Dilated fixed pupils and respiratory failure: a rare clinical course of Lambert-Eaton myasthenic syndrome

BackgroundLambert-Eaton myasthenic syndrome (LEMS) is a neuromuscular junction disorder and the clinical triad consists of proximal muscle weakness, autonomic symptoms and reduced tendon reflexes. Sluggish pupillary reflexes are common but dilated fixed pupils are rare.Case presentationWe report a p...

Full description

Saved in:
Bibliographic Details
Published in:BMJ neurology open 2023-08, Vol.5 (2), p.e000426-e000426
Main Authors: ten Brinck, Michelle FM, Verheijen, Inge WH, van de Wardt, Jaron, van Dijk, Gert W, Nijhuis, Frouke AP, Verrips, Aad
Format: Article
Language:English
Subjects:
Antibodies
Dyspnea
Hospitalization
Intubation
Laboratories
LAMBERT EATON SYNDROME
Muscle strength
NEUROMUSCULAR
Patients
PUPILS
Respiratory failure
Short Report
Citations: Items that this one cites
Items that cite this one
Online Access:Get full text
Tags: Add Tag
No Tags, Be the first to tag this record!
Description
Summary:BackgroundLambert-Eaton myasthenic syndrome (LEMS) is a neuromuscular junction disorder and the clinical triad consists of proximal muscle weakness, autonomic symptoms and reduced tendon reflexes. Sluggish pupillary reflexes are common but dilated fixed pupils are rare.Case presentationWe report a patient with a rare clinical course of LEMS. The patient was hospitalised due to progressive dyspnoea. She was ambulant and independent of oxygen at hospitalisation. The following day she suffered an in-hospital cardiac arrest based on hypoxia due to sputum stasis. The neurology department was consulted since the patient did not trigger on the ventilator after cessation of sedation. On neurological examination, the patient had dilated and fixed pupils, severe muscle weakness and areflexia, but a normal consciousness. Finally, she was diagnosed with LEMS. In this case report, the clinical course and diagnostic workup including anti-VGCC antibody testing, imaging and the results of electrophysiological studies are discussed. We also emphasise the importance of malignancy screening since the conventional chest CT was negative for lung carcinoma, but PET-CT raised a high suspicion for small-cell lung carcinoma.ConclusionsA rare course of LEMS, with early respiratory failure and wide, fixed pupils. Regarding repetitive nerve stimulation, it is important to stimulate long enough to see the incremental response. Furthermore, this study illustrated the importance of malignancy screening with PET-CT when there is a high suspicion of small-cell lung carcinoma with negative conventional CT.
ISSN:2632-6140
2632-6140
DOI:10.1136/bmjno-2023-000426