Abstract Amyotrophic lateral sclerosis (ALS) is a rare motor neuron disease with an incidence of 5.2 per 100,000 people. Diagnosis is made by presence of progressively worsening symptoms and demonstration of upper motor neuron (UMN) and lower motor neuron lesions (LMN) in three separate regions. The patient in this case was diagnosed with ALS during outpatient evaluation by a local neurology clinic one month prior to admission. The patient was found to have fasciculations of the tongue, reduced LMN amplitudes of the left leg noted on electromyography (EMG), and absent LMN amplitudes of the right leg on EMG. MRI of the brain showed UMN lesions consistent with ALS.The patient is a 63-year-old male with no known prior medical history. Over the past 7 months he has been having worsening constipation and oropharyngeal dysphagia characterized by coughing and choking when he eats solids or liquids. The patient was admitted for failure to thrive and placement of a percutaneous endoscopic gastrostomy (PEG) tube. The patient required supplemental oxygen via nasal cannula after the procedure, and he was started on empiric treatment for pneumonia. The patient was eventually intubated for hypercapnic respiratory failure, acute respiratory acidosis was noted on ABG (pH 7.2, PCO2 125 mmHg). ICU staff noted that the patient was having increasing abdominal distention. CT abdomen and pelvis showed PEG malposition and hydro-pneumoperitoneum from tube feeding. General surgery was consulted early and laparotomy with colectomy was offered as the patient also had persistent ileus. However, the patient declined the surgery, so interventional radiology placed an intra-abdominal drain. EGD was performed to retract the PEG tube and close the gastric perforation. During the 64-day hospital course, the patient’s respiratory weakness progressed to the point that tracheostomy and mechanical ventilation were required prior to discharge to long term acute care facility. The patient and his family had difficulty with accepting the diagnosis, and terminal nature of the disease. Knowing death often occurs within five years of diagnosis, the ICU team alongside palliative care consultants held multiple goals of care discussions. Clinicians should maintain empathy and compassion when treating patients who may be suffering from complications associated with ALS. This abstract is funded by: None
Thota et al. (Fri,) studied this question.