A Child with Medical Complexity and Worsening Irritability

Symptoms of dysautonomia are often seen in children with medical complexity, particularly those who have suffered a significant neurologic insult. Symptoms include hypothermia, cool extremities, ileus, or irregular respiratory drive due to abnormal regulation by areas of the hypothalamus, medulla, and pons that control these autonomic functions. Baclofen is a medication used to regulate spasticity and may be given via an intrathecal pump. Symptoms of abrupt intrathecal baclofen (ITB) withdrawal may include fever and increased spasticity and can progress to hallucinations, delirium, seizures, and muscle rigidity and potentially to severe autonomic instability, rhabdomyolysis, cardiac arrest, and even death. The primary treatment for baclofen withdrawal is resumption of baclofen dosing. Management options include benzodiazepines and cyproheptadine (first-line), propofol, dantrolene, and dexmedetomidine.