VOLUME 66 , ISSUE 1 ( January-March, 2024 ) > List of Articles
Aparna S Kilani, Raghu Srikanti, Sankari P Arulmozhi Palaniraj, Bhaskara R Nalamala, Priyanka Boppe, Sudheer Diyya, Kalyan Kumar V Penumuchu, Sivaprasad Chilaka, Kaviya Balaji, Raja Annadurai, Eunice Gera, Yashwanth Gunti, Navya S Imadabathuni
Keywords : Anti-immunoglobulin-like cell adhesion molecule 5 disease, Autoimmunity, Case report, Hypercapnic respiratory failure, Noninvasive ventilation, Nonrapid eye movement, Obstructive sleep apnea, Rapid eye movement related, Sleep apnea, Slow-wave sleep
Citation Information : Kilani AS, Srikanti R, Palaniraj SP, Nalamala BR, Boppe P, Diyya S, Penumuchu KK, Chilaka S, Balaji K, Annadurai R, Gera E, Gunti Y, Imadabathuni NS. A Rare Cause of Respiratory Failure: Anti-immunoglobulin-like Cell Adhesion Molecule 5 Disease. Indian J Chest Dis Allied Sci 2024; 66 (1):27-30.
DOI: 10.5005/jp-journals-11007-0107
License: CC BY-NC 4.0
Published Online: 03-04-2024
Copyright Statement: Copyright © 2024; The Author(s).
Background: Anti-immunoglobulin-like cell adhesion molecule 5 (anti-IgLON5) disease is a strange disorder with a complex interplay between autoimmunity and neurodegeneration. The first case with severe air-flow disturbance and sleep apnea associated with the presence of anti-IgLON5 in cerebrospinal fluid (CSF) or serum was described in 2014. The initial common presentation among these patients is sleep apnea with respiratory failure. Case description: A 68-year-old man presented with excessive daytime sleepiness, loud snoring, nocturnal awakening, breathlessness, involuntary movements, and difficulty in swallowing for 1 year. His arterial blood gas (ABG) showed hypercapnic respiratory acidosis. Both CSF analysis and magnetic resonance imaging (MRI) brain were normal. Polysomnography (PSG) showed sleep apnea and rapid eye movement (REM) behavioral disorders. He was tested positive for myasthenia gravis and treated accordingly. Neurological involvements are explained by the presence of serum anti-IgLON5 antibodies. Case discussion: The IgLON5 proteins are cell adhesion molecules involved in neuroplasticity. Patients with anti-IgLON5 disease present with obstructive sleep apnea (OSA), REM, and nonrapid eye movement (NREM) parasomnia, chorea, cognitive decline, and sleep-disordered breathing with stridor and bulbar symptoms. Respiratory failure is explained by bulbar symptoms, sleep apnea, and respiratory muscle fatigue due to myasthenia gravis. Detection of anti-IgLON5 antibodies is crucial for diagnosis. Patients with anti-IgLON5 disease were treated with steroids and immunosuppressants. Conclusion: The variable clinical presentation of neurological symptoms makes it difficult to distinguish the anti-IgLON5 disease from other neurological diseases. When a patient presents with heterogeneous neurological symptoms including distinctive sleep disorders with respiratory failure often accompanied by bulbar symptoms, the anti-IgLON5 disease should always be suspected.