Dear Editor, Hirayama disease is a sporadic, self-limiting juvenile muscular atrophy which affects the muscles of the arm of people of the Asian population in the third decade of life.[1] In the past 35 years, only 279 cases have been reported in India.[2] A 40-year-old male presented with complaints of weakness and pain in the right upper limb with grade 2/5 power and wasting of thenar, hypothenar, interossei muscles, wrist flexors, and extensors with the sparing of the brachioradialis muscle. It is called oblique amyotrophy.[3] The Babinski sign was positive. There was no history of involuntary movements, trauma, exaggeration of symptoms in winter, allergy to peanuts, and sulpha-containing drugs. There was no cranial nerve, sensory, bulbar, bladder, or bowel involvement. The Magnetic Resonance Imaging (MRI) [Figure 1] showed forward displacement of the posterior subdural sac on the flexion of the neck with prominent epidural space. Electromyographic nerve conduction velocity (EMG-NCV) findings showed partial denervation in the upper limb muscles. The thyroid function tests, creatinine kinase, and serum potassium levels were normal.Figure 1: MRI Hirayama diseaseAnticholinergic intravenous glycopyrrolate 0.2 mg was given as an anti-sialagogue. Following an intravenous propofol 1.5 mg/kg induction, we preferred rocuronium bromide 0.9 mg/kg for the neuromuscular blockade, for its rapid onset of action. Injection atracurium was avoided as it has a propensity to raise the ImmunoglobulinE (IGE) levels which precipitate atopic myelitis in the Hirayama disease.[4] Also, in patients with denervation because of spinal cord lesions, an increased number of peri-junctional nicotinic acetylcholine receptors on skeletal muscle can cause hyperkalemia after suxamethonium chloride. Laryngoscopy was performed with manual inline stabilization of the spine (MILS) and a LaerdalR Philadelphia hard cervical collar on, using a McCoy laryngoscope blade and the patient was intubated with a size 8.0 cuffed flexo-metallic tube. The immobilization aims to protect patients with an unstable cervical spine from secondary cervical spine injury induced by accidental movement of the spine.[5] Proning was done by proper precautions, adequate eye care, and padding of pressure points were done. Auto-compression stockings were applied on the lower limbs for deep venous thrombosis (DVT) prophylaxis. Sevoflurane was given to maintain Bispectral Index (BIS)-guided adequate depth of anesthesia. These patients may have a less physiological reserve to tolerate hypotension-induced ischemia and require higher arterial pressures to maintain spinal autoregulation.[5] Injection phenylephrine infusion 1 mg/cc was kept ready. Train Of Four (TOF) monitoring was done intraoperatively and readings were taken before reversal and extubation. Hirayama disease is a sporadic focal degenerative motor neuron disease affecting the cervical cord when the neck is in flexion. Repeated compression changes make the anterior horn cells prone to arterial or venous ischemic injury which causes increased intramedullary pressure and micro-circulatory disturbance.[3] This is in line with the Poisson effect which dictates that if a column of fixed volume is compressed, its cross-sectional area will increase. Conversely, if it is stretched, the cross-sectional area will decrease, which is based on the Chinese finger trap model [Figure 2]. The basic principle is to keep the neck in extension.Figure 2: The Chinese finger trap modelRational airway management, appropriate choice of muscle relaxants in motor neuron diseases, and the use of intraoperative neuromuscular monitoring helped in the optimal, safe, and effective anesthetic management. Declaration of patient consent The authors certify that they have obtained all appropriate patient consent forms. In the form the patient has given hid consent for his images and other clinical information to be reported in the journal. The patients should understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest.