Loretta A. Staudt

Selective Control Assessment of the Lower Extremity (SCALE): development, validation, and interrater reliability of a clinical tool for patients with cerebral palsy

Normal selective voluntary motor control (SVMC) can be defined as the ability to perform isolated joint movement without using mass flexor/extensor patterns or undesired movement at other joints, such as mirroring. SVMC is an important determinant of function, yet a valid, reliable assessment tool is lacking. The Selective Control Assessment of the Lower Extremity (SCALE) is a clinical tool developed to quantify SVMC in patients with cerebral palsy (CP). This paper describes the development, utility, validation, and interrater reliability of SCALE. Content validity was based on review by 14 experienced clinicians. Mean agreement was 91.9% (range 71.4–100%) for statements about content, administration, and grading. SCALE scores were compared with Gross Motor Function Classification System Expanded and Revised (GMFCS‐ER) levels for 51 participants with spastic diplegic, hemiplegic, and quadriplegic CP (GMFCS levels I – IV, 21 males, 30 females; mean age 11y 11mo [SD 4y 9mo]; range 5–23y). Construct validity was supported by significant inverse correlation (Spearmans r=‐0.83, p<0.001) between SCALE scores and GMFCS levels. Six clinicians rated 20 participants with spastic CP (seven males, 13 females, mean age 12y 3mo [SD 5y 5mo], range 7–23y) using SCALE. A high level of interrater reliability was demonstrated by intraclass correlation coefficients ranging from 0.88 to 0.91 (p<0.001).

Spasticity in Cerebral Palsy and the Selective Posterior Rhizotomy Procedure

A review of the selective posterior rhizotomy procedure for reduction of spasticity in cerebral palsy is presented. The history of the procedure, selection of patients, operative technique, and results are described. The neurophysiologic basis for spasticity is considered, as well as the role of spasticity in the complex motor disorder of cerebral palsy. Cerebral palsy is a multifaceted disorder of which spasticity is only one aspect. Reduction of spasticity can be effectively achieved using the current technique of selective posterior rhizotomy, but careful patient selection and establishment of realistic goals are vital to successful outcome. Postoperative physical and occupational therapy are felt to be essential for regaining strength and improving motor function following the rhizotomy procedure. Further study in the areas of spasticity, cerebral palsy, and the effects of rhizotomy is expected to advance our treatment of spastic children. ( J Child Neurol 1990;5:179-185).

Lower-extremity selective voluntary motor control in patients with spastic cerebral palsy: increased distal motor impairment

Aim  Multiple impairments contribute to motor deficits in spastic cerebral palsy (CP). Selective voluntary motor control (SVMC), namely isolation of joint movement upon request, is important, but frequently overlooked. This study evaluated the proximal to distal distribution of SVMC impairment among lower extremity joints.

Intraoperative monitoring during selective posterior rhizotomy: technique and patient outcome.

We evaluated intraoperative electromyographic (EMG) results among 110 pediatric patients with spastic cerebral palsy who underwent selective posterior rhizotomy. We analyzed surgical outcomes for 60 of these patients who returned for follow-up assessment between 4 and 17 months postoperatively. Reduction in muscle tone (resistance to passive movement), increased range of motion and improvements in functional skills were seen at follow-up. To control for possible changes due to development, participation in therapy, or instability of measurements, 30 rhizotomy patients were evaluated twice during a baseline period of several months prior to surgery. No significant changes were found between these two measurement sessions during the baseline control period suggesting that the rhizotomy surgery itself caused the postoperative improvements. These intraoperative EMG monitoring techniques have been adopted at many other centers but variations in specific methods and EMG criteria have developed subsequently among major hospitals where selective posterior rhizotomy is performed. These variations in neurophysiologic methods and recent controversy about the usefulness of such intraoperative EMG monitoring created a need for us to publish our standard EMG selection technique. We describe here, in detail, methods for nerve rootlet testing and selection.

Selective Posterior Rhizotomy: Evolution of Theory and Practice

The spastic type of cerebral palsy has become more prevalent due to improved survival rates of premature infants. Selective posterior rhizotomy is a neurosurgical procedure which is designed to reduce spasticity and has been successfully used for children with spastic cerebral palsy. Although the procedure of posterior rhizotomy is nearly 100 years old, a revised technique was introduced in 1981. Since that time, the procedure has been further refined and its use has altered the theory and practice of cerebral palsy management. This paper reviews the neurophysiological and anatomical facts that have influenced the development of the technique. The current practice of selective posterior rhizotomy is an evolving process which has stimulated scientific interest in spasticity, cerebral palsy and its management.

