Erin E. Butler

DEFINITION AND CLASSIFICATION OF HYPERKINETIC MOVEMENTS IN CHILDHOOD

Hyperkinetic movements are unwanted or excess movements that are frequently seen in children with neurologic disorders. They are an important clinical finding with significant implications for diagnosis and treatment. However, the lack of agreement on standard terminology and definitions interferes with clinical treatment and research. We describe definitions of dystonia, chorea, athetosis, myoclonus, tremor, tics, and stereotypies that arose from a consensus meeting in June 2008 of specialists from different clinical and basic science fields. Dystonia is a movement disorder in which involuntary sustained or intermittent muscle contractions cause twisting and repetitive movements, abnormal postures, or both. Chorea is an ongoing random‐appearing sequence of one or more discrete involuntary movements or movement fragments. Athetosis is a slow, continuous, involuntary writhing movement that prevents maintenance of a stable posture. Myoclonus is a sequence of repeated, often nonrhythmic, brief shock‐like jerks due to sudden involuntary contraction or relaxation of one or more muscles. Tremor is a rhythmic back‐and‐forth or oscillating involuntary movement about a joint axis. Tics are repeated, individually recognizable, intermittent movements or movement fragments that are almost always briefly suppressible and are usually associated with awareness of an urge to perform the movement. Stereotypies are repetitive, simple movements that can be voluntarily suppressed. We provide recommended techniques for clinical examination and suggestions for differentiating between the different types of hyperkinetic movements, noting that there may be overlap between conditions. These definitions and the diagnostic recommendations are intended to be reliable and useful for clinical practice, communication between clinicians and researchers, and for the design of quantitative tests that will guide and assess the outcome of future clinical trials.

Neonatal brain structure on MRI and diffusion tensor imaging, sex, and neurodevelopment in very‐low‐birthweight preterm children

The neurological basis of an increased incidence of cerebral palsy (CP) in preterm males is unknown. This study examined neonatal brain structure on magnetic resonance imaging (MRI) and diffusion tensor imaging (DTI) at term‐equivalent age, sex, and neurodevelopment at 1 year 6 months on the basis of the Amiel−Tison neurological examination, Gross Motor Function Classification System, and Bayley Scales of Infant Development in 78 very‐low‐birthweight preterm children (41 males, 37 females; mean gestational age 27.6wks, SD 2.5; mean birthweight 1021g, SD 339). Brain abnormalities on MRI and DTI were not different between males and females except in the splenium of the corpus callosum, where males had lower DTI fractional anisotropy (p=0.025) and a higher apparent diffusion coefficient (p=0.013), indicating delayed splenium development. In the 26 infants who were at higher risk on the basis of DTI, males had more abnormalities on MRI (p=0.034) and had lower fractional anisotropy and a higher apparent diffusion coefficient in the splenium (p=0.049; p=0.025) and right posterior limb of the internal capsule (PLIC; p=0.003; p=0.033). Abnormal neurodevelopment was more common in males (n=9) than in females (n=2; p=0.036). Children with abnormal neurodevelopment had more abnormalities on MRI (p=0.014) and reduced splenium and right PLIC fractional anisotropy (p=0.001; p=0.035). In children with abnormal neurodevelopment, right PLIC fractional anisotropy was lower than left (p=0.035), whereas in those with normal neurodevelopment right PLIC fractional anisotropy was higher than left (p=0.001). Right PLIC fractional anisotropy correlated to neurodevelopment (rho=0.371, p=0.002). Logistic regression predicted neurodevelopment with 94% accuracy; only right PLIC fractional anisotropy was a significant logistic coefficient. Results indicate that the higher incidence of abnormal neurodevelopment in preterm males relates to greater incidence and severity of brain abnormalities, including reduced PLIC and splenium development.

Three-dimensional kinematics of the upper limb during a Reach and Grasp Cycle for children

