A case of hypercapnic respiratory failure

Woensel, Julie Van; Goeminne, Pieter; Valcke, Y.

doi:10.1183/20734735.0217-2020

Cited by 1 publication

(2 citation statements)

References 16 publications

(47 reference statements)

Supporting

Mentioning

Contrasting

Order By: Relevance

“…12 Home noninvasive ventilation (NIV) improves respiratory symptoms and hypercapnic state but has no effect on abnormal sleep behavior and movements. 10,13 In this case, involuntary movements were decreased but respiratory symptoms were not improved. Large randomized trials are needed to determine the safety and long-term efficacy of treatment options for sleep apnea.…”

Section: Treatmentmentioning

confidence: 72%

“…Previous studies show that patients who received steroids alone or patients not receive treatment other than steroids have higher mortality. 10 Combination therapy which includes steroids, intravenous immunoglobulin (IVIg), plasmapheresis, and Rituximab is recommended. A combination of immunotherapy with IVIg could decrease the IgLON5 immunoglobulin G (IgG) titer and improve REM sleep disorder.…”

Section: Treatmentmentioning

confidence: 99%

See 1 more Smart Citation

A Rare Cause of Respiratory Failure: Anti-immunoglobulin-like Cell Adhesion Molecule 5 Disease

Srikanti,

Diyya,

Kilani

et al. 2024

The Indian Journal of Chest Diseases and Allied Sciences

View full text Add to dashboard Cite

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.

show abstract

Section: Treatmentmentioning

confidence: 72%

Section: Treatmentmentioning

confidence: 99%