Anesthetic Management for Stereotactic Neurosurgical Treatment of Patients with Pantothenate Kinase – Associated Neurodegeneration (PKAN) – An Experience of Two Cases with Dexmedetomidine - Abstract
Pantothenate Kinase – associated neurodegeneration (PKAN) is a rare autosomal recessive disorder. PKAN presents with severe incapacitating dystonias, rigidity and cognitive impairment usually in early childhood. The initial treatment of patients suffering from PKAN constitutes medical line of management, but for those patients who are refractory to medical management surgical treatment is an option. Perioperative anaesthetic management is a challenge in view of generalized dystonias, restricted airway access due to head frame, need for an awake and co-operative patient, and to provide ideal conditions for intraoperative microelectrode recordings (MERs) under anesthesia. All these factors demand an appropriate anesthetic technique. It further becomes challenging if the surgical procedure is performed on children, since they require general anaesthesia which in turn can interfere with the interpretation of MERs and clinical assessment. We report two cases of PKAN who underwent surgical treatment using dexmedetomidine (alpha agonist) as the principal anesthetic agent.