, the aberrations in sensorimotor cortical activity in those with CP could have an adverse effect on the spinal cord microstructure. To investigate this knowledge gap, we utilized magnetoencephalographic (MEG) brain imaging to quantify motor-related oscillatory activity in fourteen adults with CP and sixteen neurotypical (NT) controls. A subset of these participants also underwent cervical-thoracic spinal cord MRI. Our results showed that the strength of the peri-movement beta desynchronization and the post-movement beta rebound were each weaker in the adults with CP relative to the controls, and these weakened responses were associated with poorer task performance. Additionally, our results showed that the strength of the peri-movement beta response was associated with the total cross-sectional area of the spinal cord and the white matter cross-sectional area. Altogether these results suggest that the altered sensorimotor cortical activity seen in CP may result in activity-dependent plastic changes within the spinal cord microstructure, which could ultimately contribute to the sensorimotor deficits seen in this population.

Therapeutic Lower Extremity Power Training Alters the Sensorimotor Cortical Activity of Individuals With Cerebral Palsy.

Objective: To utilize magnetoencephalographic (MEG) brain imaging to examine potential changes in sensorimotor cortical oscillations after therapeutic power training in individuals with cerebral palsy (CP). Design: Cohort. Setting: Academic medical center. Participants: Individuals with CP (N=11; age=15.9±1.1 years; Gross Motor Function Classification System I- III) and neurotypical controls (NTs; N=16; age=14.6±0.8 years). Interventions: Participants with CP underwent 24 (8 weeks; 3 days a week) sessions of high-velocity lower extremity power training on a leg press. The NTs underwent single baseline MEG assessments. Main Outcome Measures: Pre-post bilateral leg press 1-repetition maximum and peak power production were used to assess the muscular performance changes. The 10-m walk and 1-minute walk tests were used to assess mobility changes. During MEG recordings, participants used their right leg to complete a goal-directed isometric target-matching task. Advanced beamforming methods were subsequently used to image the strength of the sensorimotor beta oscillatory power. Results: Before the therapeutic power training, the participants with CP had stronger beta sensorimotor cortical oscillations compared with the NT controls. However, the beta sensorimotor cortical oscillations were weaker and approximated the controls after the participants with CP completed the therapeutic power training protocol. There also was a link between the amount of improvement in leg peak power production and the amount of reduction in sensorimotor cortical oscillations seen after therapy. Conclusions: Therapeutic power training appears to optimize the sensorimotor cortical oscillations of individuals with CP, and these neuroplastic changes partly contribute to improvements in the leg peak power production of individuals with CP. Therapeutic power training might provide the key ingredients for beneficial neuroplastic change.