Related Experiment Videos
Progressive bulbar dysfunction and respiratory failure in anti-IgLON5 disease
Zayna Ahmed1, William Hartrey2, Andrew Graham2
1Neurology, Ipswich Hospital NHS Trust, Ipswich, UK zayna.ahmed2@nhs.net.
None:
A man in his 60s presented with progressive bulbar dysfunction, exertional dyspnoea and nocturnal respiratory disturbance. His symptoms began a decade earlier with throat clearing, mild dysphagia and sleep disruption, initially attributed to obstructive sleep apnoea treated with continuous positive airway pressure. Despite therapy, he developed worsening dysphagia, dream enactment, stridor and acute type II respiratory failure requiring emergency intubation and subsequent tracheostomy after repeated failed extubation attempts. Investigations revealed status dissociatus and agrypnia excitata on polysomnography, while serum studies confirmed high-titre anti-IgLON5 antibodies. Immunotherapy with corticosteroids, plasma exchange and rituximab achieved partial improvement, but tracheostomy dependence and bulbar symptoms persisted. He stabilised on nocturnal invasive ventilation and was discharged with coordinated multidisciplinary care. This case highlights the diagnostic pitfalls of attributing progressive neurological features to common mimics, the dual pathology of anti-IgLON5 disease that limits therapeutic response, and the importance of early airway intervention to prevent avoidable mortality.
Related Concept Videos
Respiratory Syncytial Virus Disease
Acute Respiratory Failure-III
Myasthenia Gravis: Overview and Treatment
These antibodies interfere with the function of the nicotinic receptors in three ways: by binding to the receptor and disrupting acetylcholine binding; by causing cross-linking of receptors which leads...
Myasthenia Gravis ll: Pathophysiology
Acute Respiratory Failure-V
Ensure that patients are monitored continuously for their response to therapy, including changes in...
Botulism