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Final Program


Final Program

249 - Intrathecal Baclofen Pump as Unique Treatment of Paroxysmal Sympathetic Hyperactivity (Neurostorming) in a Pediatric Cerebral Palsy Patient

Friday, February 28, 2025
5:00 PM - 6:30 PM MST

    Presenting Author(s)

  • MT

    Michelle C. Tan, BA

    Medical Student
    McGovern Medical School
    Bellaire, Texas, United States

    • Co-Author(s)

  • AC

    Aaron Charnay, MD

    Assistant Professor
    Baylor College of Medicine
    Houston, Texas, United States

    • Corresponding Author(s)

  • Unoma Akamagwuana, MD

    Assistant Professor
    Baylor College of Medicine
    Houston, Texas, United States

Case Diagnosis: 13-year-old male has mixed spastic-dystonic cerebral palsy and chronic respiratory failure complicated by PSH. Due to failure of more traditional medications for PSH, an intrathecal baclofen (ITB) pump was placed with the catheter tip located atypically in foramen magnum. In addition to the pump, the patient’s spasticity, dystonia, and PSH are managed with bromocriptine and clonidine on a scheduled basis with diazepam as his rescue medication.  The dose gradually increased to 1642.2 mcg/day before PSH was controlled.

Case Description: Current treatment involves the ITB flex dosing with a maintenance dose of 1567.8 mcg/day. This dose was decreased from admission dose to improve respiratory drive. The patient’s PSH improved with larger doses of baclofen. Decreases in dosing could lead to increased PSH. Labetalol is used to control his baseline hypertension. Prevention of hypoxia is managed with albuterol, acetylcysteine, and positive airway support. Due to his unique catheter placement and high dose of baclofen, there is an increased risk of respiratory depression. ITB allows the flexibility to balance his tone management and respiratory status.

Discussions:

ITB for PSH is unique. ITB can be useful in managing PSH episodes. Baclofen is a GABA-B agonist that decreases Ca2+ influx at presynaptic nerve terminal helping inhibit excess catecholamines produced during PSH and reducing the postsynaptic motor neuron action potential contributing to spasticity.

Conclusions:

With the common use for ITB being spasticity, this case explores its potentially beneficial findings for PSH. A study by Pucks-Faes, et al. showed that ITB in TBI PSH patients helped improve hypertonia, tachycardia, hypertension and decreased episodes of motor neuron hyperactivity. Information on ITB in PSH pediatric cerebral palsy patients or non-TBI patients is lacking. This report gives an example of the beneficial effects of ITB which could be included when developing guidelines for treatment of PSH patients.