The New York Times recently ran a story of a 31 year –old with cerebral palsy and his remarkable response to dance lessons and muscle relaxation techniques. AOL picked up the story and interviewed our Medical Director, Dr. Mindy Aisen, about its potential usefulness as a therapy to improve function in individuals with CP.  Now, Good Morning America has done a piece on this same remarkable story of Gregg Mozgla and how he reduced his symptoms of cerebral palsy through ballet.

http://www.aolhealth.com/condition-center/cerebral-palsy/overcoming-cerebral-palsy

Here’s a link to the GMA piece: http://abcnews.go.com/video/playerIndex?id=9600885

Is Dance an Effective Therapy for Cerebral Palsy?

By Justine van der Leun

Photo: Andrea Mohin, The New York Times/Redux

Gregg Mozgala, a 31-year-old actor, used to feel inhibited by his cerebral palsy, a neurological condition that occurs when a child’s brain is damaged before the age of two and afflicts a million Americans — most often in the form of poor coordination, weak muscles, and compromised posture. But with a load of determination and the help of an unconventional choreographer, Mozgala is now set to star in an hour-long dance piece in New York City. “I have felt things that I felt were completely closed off to me for the last 30 years,” Mozgala told The New York Times. “The amount of sensation that comes through the work has been totally unexpected and is really quite wonderful.” While there is no cure for cerebral palsy, Mozgala’s success suggests that a change in approach to the condition can translate into a change in the lives and capabilities of sufferers.

Mozgala’s journey began in 2008 when he met choreographer Tamar Rogoff (pictured above doing body work with Mozgala). After seeing Mozgala play the lead in “Romeo and Juliet,” produced by Theater Breaking Through Barriers, a group composed of disabled and non-disabled actors, Rogoff was inspired to create a dance piece for him. At first, both Mozgala and Rogoff imagined a 10-minute performance: Mozgala, who then walked on his toes with his upper body thrown back, assumed he could not manage much more, and Rogoff figured she would create some basic choreography for him. But as they began to work together, her imagination and his capacities began to expand.

Rogoff, who knew little about cerebral palsy, taught Mozgala techniques to release muscular tension. She helped him locate areas of his body over which he had previously exercised no control. In agonizing and illuminating sessions, they worked together to increase his range of movement, employing dance and stretching techniques, and finding his true physical limits. Soon enough, Mozgala was able to stand up straight, to place both feet on the floor, as well as to feel his Achilles tendon, which he had never before done. He called these revelations “eureka moments” in the New York Times interview.

“There are pre-existing structures in the brain that are very receptive to music, rhythm, and moving to music, which is why at a rock concert, everyone is swaying,” explained Mindy L. Aisen, MD, medical director of The Cerebral Palsy International Research Foundation. “The innate pleasure we get from music acted as a reinforcement for getting [Mozgala's] body re-engaged and for forging new pathways in his brain.”

Mozgala had been to physical therapists for over a decade, but his dance training was different: While before, the therapists had moved his body for him, now he learned how to move his own body. Rogoff identified some of the physical patterns he had been stuck in and gave him specific instructions on how to overcome them, both in the studio and out. His daily life has changed: His balance and strength are so improved that he rarely falls; his gait is steadier, and he is subject to fewer stares on the street. Most important, he no longer feels mentally constrained by cerebral palsy. As he told the Times: “Everybody told me there was nothing I could do,” he said. “That’s just what you hear, from the time you’re five to adulthood. Tamar gave me an option.”

According to Aisen, Mozgala’s story supports an open-minded, patient-centered approach to Cerebral Palsy and other neurological impairments. CPIRF is considering funding a dance therapy program, and at their Washington, D.C. Center, they have begun to use robotics and fun, motivational virtual games to help children use afflicted muscles. “Just as musicians have to practice to hone a motor skill, a brain that’s never had a chance to develop in areas needs the opportunity,” says Aisen. “We think we can help anyone with cerebral palsy reshape their nervous system in a way. It’s not a cure, but it is going towards a cure.”

Dr. Mindy Aisen gave a plenary talk at the 63rd AACPDM Annual Meeting on September 26th in Scottsdale, Arizona. Her talk was entitled ‘ Updating Research Priorities for Cerebral Palsy’.

