Rashi Sarna1, Anie Gupta1, Geetika Arora1. 1. Department of Anaesthesia and Intensive Care, Postgraduate Institute of Medical Education and Research, Madhya Marg, Sector 12, Chandigarh, India.
Sir,Amyotrophic lateral sclerosis (ALS) is a rare fatal neurodegenerative disease characterized by rapidly progressive muscular paralysis.[1] We report anaesthetic management of a 48-year-old male, weighing 50 kg presented with a painful abdomen and fever for 3 days. Appendicular abscess suspected by ultrasonography was confirmed by a computed tomography (CT) scan. He was planned for exploratory laparotomy under general anaesthesia. He was a diagnosed case of ALS for five years when he had developed a slow onset, progressive, asymmetric weakness of upper limbs and lower limbs with associated difficulty in walking, had a progressive course and developed dysphagia and dysarthria and was on oral Riluzole100 mg/day for last 3 years. The patient was hemodynamically stable and routine laboratory investigations were within normal limits except for raised white blood cell (WBC) count. Arterial blood gas (ABG) revealed PCO2 of 43.1 mmHg and oxygen saturation of 92% on room air. Pulmonary function tests could not be performed in view of emergency surgery. The patient was induced with 80 mcg fentanyl and 2 mg/kg of propofol. 10 mg of rocuronium was administered, prior to endotracheal intubation. Prior to the start of surgery, the patient was given a bolus of 1.5 mg/kg lignocaine followed by an infusion of 0.5 mg/kg/hr to decrease post-op pain. Anesthesia was maintained with continuous infusion of propofol at 0.05-0.1 mg/kg/min. No additional top-up of muscle relaxant was given. Surgery was completed without any significant sequelae, and total anesthesia time was 150 min. Glycopyrrolate (0.2 mg) and neostigmine (2.5 mg) were used to reverse the effects of muscle relaxants. On meeting the extubation criteria, the patient was extubated and was observed in the post-anesthesia care unit (PACU) for about 1 hour until he reached the discharge criteria. ABG conducted in PACU showed PCO2 47.5 mmHg and oxygen saturation of 91% on room air. The patient was comfortable, pain-free (visual analogue score (VAS) 1-2) and did not demand any analgesic in the postoperative period for the first 24 hours. ALS causes the degeneration of upper and lower motor neurons.[2] leading to muscle atrophy, muscle spasticity, and muscle weakness.[1] In the majority of ALSpatients, the respiratory failure and death are mainly caused due to atrophy and weakness of the respiratory muscles.[2] limiting the survival to 2-5 years after the onset of the disease.[3] Aspiration or ventilatory depression can be caused by general anaesthesia due to abnormal response of muscle relaxants whereas spinal anesthesia can exacerbate the preexisting neurological diseases.[4] Thus, anaesthesia needs modification, short-acting agents must be used to prevent an unpredictable response to muscle relaxants and to ensure quick reversal in a patient of ALS.[5] The depolarizing neuromuscular blocking drugs may increase rhabdomyolysis and massive hyperkalemia in such patients.[6] Therefore, the use of succinylcholine was avoided in this patient admitted for emergency laparotomy. The non-depolarizing neuromuscular blocking drugs and reversal agents both should be used judicially, under a peripheral nerve stimulation monitor due to increased susceptibility of patients with motor nerve diseases.[7] The normal intubating dose of rocuronium (a non-depolarizing muscle relaxant) is 1.0 mg/kg, therefore, a low dose (only 10 mg) was used in this case. No additional top-up was given and the patient was maintained on propofol infusion. Epidural was avoided as it can exacerbate the disease condition. The presence of muscle rigidity can decrease the post-operative respiratory function hence the opioids should be used with caution. Therefore, an infusion of lignocaine was used peri-operatively to provide postoperative analgesia and to reduce the opioid requirement. The patient tolerated lignocaine infusion without any adverse effects. Paracetamol was given intravenously four times a day postoperatively. Though PCA with fentanyl was attached for the relief of postoperative pain. However, the need for any bolus was not required for the first 24 hours. The patient was discharged in a stable condition at postoperative day 7. Thus, in patients who require general anesthesia, one can use lignocaine infusion that has little effect on the progression of the disease as well as significantly decreases the requirement of opioids for pain relief.
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