Friedreich's Ataxia (FA) is a rare autosomal recessive disease. FA is clinically characterized by progressive ataxia, especially loss of reflex and power in the lower extremities, spasticity and loss of proprioceptive sensory. There is an increased sensitivity to non-depolarizing muscle relaxants in neurodegenerative diseases. Care must be taken of anesthesia management for the use of volatile anesthetics and muscle relaxants, and precautions should be taken for difficult airway management which is due to secondary to kyphoscoliosis and comorbidities. In this case, anesthesia management of emergency operation for septal hematoma by TIVA without using of muscle relaxants and minimalized hemodynamic response depending on the intubation by videolaryngoscope were presented.
Key words: Friedreich's Ataxia, anesthesia management, neuromuscular blockade, videolaryngoscope
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