Download her Power Point Presentation here.
(Problems? Try right-clicking and “Save Target As…”)

By Dr. Mindy Aisen

The field of stem cell research offers enormous promise for the treatment of human disease. Stem cells are “undifferentiated” cells; they have the potential to develop into virtually any specialized type of cell in the body. So theoretically, stem cells might be used to regenerate human tissue or organs damaged by disease. There is hope that some day stem cells may provide effective restorative therapy for many human conditions, including those caused by damage the nervous system such as stroke, Parkinson’s Disease, spinal cord injury and cerebral palsy. At the present time, however, the field of stem cell research is in its infancy, and there are few effective uses of this type of therapy.

Stem cells can be derived in several ways. Embryonic stem cells (removed from early stage embryos) can be maintained in the laboratory and coaxed into differentiating into various cell types. Fetal cord blood contains human stem cells that can also be grown in the laboratory or frozen for later use. A recent technique for generating human stem cells involves obtaining cells from skin biopsies and “deprogramming” them into stem cells that can then be differentiated into various human cell types. The adult human brain contains viable neural stem cells; it may be possible to use medications to stimulate these natural stem cells into regenerating damaged brain tissue.

Stem cell scientists are currently working on the optimal conditions to maintain stem cells in the laboratory, the precise methods necessary to convert stem cells into the specific cells that might reconstitute damaged human tissues, and the techniques required to direct such cells to function effectively together. At the present time, much work remains to be done before the practical application of stem cells to the treatment of human disease becomes feasible.

In cerebral palsy, brain tissue is damaged at a very early stage of development: in utero, at or around the time of birth, or up until the age of 2 years. The brain is, of course, the most incredibly complex of all human organs. The brain consists of many different cell types interacting in a precisely organized fashion to produce the different aspects of thought and behavior in different brain regions. Before stem cells can be used to repair damaged brain tissue in cerebral palsy or any other brain disorder, scientists will have to discover how to turn stem cells into various specific types of brain cells, and induce them to form the precise connections and organization necessary for meaningful brain function. It is likely that stem cells converted into brain cells will have to be implanted in the precise areas that they are needed in order to provide effective treatment.

Specific Types of Approaches: Cord Blood Infusions

It is extremely unlikely that the administration of stem cells by peripheral infusion of cord blood can effectively treat cerebral palsy. Such cells have not been “taught” to form the necessary types of brain cells, and they will not be able to enter the brain, because there is an anatomic and physiological barrier which prevents certain medications and cells from reaching the brain, known as the blood brain barrier.

How can one explain the reports of improvements in the symptoms of cerebral palsy (CP) following cord blood infusions? Medicine is filled with such “anecdotal” reports of improvements when novel treatments are applied to chronic conditions. The symptoms of all chronic conditions fluctuate, and subjective factors, including the optimistic expectations that accompany novel therapies, often seem to alleviate disease manifestations. But in almost all cases, the underlying disease remains unchanged, and there is no meaningful long-term benefit. Indeed, in most cases, the risk of harm outweighs the chance of benefit when unproven novel therapies are used.

CPIRF strongly supports research into the use of stem cells to treat CP. But at present, CPIRF, in consultation with leading stem cell scientists, has reached the firm conclusion that use of cord blood or other forms of stem cell treatments for CP is inadvisable. Basic and applied research into various approaches to stem cell neural regeneration therapy must be vigorously pursued, and CPIRF will continue to fund such efforts. But at present, administration of cord blood to people with CP offers no meaningful chance of benefit.

Furthermore, the long term risks of cord blood infusions have not been studied, receiving infusions in other countries from donated cord blood may have significant risk and certainly present substantial financial strain for the families of those being treated.

We strongly endorse an organized scientifically rigorous initiative focused on rapidly identifying the most effective methods for using stem cell treatments to help repair the damaged brains of children and adults with developmental brain conditions.

By Mindy Aisen, MD

Last month Dr. Paolo Bonato of Spaulding Rehabilitation Hospital reported on his study of using robotic assisted body weight supported treadmill training (BWSTT) to improve walking ability in children with CP. I want to expand on this topic by reporting on the recently published results of three different groups of investigators who are also evaluating the effectiveness of this treatment modality in CP kids. In addition, I want to discuss the use of neuromuscular stimulation used in combination with BWSTT as a potential rehabilitation technique for impaired gait, and finally, I want to re-emphasize the great need for more rigorous, well-controlled clinical trials to provide definitive scientific evidence for the widespread use of this promising intervention for the improvement of gait and ambulatory skills in children with CP.

