Management of Spasticity and Dystonia

 

Spasticity is described as velocity-dependent resistance to stretch. That is, if you move a spastic limb slowly, you meet less resistance than if you move it rapidly. Spasticity is associated with increased deep tendon reflexes, co_contractions (i.e., if one muscle contracts, many muscles in the vicinity contract at the same time), abnormal postural control, and weakness. Spasticity acts like a brake, increasing energy consumption. It inhibits voluntary control, reduces normal stretch on muscles, inhibits growth of muscles and causes excess torque on bones. As a result, spasticity can lead to deformities of bones and joints (contractures). Functionally, it decreases mobility, impairs self care and hygiene, has adverse cosmetic effects, and can lead to pain.

Dystonia, on the other hand, is described as sustained muscle contractures that lead to abnormal posture and twisting. Dystonia can occur in any muscle, including the muscles of the face. Like spasticity, it can lead to deformities of the bones and joints as well as major functional impairments. Spasticity and dystonia often occur together, especially in children who have cerebral palsy.

The goal of treating spasticity and dystonia is to optimize functioning. The usual treatments include (1) physical and occupational therapy (PT and OT), (2) medications, (3) orthopaedic surgery, and (4) neurological surgery. PT and OT are used to maximize range of motion, improve skills of living, like transferring, and increase strength, e.g., with weight training or electrical stimulation.

Medications typically used for spasticity include diazepam (Valium), baclofen (Lioresal), tizanidine (Zanaflex), and dantrolene (Dantrium). These medications, which are the first line of medical therapy, are given orally, or in the gastrostomy feeding tube, and relax muscles in various ways. Unfortunately, dantrolene is generally not very effective. The other three medications relax muscles, but also produce sedation, so that by the time the child achieves a dose sufficient to relax the muscles, so much sedation has occurred that the child has lost function. Baclofen also can be administered intrathecally (in the area around the spinal cord). This is achieved with a pump that is inserted into the abdomen with a catheter running from the pump into the intrathecal sac. Prior to the insertion of a pump, a trial of Baclofen is given through a lumbar puncture to document its effects on the child.

Another medication commonly used to treat spasticity is Botulinum toxin. This is available in two forms, type A (Botox) and type B (Myobloc). Both are injected into muscles where they interfere with neuromuscular transmission producing weakness and decreasing spasticity. Their effects typically last 2-4 months and require repeated injections. The use of Botulinum toxin injections is combined with therapy (either PT or OT) to improve range of motion and strengthen opposing muscles.

Intrathecal baclofen also is quite effective for the treatment of dystonia, though typically much higher doses are required than for spasticity. Carbidopa_levodopa (Sinemet), a drug used to treat people with Parkinsonism, has been effective in the treatment of dystonia in some individuals.

Orthotics (braces) are used to control dynamic deformities, e.g., excessive ankle plantar flexion. Orthopaedic surgery is used to correct fixed deformities such as fixed joint contractures and abnormal twisting of bones (e.g., femoral antetorsion (anteversion)). Prior to surgery, formal analysis of the gait may be helpful to determine the nature of the surgery that would be most useful to the child.

The most common neurosurgical procedure performed to help spasticity is selective dorsal rhizotomy (SDR), in which selected sensory nerves are severed. SDR is most effective in the treatment of children who have pure spasticity and who are able to cooperate with intensive therapy. The ideal candidate is a child who is 4_7 years old, ambulatory with spastic diplegia (typically following premature birth), and motivated to walk. Following the procedure, six or more weeks of inpatient rehabilitation is performed to maximize the child’s functional abilities.

The evaluation and management of a child who has spasticity and/or dystonia is best performed by a team of individuals familiar with the developmental needs of children. These specialties typically include orthopaedics, PT and OT, developmental pediatrics or pediatric neurology or pediatric physical medicine and rehabilitation, nursing, and neurosurgery.

