Anesthetic challenges in a patient with Hirayama disease with quadriparesis and autonomic dysfunction undergoing cervical spine surgery

Abstract

Hirayama disease is a rare neurological disorder, characterized by muscular atrophy of the distal upper extremities. The occurrence of spastic quadriparesis and autonomic dysfunction is rarely reported and has important perioperative considerations during cervical spine surgery for the treatment of this disorder. The role of the anesthesiologist is vital in the thorough assessment of the patient for the involvement of the pyramidal tract, autonomic dysfunction, gastroparesis, hyperreactive airway disease, and documentation of neurological deficits. Intraoperative concerns include safe manipulation of the airway during mask ventilation and the use of a flexible fibreoptic bronchoscope during endotracheal intubation to prevent neck flexion. It is also essential to avoid drugs, leading to histamine release. The use of multimodal monitoring including bispectral index and neuromuscular monitoring is crucial to prevent delayed recovery. Anticipation and management of exaggerated hypotension in response to anesthetic induction agents and prone position is the key to a successful outcome in patients with autonomic dysfunction.

Keywords: Autonomic dysfunction; Hirayama disease; Monomelic amyotrophy; Spastic quadriparesis.