As Dr. Bonato previously stated, there is growing evidence that the human central nervous system is capable of significant recovery after insult or injury when prescribed an effective treatment modality at the proper dose. In particular, the use of task-specific training such as BWSTT, has shown great promise in helping stroke and motor incomplete spinal cord injured patients regain some walking ability. In addition, BWSTT has shown promise in helping to correct the gait and improve functional ambulation in children with CP. In a non-randomized study of 10 children with CP, some of who were non ambulatory, Schindl et al reported significant improvement in functional ambulation of all 10 children after 3 months of BWSTT ¹. In 2004, Day et al reported a case study in which a 9 year old child with spastic tetraplegic CP who could not support his own weight and had never experienced walking began to walk short distances with a rolling walker after 44 sessions of locomotor training that included BWSTT ².

More recently, Provost et al reported improvement in four out of six ambulatory children with CP after only 2 weeks of BWSTT in twice daily therapy sessions lasting 30 minutes each ³. Four of these children showed improvement in endurance and a functional gait measure. Begnoche et al combined intensive physical therapy with BWSTT in a study of 5 children with spastic CP. The training sessions consisted of 4 weeks of training, three to four sessions per week for 2 hours each. All five children showed significant improvements in motor and ambulatory skills ⁴. Finally, Dodd and Foley conducted a small, controlled clinical trial of 14 children who were matched according to type of CP (spastic, athetoid), age, sex and gross motor functional classification system level. The experimental group underwent BWSTT twice a week for 6 weeks in a school-based program. The experimental group showed significant improvements over the control group in walking speed and a trend towards increased endurance over the range of moderate to severe disability ⁵.

CP may lead to profound muscle weakness in the affected extremities. Stackhouse et al demonstrated that children with CP have large deficits in voluntary muscle activation as compared to a group of age-matched unaffected children ⁶. This inability to produce sufficient force using voluntary contractions may not induce muscle growth during training exercises prescribed for CP kids. Recently, this same group conducted a study using neuromuscular electrical stimulation (NMES) in conjunction with a 12 week isometric strength training program in a group of children with spastic diplegic CP ⁷. The control group participated in the strength training program without the NMES. The investigators found that the NMES group had greater normalized force production for the quadriceps (muscle strength) and greater walking speed post training than did the control group. While to date, NMES in conjunction with BWSTT has not been reported in CP children, it has been shown to be more effective in restoring gait in stroke patients than BWSTT alone ⁸. NMES may enhance the benefits of BWSTT already demonstrated in CP children by recruiting and strengthening muscles that are needed to complete normal gait cycles.

There is limited scientific evidence that supports the use of BWSTT, NMES and many other treatment modalities to improve strength, endurance and functional mobility in children with CP. Unfortunately, none of these modalities have been clearly established as effective in scientifically rigorous, well-controlled clinical trials. Until there is well-established evidence for the use of these interventions they will never come into widespread use for the treatment of gait abnormalities in children with CP because of third-party reimbursement issues. I urge investigators interested in the neurorehabilitation of CP to begin to collaborate on issues of dose, frequency of therapy and different combinations of treatment modalities so that the much needed large clinical trials can begin to take place. Without these studies, treatment advances that are taking place in the treatment of stroke, spinal cord injury and other nervous system disorders will not be realized in the treatment of CP.

       1. Schindl MR. Forstner, C, Kern H, and Hesse S. Treadmill training with partial body weight support in nonambulatory patients with cerebral palsy. Arch Phys Med Rehabil. 2000:81(3) 301-6

       2. Day JA, Fox EJ, Lowe J, Swales HB and Behrman AL. Locomotor training with Partial Body Weight Support on a Treadmill in a non-ambulatory Child with Spastic Tetraplegia Cerebral Palsy: A Case Report. Pediatr Phys Ther 2004: 16(2):106-113.

       3. Provost B, Dieruf K, Burtner PA, Phillips JP, Bernitsky-Beddingfield A, Sullivan KJ, Bowen CA, Toser L. Endurance and gait in children with cerebral palsy after intensive body weight-supported treadmill training. Pediatr. Phys Ther 2007: 19(1)2-10.

