Aim: The aim of this study was to compare passive muscle stiffness in children with cerebral palsy (CP) and children with typical development using a novel ultrasound technique: ultrasound shear wave elastography (SWE).Method: We conducted a prospective study of 13 children with CP (six females and seven males, median age 5y 1mo [interquartile range 4y 4mo–7y 8mo]) and 13 children with typical development (six females and seven males, median age 5y 3mo [interquartile range 4y 4mo–9y 4mo]). Demographic information and physical exam measurements were obtained in addition to shear modulus measurements (passive muscle stiffness) of the lateral gastrocnemius muscle at 20° plantar flexion, 10° plantar flexion, and 0° plantar flexion using SWE.Results: Children with CP had significantly greater shear modulus measurements at all three foot positions (p<0.050). When the shear modulus values were normalized to the baseline value for each child, there was no significant difference between the two groups.Interpretation: Passive muscle stiffness, measured without the influence of spasticity, is greater in children with CP than in children with typical development when a muscle is at slack and at stretch. When shear modulus was normalized, the results indicate that muscle in children in both groups responds similarly to passive stretch. Further work includes evaluating effect of botulinum toxin on passive muscle properties.

Objectives: The purpose of this study was to investigate the feasibility and reliability of passive muscle stiffness measurements in children by shear wave ultrasound elastography.Methods: We conducted a prospective cross‐sectional study quantifying the passive stiffness of bilateral lateral gastrocnemius muscles during passive stretching in 20 typically developing children (age range, 2.0–12.6 years). Data collected included passive stiffness of the lateral gastrocnemius muscle (shear modulus in kilopascals) at 4 positions of progressive passive foot dorsiflexion, demographic characteristics of the participants, and comparison of demographic characteristics with the shear modulus.Results: Passive stiffness increased with increasing stretching (mean [SD] range of stiffness, 7.1 [2.0] to 36.2 [22.0] kPa). For all 4 foot positions, no significant difference was found between right and left legs (range, P = .42 to P = .98) or between the sexes (range, P = .28 to P > .99). No correlation of passive muscle stiffness with age, body mass index, or ankle range of motion was found. The reliability of measurements was good to excellent (mean [95% confidence interval] range of reliability, 0.67 [0.44–0.83] to 0.80 [0.63–0.90]).Conclusions: Measurements of passive stiffness of the lateral gastrocnemius muscle are feasible and reliable in children as young as 2 years. Because this study found no significant difference between sex and the side tested in this age group, future studies involving children of this age range may not need to be stratified on the basis of these parameters. Defining normal passive muscle stiffness in children is critical for identifying and understanding the implications of abnormal passive muscle stiffness in children with neuromuscular disorders.

Spastic cerebral palsy (CP), despite the name, is not consistently identifiable by specific brain lesions. CP animal models focus on risk factors for development of CP, yet few reproduce the diagnostic symptoms. Animal models of CP must advance beyond risk factors to etiologies, including both the brain and spinal cord.

Motor neuron (MN) development in early onset spasticity is poorly understood. For example, spastic cerebral palsy (sCP), the most common motor disability of childhood, is poorly predicted by brain imaging, yet research remains focused on the brain. By contrast, MNs, via the motor unit and neurotransmitter signaling, are the target of most therapeutic spasticity treatments and are the final common output of motor control. MN development in sCP is a critical knowledge gap, because the late embryonic and postnatal periods are not only when the supposed brain injury occurs but also are critical times for spinal cord neuromotor development. Using an animal model of early onset spasticity [ spa mouse (B6.Cg- Glrb^spa/J) with a glycine (Gly) receptor mutation], we hypothesized that removal of effective glycinergic neurotransmitter inputs to MNs during development will influence MN pruning (including primary dendrites) and MN size. Spa (Glrb−/−) and wild-type (Glrb+/+) mice, ages 4–9 wk, underwent unilateral retrograde labeling of the tibialis anterior muscle MNs via peroneal nerve dip in tetramethylrhodamine. After 3 days, mice were euthanized and perfused with 4% paraformaldehyde, and the spinal cord was excised and processed for confocal imaging. Spa mice had ~61% fewer lumbar tibialis anterior MNs ( P < 0.01), disproportionately affecting larger MNs. Additionally, a ~23% reduction in tibialis anterior MN somal surface area ( P < 0.01) and a 12% increase in primary dendrites ( P = 0.046) were observed. Thus MN pruning and MN somal surface area are abnormal in early onset spasticity. Fewer and smaller MNs may contribute to the spastic phenotype.NEW & NOTEWORTHY Motor neuron (MN) development in early onset spasticity is poorly understood. In an animal model of early onset spasticity, spa mice, we found ~61% fewer lumbar tibialis anterior MNs compared with controls. This MN loss disproportionately affected larger MNs. Thus number and heterogeneity of the MN pool are decreased in spa mice, likely contributing to the spastic phenotype.

Phrenic motor neuron (PhMN) development in early onset hypertonia is poorly understood. Yet, respiratory disorders are a common cause of morbidity and mortality. In spa mice, an animal model of early onset hypertonia, we found ~30% fewer PhMNs, compared with controls. This PhMN loss disproportionately affected larger PhMNs. Thus, the number and heterogeneity of the PhMN pool are decreased in spa mice, likely contributing to the hypertonia, impaired neuromotor control, and respiratory disorders.