Perioperative complications in children undergoing selective posterior rhizotomy: a review of 105 cases

Medical histories for 105 consecutive children who underwent selective posterior rhizotomy (SPR) were reviewed to determine the incidence and clinical significance of adverse events related to anaesthesia and surgery. No intraoperative or postoperative events with potential for lasting morbidity, nor life threatening events, were identified. Intraoperatively, the most common adverse events were moderate elevation of body temperature (13/105) and transient dysrhythmias (8/105). The most frequent postoperative complications were fever, marginal oxygen saturation in the absence of supplemental oxygen, and postcatheterization cystitis. Early surgical complications, such as wound infection, cerebrospinal fluid leak, haemorrhage, and bowel or bladder disturbance were absent in this series. Surgical technique and anaesthetic management are described.

Selective Posterior Rhizotomy: Further Comments:

neurologist sees patients who suffered an upper motor neuron lesion as a result of insult to the mature central nervous system due to spinal cord injury, stroke, multiple sclerosis, etc. Children with cerebral palsy are not miniature adults with upper motor neuron lesions, but have sustained an upper motor neuron lesion at an early point in development. Frequently the neurologic damage is associated with the sequelae of premature birth or the insult may occur perinatally in the full-term infant. The peak time period for organizational events in the development of the central nervous system is from the sixth month of gestation to several years postnatal.2 This period is critical for establishing the circuitry of the brain and is followed

Hip kinetics during gait are clinically meaningful outcomes in young boys with Duchenne muscular dystrophy

Duchenne muscular dystrophy (DMD) is an X-linked genetic neuromuscular disorder characterized by progressive proximal to distal muscle weakness. The success of randomized clinical trials for novel therapeutics depends on outcome measurements that are sensitive to change. As the development of motor skills may lead to functional improvements in young boys with DMD, their inclusion may potentially confound clinical trials. Three-dimensional gait analysis is an under-utilized approach that can quantify joint moments and powers, which reflect functional muscle strength. In this study, gait kinetics, kinematics, spatial-temporal parameters, and timed functional tests were quantified over a one-year period for 21 boys between 4 and 8 years old who were enrolled in a multisite natural history study. At baseline, hip moments and powers were inadequate. Between the two visits, 12 boys began a corticosteroid regimen (mean duration 10.8±2.4 months) while 9 boys remained steroid-naïve. Significant between-group differences favoring steroid use were found for primary kinetic outcomes (peak hip extensor moments (p=.007), duration of hip extensor moments (p=.007), peak hip power generation (p=.028)), and spatial-temporal parameters (walking speed (p=.016) and cadence (p=.021)). Significant between-group differences were not found for kinematics or timed functional tests with the exception of the 10m walk test (p=.03), which improves in typically developing children within this age range. These results indicate that hip joint kinetics can be used to identify weakness in young boys with DMD and are sensitive to corticosteroid intervention. Inclusion of gait analysis may enhance detection of a treatment effect in clinical trials particularly for young boys with more preserved muscle function.

The role of selective posterior rhizotomy in the management of cerebral palsy

Cerebral palsy is a static, central nervous system disorder variably manifested by spasticity, dystonia, athetosis, weakness, persistent primitive reflexes, and disordered motor control. When spasticity is the major interfering factor in a childs independent function or daily care, surgical reduction of spasticity can be beneficial. Selective posterior rhizotomy is a neurosurgical procedure designed to decrease spasticity. The history, rationale, patient selection, surgical technique, and postoperative management of selective posterior rhizotomy are reviewed, emphasizing the role of a multidisciplinary approach to facilitate patient evaluation and management.

Prednisone and Deflazacort in Duchenne Muscular Dystrophy: Do They Play a Different Role in Child Behavior and Perceived Quality of Life?

The aim of this study was to determine whether prednisone and deflazacort play a different role in child behavior and perceived health related psychosocial quality of life in ambulant boys with Duchenne Muscular Dystrophy. As part of a prospective natural-history study, parents of sixty-seven ambulant boys with DMD (27 taking prednisone, 15 taking deflazacort, 25 were steroid naïve) completed the Child Behavior Checklist (CBCL) for assessment of behavioral, emotional and social problems and both parents and boys with DMD completed the PedsQL™4.0 generic core scale short form. Boys with DMD had higher rates of general behavioral problems than age-matched peers. No significant differences were found among the groups for any of the CBCL syndrome scales raw scores, including internalizing and externalizing behaviors; however, on average boys taking deflazacort demonstrated more withdrawn behaviors than those taking prednisone, while on average the boys taking prednisone demonstrated more aggressive behaviors than boys taking deflazacort. Age, internalizing and externalizing behaviors accounted for 39 and 48% of the variance in psychosocial quality of life for both parents and boys with DMD, respectively. Overall, the use of steroids was not associated with more behavioral problems in boys with DMD. As behavior played a significant role in psychosocial quality of life, comprehensive assessment and treatment of behavioral problems is crucial in this population.