       4. Begnoche DM and Pitetti KH. Effects of traditional treatment and partial body weight treadmill training on the motor skills of children with spastic cerebral palsy. A pilot study. Pediatr Phys Ther. 2007 19(1): 11-19.

       5. Dodd KJ and Foley S. Partial body-weight supported treadmill training can improve walking in children with cerebral palsy: a clinical controlled trial. Dev Med Child Neurol. 2007 49(2):101-105.

       6. Stackhouse SK, Binder-Macleod SA, and Lee SC. Voluntary muscle activation, contractile properties and fatigability in children with and without cerebral palsy. Muscle Nerve 2005 31(5):594-601.

       7. Stackhouse SK, Binder-Macleod SA, Stackhouse CA, McCarthy JJ, Prosser LA and Lee SC. Neuromuscular Electrical Stimulation versus Volitional Isometric Strength Training in Children with Spastic Diplegic Cerebral Palsy: A Preliminary Study. Neurorehabil Neural Repair. 2007. Mar 16

       8. Daly JJ, Roenigk KL, Rogers JM, Butler K, Gansen J, McCabe J, Fredrickson E, Holcomb J, Ruff RL: A Randomized Controlled Trial of FNS in Chronic Stroke Subjects. Stroke 2006, 37:172-178.

The Challenges Faced by Today’s Clinical Researchers Measuring Effects of Treatment on Motor Function in Children with Cerebral Palsy

A Review of ongoing efforts supported by the NIH

The NIH Taskforce on Childhood Motor Disorders Met March 4-5, 2006 Terry Sanger, MD, PhD, Daofen Chen, PhD, Mauricio R. Delgado, MD, FRCPC, Deborah Gaebler-Spira, MD, Mark Hallett, MD, Amy Bastian, PhD PT, Max Wiznitzer, MD, Hilla Ben-Pazi, MD, Kristie Bjornson, MS, PT, PCS, Susan Brown, PhD, Nancy Byl, PhD, PT, FAPTA, Noemi Cantin, Dr. Jorge Carranza, Robert Chen, MB, FRCPC, Edward Dabrowski, MD, Diane Damiano, PhD, PT, Scott Delp, PhD, Ruthmary Deuel, MD, Darcy L. Fehlings, MD, Eileen Fowler, PhD, PT, Marjorie A. Garvey, MD, Sharon I. Gorman, PT, MS, Mark Gormley, MD, Edward Hurvitz, MD, Mary Jenkins, MD, PT, JoAnn Kluzik, PhD, PT, Sahana Kukke, MS, Maria Lebiedowska, PhD, Mindy Levin, PhD, Colleen Lewis, Dennis Matthews, MD, Margaret Barry Michaels, PhD, PT, PCS, Cheryl Missiuna, PhD, OTR/L, Helene Polatajko, PhD, OT Reg. (Ont.), OT(C), FCAOT, Karl Rathjen, MD, Jessica Rose Agramonte, PhD, Paul Steinbok, MD, Dagmar Sternad, PhD, Ann Tilton, MD, Johan van Doornik, PhD, Jason Wingert

Note from Mindy Aisen MD, CEO United Cerebral Palsy Research and Educational Foundation. I would like to express my profound thanks to Dr. Sanger and colleagues who are serving on the NIH Taskforce on Childhood Motor Disorders. I provide a summary of the discussions which occurred at the Group’s most recent meeting. I hope this illustrates the challenges that face clinical researchers trying to understand the neurobiology of Cerebral Palsy, and trying to design clinical trials that will provide rigorous evidence to improve care and neurological functions in children and adults with motor disorders. This group is inclusive of all relevant disciplines and represents a bench to bedside philosophy. Their dedication is to be admired, and the work has only just begun.