The ability to reach, grasp, transport, and release objects is essential for activities of daily living. The objective of this study was to develop a quantitative method to assess upper limb motor deficits in children with cerebral palsy (CP) using three-dimensional motion analysis. We report kinematic data from 25 typically developing (TD) children (11 males, 14 females; ages 5-18 years) and 2 children with spastic hemiplegic CP (2 females, ages 14 and 15 years) during the Reach and Grasp Cycle. The Cycle includes six sequential tasks: reach, grasp cylinder, transport to mouth (T(1)), transport back to table (T(2)), release cylinder, and return to initial position. It was designed to represent a functional activity that was challenging yet feasible for children with CP. For example, maximum elbow extension was 43+/-11 degrees flexion in the TD group. Consistent kinematic patterns emerged for the trunk and upper limb: coefficients of variation at point of task achievement for reach, T(1), and T(2) for trunk flexion-extension were (.11, .11, .11), trunk axial rotation (.06, .06, .06), shoulder elevation (.13, .11, .13), elbow flexion-extension (.25, .06, .23), forearm pronation-supination (.08, .10, .11), and wrist flexion-extension (.25, .21, .22). The children with CP demonstrated reduced elbow extension, increased wrist flexion and trunk motion, with an increased tendency to actively externally rotate the shoulder and supinate the forearm during T(1) compared to the TD children. The consistent normative data and clinically significant differences in joint motion between the CP and TD children suggest the Reach and Grasp Cycle is a repeatable protocol for objective clinical evaluation of functional upper limb motor performance.

Temporal-spatial parameters of the upper limb during a Reach & Grasp Cycle for children

The objective of this study was to characterize normal temporal-spatial patterns during the Reach & Grasp Cycle and to identify upper limb motor deficits in children with cerebral palsy (CP). The Reach & Grasp Cycle encompasses six sequential tasks: reach, grasp cylinder, transport to self (T(1)), transport back to table (T(2)), release cylinder, and return to initial position. Three-dimensional motion data were recorded from 25 typically developing children (11 males, 14 females; ages 5-18 years) and 12 children with hemiplegic CP (2 males, 10 females; ages 5-17 years). Within-day and between-day coefficients of variation for the control group ranged from 0 to 0.19, indicating good repeatability of all parameters. The mean duration of the Cycle for children with CP was nearly twice as long as controls, 9.5±4.3s versus 5.1±1.2s (U=37.0, P=.002), partly due to prolonged grasp and release durations. Peak hand velocity occurred at approximately 40% of each phase and was greater during the transport (T(1), T(2)) than non-transport phases (reach, return) in controls (P<.001). Index of curvature was lower during transport versus non-transport phases for all children. Children with CP demonstrated an increased index of curvature during reach (U=46.0, P=.0074) and an increased total number of movement units (U=16.5, P<.0001) compared to controls, indicating less efficient and less smooth movements. Total duration of the Reach & Grasp Cycle (rho=.957, P<.0001), index of curvature during reach and T(1) (rho=.873, P=.0002 and rho=.778, P=.0028), and total number of movement units (rho=.907, P<.0001) correlated strongly with MACS score. The consistent normative data and the substantial differences between children with CP and controls reflect utility of the Reach & Grasp Cycle for quantitative evaluation of upper limb motor deficits.

The Pediatric Upper Limb Motion Index and a temporal-spatial logistic regression: Quantitative analysis of upper limb movement disorders during the Reach & Grasp Cycle

This study describes a novel pediatric upper limb motion index (PULMI) for children with cerebral palsy (CP). The PULMI is based on three-dimensional kinematics and provides quantitative information about upper limb motion during the Reach & Grasp Cycle. We also report key temporal-spatial parameters for children with spastic, dyskinetic, and ataxic CP. Participants included 30 typically-developing (TD) children (age=10.9±4.1 years) and 25 children with CP and upper limb involvement (age=12.3±3.7 years), Manual Ability Classification System (MACS) levels I-IV. The PULMI is calculated from the root-mean-square difference for eight kinematic variables between each child with CP and the average TD values, and scaled such that the TD PULMI is 100±10. The PULMI was significantly lower among children with CP compared to TD children (Wilcoxon Z=-5.06, p<.0001). PULMI scores were significantly lower among children with dyskinetic CP compared to spastic CP (Z=-2.47, p<.0135). There was a strong negative correlation between PULMI and MACS among children with CP (Spearmans rho=-.78, p<.0001). Temporal-spatial values were significantly different between CP and TD children: movement time (Z=4.06, p<.0001), index of curvature during reach (Z=3.68, p=.0002), number of movement units (Z=3.72, p=.0002), angular velocity of elbow extension during reach (Z=-3.96, p<.0001), and transport(1):reach peak velocities (Z=-2.48, p=.0129). A logistic regression of four temporal-spatial parameters, the Pediatric Upper Limb Temporal-Spatial Equation (PULTSE), correctly predicted 19/22 movement disorder subtypes (spastic versus dyskinetic CP). The PULMI, PULTSE, and key temporal-spatial parameters of the Reach & Grasp Cycle offer a quantitative approach to analyzing upper limb function in children with CP.