Our Experience with Dexmedetomidine in General Anesthesia for Cesarean Section in a Rare Case of Complicated Hereditary Spastic Paraplegia Patient

Hereditary spastic paraplegia (HSP) is a rare inherited neurodegenerative disorder causing progressive limb weakness and spasticity. Providing anesthesia to HSP patients poses a unique challenge. Ours is the first case report of providing anesthesia to HSP patient in SAARC countries. A 30-year-old parturient female with 37 weeks of gestational age presented to us for emergency cesarean section. She was a known HSP patient with ATP13A2 gene mutation and symptoms of weakness, slurred speech, coughing on swallowing, bradykinesia, and bladder incontinence. Nondepolarizing muscle relaxants carry a risk of exaggerated response, and opioids accentuate the relaxant response, leading to delay in recovery from anesthesia. To avoid opioids and reduce the use of neuromuscular blockers, we have used dexmedetomidine loading dose preintubation and continuous infusion for maintenance with transverse abdominis plane block at the end for postoperative pain. The patient was extubated on table without any adverse events and opioid usage in the perioperative period.