Introduction

Abnormalities of motor function in childhood are classified by clinicians as “positive” and “negative” signs.
The positive signs are characterized by excessive involuntary muscle activity (muscle tightness: increased tone, spasms, dystonic muscle contractions, spasticity), while negative signs are characterized by an inability to generate desired muscle activity (weakness, paralysis, incoordination (clumsy or inaccurate movements), tremor, inability to perform complex tasks). Medical therapy has often focused upon positive (hypertonicity) signs due to the availability of treatments that reduce muscle strength or activation. However, for many children negative signs (weakness) can be an even greater contributor to disability and decreased participation at school, home, or in social activities.
The first step in finding solutions to medical problems is having a consensus about terminology and interpretation of signs on physical examination. This allows clinician researchers to collaborate on research hypotheses and to develop collaborative clinical research programs with certainty that their colleagues are recording the same clinical signs and responses to treatments.
At a meeting in March 2005 on the campus of the National Institutes of Health, a taskforce was created to develop consensus definitions of four negative signs in childhood: weakness, reduced selective motor control, ataxia, and deficits of praxis. The latter was divided into apraxia and developmental dyspraxia based upon whether or not skills had previously been acquired. The definitions are restated here:

Weakness is defined as the inability to generate normal voluntary force in a muscle or normal voluntary torque about a joint.
Reduced selective motor control is defined as the impaired ability to isolate the activation of muscles in a selected pattern in response to demands of a voluntary posture or movement. (for example moving one finger at a time)
Ataxia is defined as an inability to generate a normal or expected voluntary movement trajectory that cannot be attributed to weakness or involuntary muscle activity about the affected joints. (being able to accurately touch a target without extra movements or tremor)
Apraxia is defined as impairment in the ability to accomplish previously-learned and performed complex motor actions, although each subcomponent of the action is possible for the child to perform.
Developmental dyspraxia is defined as a failure to have ever acquired the ability to perform age-appropriate complex motor actions that is not explained by the presence of inadequate demonstration or practice, ataxia, reduced selective motor control, weakness, or involuntary motor activity (and as in the case of apraxia, each performance of each subcomponent of the task is possible)

Following the meeting in 2005, the taskforce recognized the need to develop assessment tools that can be used to categorize and quantify negative symptoms. These clinical experts recognized that the very process of coming together to exchange ideas and develop agreement about terminology had great potential value for improving research and care.
The goal was to provide a set of measures that can be used clinically to diagnose disorders, quantify impairments, and assess treatment effects of interventions.
By providing measurement tools, clinicians and researchers can use these tools to establish inclusion in clinical research trials and measure outcome.
The dialog and interactions involved in the validation of definitions and identification of potential areas for modification of definitions has great value. For example using widely accepted and validated measures and definitions will lead e better specificity of diagnosis and closer relation to the underlying pathophysiology.
Development of appropriate assessment tools is a prerequisite to the performance of clinical trials to evaluate potential treatments; the taskforce concluded that a high priority should be placed upon the rapid development of valid, reliable, and sensitive tools.
Therefore, the NIH funded a second meeting in Stanford, California in March 2006 to have different members of the task force review the current state of the art in clinical or research assessment of “negative signs” in childhood.

Methods

Four working groups, one for each of the negative signs (with apraxia and dyspraxia being considered by a single group) reported progress they had made toward assessing the sensitivity, specificity and relavency of currently available clinical measures. The working group leaders are:

    Weakness: M Delgado
    Reduced Selective Motor Control: D Gaebler-Spira
    Ataxia: A Bastian
    Apraxia/Dyspraxia: M Hallett and M Wiznitzer

Each working group met at least once by telephone or in person prior to the March 2006 meeting in order to review current technologies. The working group conclusions were presented at the meeting. Discussion within and between groups at the meeting led to specific recommendations for further research and evaluation of particular tools.
Groups were instructed to evaluate each rating scale or instrumented measurement technique according to the following criteria (Modified from Herndon, 1996):
1. It should measure what it purports to measure (Validity).
2. It should successfully identify those who do not have the impairment (Specificity).
3. It should successfully identify those who do have the impairment (Sensitivity).
4. It should be responsive to change of the impairment yet relatively insensitive to day-to-day symptom fluctuation.
5. It should have low test-retest and inter-observer variability (Reliability).
6. It should be rapid and easy to use with little special training (Efficiency).
Groups were further instructed to separate what should be measured from conclusions about how it should be measured. In particular, it was noted that how to measure may depend upon whether the measurement needs to be performed in the clinic (where speed and portability are essential) or in a laboratory setting (where accuracy and reliability are essential).

