UMEM Educational Pearls

Intrathecal baclofen pumps are increasingly used to manage spasticity in patients with conditions such as cerebral palsy, spinal cord injury, multiple sclerosis, traumatic brain injury, and other dystonias. The most common causes of baclofen pump dysfunction include pump-related issues (e.g., programming errors, battery failure), catheter problems (e.g., extra-thecal dislodgement, kinking, leaks), and medication depletion (e.g., overdue or insufficient refills). Symptoms of dysfunction can be nonspecific, ranging from mild (spasticity, dysphoria, dysesthesias) to severe (e.g., rigidity, rhabdomyolysis, seizures, fever, autonomic dysfunction, cardiomyopathy).

Once dysfunction is recognized, management involves stabilizing vital functions (ABCs, temperature management, fluids), administering multimodal antispasmodics (enteral or parenteral baclofen, benzodiazepines, dexmedetomidine, tizanidine), and performing pump interrogation, often in collaboration with neurology or PM&R specialists. Restoration of intrathecal flow is the preferred and definitive therapy; however, patients with severe withdrawal may require aggressive temporizing measures including intubation. Some authors describe intrathecal baclofen administration via lumbar puncture as a rescue measure for severe cases with limited access to definitive care. Imaging with plain radiographs, fluoroscopy, or CT may be indicated in select cases where there is concern for catheter displacement or kinking, and some patients may require surgical revision.

Takeaway: Consider baclofen withdrawal in patients on chronic baclofen therapy who present with nonspecific symptoms that may mimic conditions such as alcohol withdrawal, delirium, sympathomimetic toxicity, neuroleptic malignant syndrome, serotonin syndrome, thyrotoxicosis, rhabdomyolysis, sepsis, or status epilepticus. In cases of intrathecal pump dysfunction, the definitive treatment is restoration of baclofen flow, so involve consultants early for pump interrogation while temporizing with supportive measures.

References

1. Boster, A., Nicholas, J., Bartoszek, M. P., O'Connell, C., & Oluigbo, C. (2014). Managing loss of intrathecal baclofen efficacy: Review of the literature and proposed troubleshooting algorithm. Neurology. Clinical Practice, 4(2), 123–130. https://doi.org/10.1212/cpj.0000000000000000
2. Romito, J. W., Turner, E. R., Rosener, J. A., Coldiron, L., Udipi, A., Nohrn, L., Tausiani, J., & Romito, B. T. (2021). Baclofen therapeutics, toxicity, and withdrawal: A narrative review. SAGE Open Medicine, 9, 20503121211022197. https://doi.org/10.1177/20503121211022197