Case Presentation: A 54-year-old tracheostomy and gastric tube dependent, non-verbal male with chronic spastic quadriplegia, autonomic dysautonomia, and recent ICU admissions for MDR pneumonia presented to the emergency department from his skilled nursing facility with acute hypertension. His home medications were notable for hydralazine, isosorbide mononitrate, clonidine, midodrine, and an intrathecal baclofen pump. He was recently discharged from the medical intensive care unit five days prior for sepsis secondary to a proteus urinary tract infection. His admission vitals revealed a temperature of 100.6F, blood pressure of 186/132, a heart rate of 158 BPM, and a respiratory rate of 34 breaths per minute. His physical exam was notable for spasticity beyond his baseline, and he was admitted to the intermediate care unit for management of hypertensive urgency. Given his significant tachycardia, hyperthermia, and increased spasticity all out of proportion to his well-known baseline, there was clinical suspicion for baclofen withdrawal. On further review of his medical record, it was determined that he missed his outpatient appointment to refill his baclofen pump as it coincided with his recent MICU admission. Upon recognition, neurosurgery was consulted, and empiric treatment with oral baclofen, cyproheptadine, and diazepam was initiated. Neurosurgery successfully refilled his pump the evening of admission. Within hours of baclofen administration, he showed significant clinical improvement and resolution of his tachycardia. However, his stay in the intermediate care unit was complicated by multifactorial sepsis and care was escalated to the ICU for vasopressor support. Ultimately, after a prolonged ICU course complicated by bacteremia, VAP, ischemic colitis, the patient’s family elected for comfort measures and the patient died in the hospital.
Discussion: This case encapsulates the life-threatening potential of baclofen withdrawal syndrome (BWS), especially in patients with intrathecal delivery devices. As a GABA-B receptor agonist, baclofen contributes to inhibition of excitatory neurotransmission. Abrupt withdrawal of this continuous agonism in an intrathecal system can lead to profound autonomic instability with a sympathetic storm of hypertension, tachycardia, hyperthermia, spasticity, seizure, and altered mentation. This case characterizes several key features of BWS in the context of patient and logistical issues: 1) baclofen has a narrow therapeutic window, especially in the context of an implantable device, 2) medication and device reconciliation are vital at each transition of care, 3) Non-verbal patients can benefit from additional attention to records, and 4) Redundant systems of communication and recordkeeping are essential especially in vulnerable patient populations.
Conclusions: Although hypertensive urgency is a relatively common presentation, BWS is a cannot-miss, medical emergency. BWS is unresponsive to classical antihypertensive management and merits timely recognition and treatment. Clinicians are urged to maintain an index of suspicion for BWS for any patient on chronic baclofen, since the diagnostic challenge is obscured by baseline autonomic dysfunction in many of these patients. Further, it is critical to establish systems of care that allow for redundancy in the setting of patient-limiting factors (non-verbal, disability), and clinician factors (transitions of care) to mitigate a BWS adverse event.