Weakness

The weakness working group evaluated Manual Muscle Testing (MMT), Hand-held torque measurement devices (HHD), Isokinetic torque measurement devices, and surface electromyography (sEMG). They also evaluated newer methods including the Myotonometer and muscle imaging using either ultrasound or MRI. The group concluded that strength should be measured as torque during isometric maximal voluntary contraction. For this purpose, manual muscle testing is insensitive and unreliable, but due to its simplicity and common use it may have value as a clinical screening tool that could be used to refer patients for more in-depth measurement. Hand-held torque measurement devices such as strain gauges or pressure sensors show promise, but careful attention will need to be paid to standardization of use, the posture in which strength is tested, ensuring maximal effort, and stabilization of proximal joints. Isokinetic measurements using a seated system such as the Biodex may be helpful, but such devices are often not suited to children, and they would be expected to be useful only in a laboratory setting. Measurement of surface EMG, muscle hardness (myotonometer), and muscle imaging studies may provide useful related information but these do not appear to provide a direct measure of strength. In particular, the group noted that the force output of a muscle may not correlate well with surface EMG between different subjects, and therefore while surface EMG may help to differentiate between different causes of weakness, it does not provide a good measure of weakness.
The use of multi-axis force/torque sensors was identified as an important need in order to determine both the strength in “off-axis” directions as well as to determine the contribution of off-axis components of force to decreased effective torque relative to a particular task. Norms for endurance and the effects of fatigue need to be established for children of different ages. It may be helpful to establish the relationship between strength and perception of effort. Methods for motivating younger children to produce adequate effort need to be developed and standardized. The relation between weakness and limitations in function needs to be investigated in order to determine goals for intervention. This is particularly important in the upper limb, where considerable weakness may be tolerable without adverse effects on performance of common skills. Other important issues include the ability to measure strength during movement, and the ability to measure trunk and neck strength. A helpful tool may be the development of muscle models that are specific to children with motor deficits such as Cerebral Palsy, in order to be able to predict the contribution of peripheral (muscle) vs. central (neurological) factors to measured weakness.
The recommendation at this time is that weakness should be quantified by the use of torque measurements about a joint during maximum voluntary isometric contraction. Further research needs to be done to standardize methods for torque measurement, establish standards for obtaining maximum voluntary contraction, and extend measurement methods to include trunk, neck, and possibly other muscle groups. The most promising current technology for both clinical and research investigation is the use of hand-held force measurement devices, and investigation of these devices should be given high priority. New technology to permit isokinetic measurement in children should be developed. Manual muscle testing will continue to have a role as a clinical screening tool but should not be used as a quantitative assessment. It is essential to exclude possible confounding factors such as practice effects, spasticity, reduced selective motor control, contractures, task-dependent weakness, bradykinesia, and other motor or cognitive signs that could lead to inaccurate measurement of strength.
The conclusions for assessment of weakness can be summarized as follows:
Pre-requisite for assessment: ability to follow instructions, and at least some active range of movement
Identification: reduced voluntary force (in response to command)
Quantification: inability to move against resistance with proximal joints stabilized
Distinction from other signs: should be assessed in and out of synergy patterns, with balance and stability concerns eliminated, and in a single joint at a time.

