Amyotrophic lateral sclerosis with respiratory failure and dysautonomia: a case report

• Published in: Annals of medicine and surgery 2023-07, Vol.85 (7), p.3623-3625
• Main Authors: Chapagain, Subodh, Khati, Nirajan, Lama, Roshani, Karki, Rikesh, Aryal, Roshan, Pangyani, Binaya, Koirala, Anupam
• Format: Article
• Language: English
• Subjects: Case Reports
• ISSN: 2049-0801; 2049-0801
• DOI: 10.1097/MS9.0000000000000889

Amyotrophic lateral sclerosis (ALS) is a disease that affects both upper and lower motor neurons, causing a range of symptoms beyond the motor system. Recent research has shown that the autonomic nervous system can also be affected, with symptoms such as orthostatic hypotension, fluctuations in blood pressure, and dizziness being reported. A 58-year-old male presented with left lower limb limping, difficulty climbing stairs, and left foot weakness, followed by right upper limb weakness and was diagnosed with ALS and received edaravone and riluzole treatment. He presented again with right lower limb weakness, shortness of breath, and wide fluctuations in blood pressure, leading to ICU admission with new diagnosis of ALS with dysautonomia with respiratory failure and was managed with non-invasive ventilation, physiotherapy, and gait training exercises. ALS is a progressive neurodegenerative disease affecting motor neurons but non-motor symptoms can also occur, including dysautonomia, which can result in blood pressure fluctuations. Dysautonomia in ALS is caused by several mechanisms such as severe muscle atrophy, prolonged ventilatory support, and upper and lower motor neuron lesions. Management of ALS involves giving a definitive diagnosis, providing nutritional support, using disease-modifying drugs such as riluzole and non-invasive ventilation to improve survival and quality of life. Early diagnosis is essential for effective management of the disease. Early diagnosis, use of disease-modifying drugs, non-invasive ventilation, and maintaining the patient's nutritional status are crucial for managing ALS which can have non-motor symptoms as well.