1127 - Management of Dysautonomia and Spasticity using Intrathecal Therapy: A Pediatric Case Study

Case Diagnosis: Intrathecal (IT) baclofen therapy is commonly used in the treatment of severe spasticity associated with upper motor neuron syndromes in pediatric patients. Treatment of concurrent disorders using IT therapy has been less well described. We present a case of severe spasticity and dysautonomia in a 13-year-old female with Stiff Person Syndrome successfully managed with IT clonidine and baclofen.

Case Description: 13-year-old female with a presented with 4-month history of intermittent tachycardia, fevers, diaphoresis, progressive weakness, fatigue, muscle spasms and joint pain. Diagnostic work up revealed positive GAD 65+ and ultimately a Stiff Person Syndrome diagnosis. An intrathecal baclofen pump was placed to manage severe spasticity.



After the spasticity was adequately managed the patient continued to suffer from severe symptoms related to dysautonomia. These included tachycardia, postural orthostasis, hyperhidrosis, pupillary dysfunction, hyperthermia, early satiety, and auditory hypersensitivity. Despite maximizing lifestyle modifications, compression, dietary changes, and pharmacologic management, the symptoms significantly interfered with daily function.



Intrathecal medication was transitioned to combination clonidine and baclofen in attempt to improve symptoms related to dysautonomia. Titration of intrathecal clonidine allowed for discontinuation of all oral medications and resulted in improvement of many dysautonomia symptoms.

Discussions: This case illustrates the benefits of intrathecal therapy for treatment of concurrent disorders such as dysautonomia seen in patients who also suffer from severe spasticity. In this case IT clonidine used in combination with baclofen resulted in improvement spasticity and dysautonomia. As expected, delivery of medication to the intrathecal space required significantly smaller doses of medication with larger clinical benefit.

Conclusions: Consideration should be made to expand the use of IT therapy beyond severe spasticity in pediatric patients with concurrent disorders.