Reduced Selective Motor Control

Reduced selective motor control is related to the concept of obligate synergistic activation of muscles. Such synergies can occur in the arm, fingers, leg, and possibly trunk and neck, although these latter are not quantified by any current scales. It was noted that particular synergies may respond to practice, so that with training changes could be seen. An expected consequence of reduced selective motor control would be inability to activate specific muscles in combination with other muscles, and therefore the possibility of “task-dependent weakness”. Therefore selective motor control is quantified by a limitation in the maximal torque or range of motion in a joint where the limitation depends upon the posture or torque at other joints. It may also be quantified by a limitation in maximal voluntary EMG in a muscle where the limitation depends upon EMG in other muscles. There may be specific patterns of synergistic activation that are similar across children. For example, extension or flexion synergies of the leg, or a combination of adduction at the shoulder with extension at the elbow.
Instrumented measurements that show promise include the use of gait analysis with particular emphasis on the measurement of correlations between multiple joints, referred to as “angle-angle coupling”. Surface Electromyography is often reported by gait analysis laboratories and this can be particularly useful to evaluate the presence of extensor synergies in the leg. For example, involuntary activation of the gastrocnemius muscle during attempted knee extension with simultaneous ankle dorsiflexion suggests an extensor synergy coupling quadriceps activity with gastrocnemius activity. Other extensor or flexion synergies can be tested in this way. Portable or handheld surface EMG devices may allow non-quantitative screening for such synergies the clinic. The SMC is a promising clinical rating scale that directly tests the ability to isolate individual joints.
Laboratory measures could include multiple degree of freedom force and torque measurements in order to demonstrate obligate coupling between multiple joints, and changes in strength or active range of motion as a function of posture or the angle of proximal joints. Appropriate standardization of techniques, including stabilization of some proximal joints is essential. It may also be important to measure selective motor control during motion. For example, Jules DeWald presented data showing that reduced elbow extension during shoulder abduction may be well demonstrated during movement. The extent of the reduction may depend upon particular constraints on the movement, so constraining motion to particular planes, joints, or ranges of torque may be necessary for assessment. Therefore robotic systems may be needed in order to test limitations in posture or range of motion that occur during voluntary movement.
The recommendation at this time is that further study is needed for all measures. Promising areas include the use of elements of the QUEST or Fugl-Meyer as tests of the ability to achieve specific arm postures, and elements of the SMC scale as a test of lower extremity isolated joint movements. Promising laboratory measures include the use of kinematic analysis to measure angle-angle coupling, the use of multi-axis force transducers to measure changes in strength as a function of posture or torque at other joints, the use of robot manipulators to examine the effects of proximal joint posture on distal joint active range of motion during movement, and EMG evidence of obligate coupling between muscle activation. Testing the use of portable surface EMG devices in clinic will be facilitated once this method has been validated in a laboratory setting.
The conclusions for assessment of reduced selective motor control can be summarized as follows:
Pre-requisite for assessment: ability to follow instructions, and sufficient strength to move the tested limb when it is supported against gravity
Identification: inability to isolate a single muscle or joint during specific tasks
Quantification: clinical measures of the ability to achieve multi-joint postures, multi-axis force transducer measurement of change in force with changing posture of other joints, range of motion at distal joints with proximal joints constrained, and obligate coupling between multi-muscle surface EMG
Distinction from other signs: adequate strength when supported against gravity, worsening when a proximal joint is stabilized out of the synergy pattern

Ataxia

Several validated clinical rating scales are available for quantification of ataxia and ataxic disorders, including the International Cooperative Ataxia Rating Scale (ICARS), the SARA, and the Friedreich’s Ataxia Rating Scale (FARA). Although these rating scales have been developed either for adult disorders or for specific childhood syndromes, they contain common elements that could form the basis for an ataxia rating scale that is applicable to childhood disorders. In particular, several scales include observer assessments of accuracy and intention tremor during finger-to-nose, finger-following, and rapid alternating movements.
A small number of laboratories have investigated the kinematics of arm movements in subjects with ataxia. There is additional data from gait analysis, standing balance, and posturography that could also form the basis for quantitative measures. At this time, such measures have not yet been validated on large numbers of children.
The recommendation at this time is that a pediatric clinical rating scale based upon elements of existing ataxia scales should be developed. Promising techniques for laboratory quantification of ataxia include measurement of the effect of speed on endpoint accuracy and comparison between accuracy in single-joint and multi-joint movements. For assessment of gait ataxia, gait analysis including base of support, joint decomposition, obstacle avoidance, and deviation from a straight line should be evaluated. The evaluation of standing balance and toe-to-target tasks may differentiate between subgroups of patients with gait ataxia due to proprioceptive deficits, balance disorders, or dysmetria.
The conclusions for assessment of ataxia can be summarized as follows:
Pre-requisite for assessment: ability to follow instructions, and ability to reach or kick against gravity, or walk unsupported
Identification: hypermetria that is worse with fast movement or multi-joint movement, and/or irregularity of rate, rhythm, or force on repetitive movement, and/or wide based, stiff, and variable gait
Quantification: magnitude of dysmetria, accuracy as a function of speed and the number of unstabilized joints
Distinction from other signs: hypermetria, irregularity, greater impairment of multi-joint movements both within and outsisde of synergies, lack of crouch gait.

