What is the best treatment approach for a patient with athetosis (involuntary, writhing movements) of both upper and lower extremities?

Treatment of Athetosis Affecting Both Upper and Lower Extremities

Diazepam is indicated as a useful adjunct for the relief of skeletal muscle spasm due to athetosis, and should be combined with a structured functional task training program that emphasizes normal movement patterns while avoiding static immobilization. 1

Primary Pharmacological Management

• Diazepam (oral) is FDA-approved specifically for athetosis and serves as a useful adjunct for relief of skeletal muscle spasm caused by upper motor neuron disorders including athetosis 1
• The medication addresses the underlying muscle spasm and abnormal tone that characterizes athetoid movements 1
• Note that effectiveness beyond 4 months has not been systematically assessed, requiring periodic reassessment of continued benefit 1

Botulinum Toxin for Refractory Cases

• Botulinum toxin A injections should be considered for severe athetosis unresponsive to oral medications, particularly when pain and care difficulties are prominent 2
• This intervention has demonstrated effectiveness in decreasing pain and improving ease of care in children with severe athetosis from cerebral palsy 2
• Chemodenervation using botulinum toxin can increase range of motion and decrease pain for patients with focal symptomatically distressing spasticity in both upper and lower limbs 3

Structured Rehabilitation Program

Task-specific functional training forms the cornerstone of non-pharmacological management:

• Engage patients in repetitive practice using normal movement patterns to prevent learned non-use and promote functional recovery 4
• Implement functional tasks that promote normal movement, proper alignment, and even weight-bearing, such as:
  • Transfers and sit-to-stand exercises 5
  • Standing activities that challenge balance 5
  • Bilateral upper extremity tasks 6
• Grade activities progressively to increase the time affected limbs are used within functional activities 5
• Employ anxiety management and distraction techniques when undertaking tasks to improve motor control 5

Critical Splinting Considerations

Avoid static immobilization and prolonged splinting, as this worsens outcomes in athetoid movement disorders:

• Splinting may prevent restoration of normal movement and function 5
• Static immobilization increases accessory muscle use, promotes compensatory movement strategies, causes muscle deconditioning, leads to learned non-use, and increases pain 4, 6
• If orthotic support is necessary, use dynamic splinting that allows movement rather than rigid immobilization 6

Assistive Devices When Indicated

• Ankle-foot orthoses (AFOs) should be used for ankle instability or dorsiflexor weakness to improve walking disability, step/stride length, and balance 3, 5
• Ambulatory assistive devices should be prescribed to help with gait and balance impairments when needed 5
• Wheelchairs should be prescribed for non-ambulatory individuals or those with severely limited walking ability 5
• Device prescription must be specific to the patient's needs, environment, and preferences 5

Exercise and Physical Therapy Intensity

• Individually tailored aerobic training involving large muscle groups should be incorporated, with monitoring of heart rate and blood pressure 3
• Exercise is needed at least 3 times weekly for a minimum of 8 weeks, progressing to 20 minutes or more per session 3
• Progressive resistance training should be provided that is meaningful, engaging, repetitive, progressively adapted, task-specific, and goal-oriented 3

Adjunctive Interventions to Consider

• Functional Electrical Stimulation (FES) can be considered for patients with demonstrated impaired muscle contraction to provide short-term increases in motor strength and control 6
• Mental practice should be considered as an adjunct for upper and lower limb motor retraining 3
• Virtual reality and gaming devices can provide additional opportunities for engagement, feedback, repetition, and task-oriented training 3

Common Pitfalls to Avoid

• Do not use static splinting or serial casting, as this consistently worsens symptoms and can trigger complex regional pain syndrome 6
• Avoid postures that promote prolonged positioning of joints at end of range 5
• Do not completely immobilize, as strength loss is most dramatic during the first week of immobilization 4
• Address associated problems of pain and hypersensitivity which commonly accompany athetosis 5

Monitoring and Follow-Up

• Reassess function every 2-3 weeks to evaluate treatment effectiveness and adjust therapy progression 6
• Periodically reassess the usefulness of diazepam for the individual patient, particularly beyond 4 months of use 1
• Regular clinical evaluation should include assessment of limb symptoms, functional status, and overall quality of life 5