1341 - Six-Year Follow-Up of Intrathecal Baclofen Treatment in a Mixed Spastic Dystonia Cerebral Palsy: Challenges and Strategies

Case Diagnosis:



Mixed Spastic Dystonic Cerebral Palsy (GMFCS V)







Case Description:
This case follows a 24-year-old woman with Mixed Spastic Dystonic Cerebral Palsy (GMFCS V) over six years. She received intrathecal baclofen (ITB) treatment at 18 years old to manage dystonia and spasticity, showing positive initial outcomes in positioning, quality of life, and caregiver burden. In the fourth year, her cervical and upper limb issues worsened, correlated with catheter tip movement seen in imaging. Scoliosis progressed, and the catheter migrated from the C6 to C7 level. The patient's deteriorating dystonia and spasticity align with catheter positioning concerns. Thus, the multidisciplinary team recommended several interventions including raising treatment dosage by 10%, using botulinum toxin for cervical dystonia, and reanchoring the catheter at a higher cervical level during pump replacement. Constipation was reported post-increment of ITB treatment dosage which was improved with laxatives.

Discussions:
While limited evidence suggests that ITB treatment can accelerate scoliosis progression possibly due to hypotonia worsening instability, consistent scoliosis surveillance remains important. The manifestation of worsening cervical dystonia and upper limb spasticity over time in the patient aligns with observations that these issues can be related to the migration of the ITB catheter tip and require re-anchoring of the catheter to a higher cervical level. ITB for spasticity management can lead to reduced gastrointestinal peristalsis causing constipation, however administering laxative drugs can improve this effect, enabling continued ITB treatment.

Conclusions:


Despite challenges, intrathecal baclofen is essential for managing severe dystonia and spasticity, requiring a holistic multidisciplinary approach, regular follow-ups, scoliosis monitoring, and addressing potential adverse events.