Apraxia and developmental dyspraxia

The distinction between apraxia and developmental dyspraxia is based upon the history of whether or not a particular complex motor action had previously been performed by the child. Therefore assessment tools for both disorders will be similar. Recent efforts to classify adult apraxia have led to at least 5 subcategories: (1) limb kinetic, (2) ideomotor, (3) ideational, (4) conceptual, and (5) dissociation. Adult clinical rating scales are under development, and the working group suggested that pediatric rating scales should remain consistent with the adult scales to the extent possible.
Elements of the Movement Disorder Society Apraxia Scale (MDSAS) adult test may be helpful. However, the MDSAS is still only in draft form and has not been validated. This test tentatively includes the following tasks: coin rotation, toothbrush pantomime, identification and use of a real toothbrush and toothpaste, identification and use of a key with the eyes closed, and imitation of the use of a key. The intent of these tasks is to separate the different adult forms of apraxia. Modification of the particular tasks may allow a similar test to be used in children, although it would remain difficult to apply to very young children.
The recommendation at this time is to develop two clinical rating scales: a short scale and a long scale. The short scale is intended as a screening test that can be used in the clinic, and would be required to have high sensitivity but relatively low specificity. The long scale is intended as a research tool and would be required to have both high sensitivity and high specificity, as well as a graded response to change in ability. Scale elements should include screening questions for current level of skills, as well as tasks that are both transitive (using an object) and intransitive (gestures), imitation of postures and tasks, and action sequences. Scoring should attempt to determine the type of error within each subtask in order to determine the subtype of dyspraxia. Possible child-appropriate transitive tasks include dressing, writing, drawing, gripping an object, opening a door, climbing on the examining table, paper folding, use of a hammer, pencil, key, or spoon. Possible intransitive tasks include wave, blow kiss, ok sign, and silence sign. It will be important to test both novel and previously-learned tasks, and to compare performance to age norms. The order of evaluation of types of dyspraxia may be important, since children with limb-kinetic apraxia may be unable to complete any other tasks. It will also be important to test different modalities of command, including verbal command and visual demonstration of tasks.
We recognize that oromotor and gait apraxia may be significant contributors to disability, but at this time we have not addressed measurement tools for these signs.
The conclusions for assessment of disorders of praxis can be summarized as follows:
Pre-requisite for assessment: ability to follow instructions, adequate strength to resist gravity, lack of obligate synergies that interfere with task to be tested, adequate coordination to perform the task.
dentification: spatial, timing, sequencing, or conceptual errors
Quantification: clinical rating scale and skill tests
Distinction from other signs: other signs must be excluded, and performance does not improve with slow speed of movement or stabilization of proximal joints.

Conclusion

The taskforce recognizes a significant need for further research to determine the most appropriate assessment tools and assess reliability, sensitivity, specificity, and validity of those tools. We recommend that separate tools be developed for use in the clinic as a rapid screening tool and for use in the laboratory as a quantitative diagnostic tool and outcome measure. We further recommend that efforts be directed toward particular methodologies, as summarized in the following table:

Assessment of a child with negative symptoms should proceed in a stepwise fashion in order to eliminate confounders:
    1. Strength
    2. Ability for control of independent joint motion
    3. Coordination including dysmetria, dysrhythmia, dyssynergia
    4. Limb-kinetic apraxia
    5. Other deficits of praxis
The goal of the working group over the upcoming year is to develop candidate rating scales and assessment tools, and to begin to obtain reliability, validity, sensitivity, and specificity data. It is expected that this will require testing each rating instrument on children with all four types of motor signs, and possibly on children with other motor signs including hypertonic or hyperkinetic signs. It is expected that a set of clinical measures will result that can be used to guide clinical treatment and can provide meaningful outcome measures for clinical trials to develop and test new therapies for children with motor disorders.
Commentary: The process of having this group interact and focus on appropriate measurement instruments has already led to important exchanges of ideas about best treatments (and the appalling lack of rigorous data) for best practices in CP. On a VERY positive note, however, the best and the brightest are now engaged in the research questions that could revolutionize the care of developmental disorders, particularly strategies for improving strength, selective motor activation, combination therapies to improved motor and functional performance (eg focal spasticity treatment coupled with CIT, functional neuromuscular stimulation, robotics, virtual reality, the list goes on..)