 

Gregory S. Liptak, MD, MPH


 

THE PEDIATRICIAN AND SENSORY INTEGRATION THERAPY

The Committee on Children with Disabilities, American Academy of Pediatrics, Chapter 1, District II participated in a conference call with representatives of the New York State Education Department on December 29, 1999 to discuss our concerns regarding provision of sensory integration services in schools for children with disabilities. The following represents a summary of the issues discussed at this meeting:

  1. A significant number of children receiving EI and CSE services receive occupational therapy. For these children, sensory integration therapy is widely utilized for children with varying needs. We are concerned about the potential lack of relationship between this medically prescribed service and the academic needs of the child for whom it is used
  2. Questions have been raised about the effectiveness of sensory integration therapy for certain children. The American Academy of Pediatrics has published guidelines for appropriateness of motor therapy services. Sensory integration therapy may fall into the category of questionably effective service. The recent New York State Health Department Clinical Practice Guidelines for young children with autism similarly noted the lack of a body of evidence to support the use of this modality.
  3. Occupational therapy services, including sensory integration, require a physician’s prescription. Physicians need to be made aware of the specific techniques that the occupational therapist will use, and what the intended goals of the therapy are.
  4. Occupational therapy services are in short supply in many areas, yet are frequently offered to children whose primary disability is not sensory related (for example children with language delays or ADHD). As this service is medically prescribed, the indication for use in terms of academic benefit needs to be clear to the prescriber.
  5. Concerns were raised regarding the OT evaluation process, itself, i.e. that OT testing may uncover needs which may not be academically problematic. Decisions regarding provision of services should be based on advancing educational goals or functional outcomes for the child, and should be indicated as such on the IEP.
  6. The use of standard criteria to evaluate sensory integration intervention was discussed. Physicians would feel more comfortable providing prescriptions if well designed studies proved the effectiveness of such intervention. Elements of such studies might include appropriately defined populations to compare, adequate statistical power, blinded assessment, appropriate controls, a placebo intervention, valid outcome measures, and comparable “doses” or intensity of interventions. We recognize that it is beyond the purview of the New York State Education Department to mandate that such studies be done. However, we must be aware of the strength of services we provide in these times of increasing financial demands.
  7. We believe that specific measurable and time-framed functional and educational goals should be set for each child reviewed by the CSE and that outcomes be monitored prior to the renewal of the prescription for motor therapies (as is similarly done in the health insurance sector).
  8. We would be most willing to provide input to the New York State Education Department as it examines the issue of related services. An Albany-based member of our Committee, Dr. Anthony Malone, might lend a useful voice to the New York State Education Department’s Critical Friends Group. Since many of the therapies offered are in the context of the educational program, but have physical outcomes and purportedly neurological bases, it is appropriate for a developmental pediatrician to participate in this discussion.

We appreciated the opportunity to conduct this dialogue and hope to be able to continue working together to better the services offered to children with disabilities in New York State.


 

I GUIDELINES ON AUTISM AND COMMUNICATION DISORDERS

In 1996, a multi-year effort was initiated by the New York State Department of Health to develop clinical practice guidelines to support the efforts of the statewide Early Intervention Program. Two initial clinical practice guidelines (on autism and communication disorders) have now been published. These guidelines are intended to provide parents, professionals, and others with recommendations based on the best scientific evidence available about "best practices" for assessment and intervention for young children (under three years) with autism or communication disorders..

Evidence-based clinical practice guidelines are intended to help professionals, parents ,and others learn what scientific evidence exists about the effectiveness of specific clinical methods. This information can be used as the basis for informed decisions. These guidelines represent the panels' concerted attempt to interpret the available scientific evidence in a systematic and unbiased fashion and to use this as the basis for developing guideline recommendations.

Report of the Recommendations: Autism/Pervasive Developmental Disorders
Report of the Recommendations: Communication Disorders Guidelines are available from the New York State Department of Health

Single copies are free to New York State residents.

The guidelines are also available as Quick Reference Guide and Guideline Complete Technical Report.

Order from:

Publications, New York State Department of Health
P.O. Box 2000
Albany, NY 12220

The Autism Panel Guidelines are also available on the internet at

http://www.health.state.ny.us/nysdoh/eip/menu.htm