Perioperative Management of a Patient With Giant Axonal Neuropathy

Hiromi Kako, Arlyne Thung, Allan Beebe, Jan Klamar, Joseph D. Tobias


Giant axonal neuropathy (GAN) is a rare, autosomal recessive, progressive neurodegenerative disorder affecting both the central and peripheral nervous systems. The aberrant accumulation of cytoskeletal-associated proteins causes neurodegeneration resulting in both motor and sensory dysfunction. Given the invariable and progressive involvement of the neuromuscular system, anesthetic care is frequently needed to correct the orthopedic sequelae of the disorder. We present a 12-year-old girl with GAN who required anesthetic care for posterior spinal fusion to treat scoliosis. Previous reports of anesthetic care for patients with GAN are reviewed, and the perioperative considerations of these patients are discussed.

J Med Cases. 2015;6(11):512-516


Giant axonal neuropathy; Spinal fusion; Neuromuscular scoliosis; Neurodegenerative disorder

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