Dilated fixed pupils and respiratory failure: a rare clinical course of Lambert-Eaton myasthenic syndrome
Background Lambert-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 presentation We report a...
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| Format: | Article |
| Language: | English |
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BMJ Publishing Group
2023-11-01
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| Series: | BMJ Neurology Open |
| Online Access: | https://neurologyopen.bmj.com/content/5/2/e000426.full |
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| author | Gert W van Dijk AAD VERRIPS Michelle FM ten Brinck Inge WH Verheijen Jaron van de Wardt Frouke AP Nijhuis |
| author_facet | Gert W van Dijk AAD VERRIPS Michelle FM ten Brinck Inge WH Verheijen Jaron van de Wardt Frouke AP Nijhuis |
| author_sort | Gert W van Dijk |
| collection | DOAJ |
| description | Background Lambert-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 presentation We 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.Conclusions A 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. |
| first_indexed | 2024-03-08T16:36:55Z |
| format | Article |
| id | doaj.art-17bac9f518344fb8b36fa0bf715ae593 |
| institution | Directory Open Access Journal |
| issn | 2632-6140 |
| language | English |
| last_indexed | 2024-03-08T16:36:55Z |
| publishDate | 2023-11-01 |
| publisher | BMJ Publishing Group |
| record_format | Article |
| series | BMJ Neurology Open |
| spelling | doaj.art-17bac9f518344fb8b36fa0bf715ae5932024-01-05T14:50:08ZengBMJ Publishing GroupBMJ Neurology Open2632-61402023-11-015210.1136/bmjno-2023-000426Dilated fixed pupils and respiratory failure: a rare clinical course of Lambert-Eaton myasthenic syndromeGert W van Dijk0AAD VERRIPS1Michelle FM ten Brinck2Inge WH Verheijen3Jaron van de Wardt4Frouke AP Nijhuis5Department of neurology, Canisius Wilhelmina Hospital, Nijmegen, The NetherlandsDepartment of neurology, Canisius Wilhelmina Hospital, Nijmegen, The NetherlandsDepartment of neurology, Canisius Wilhelmina Hospital, Nijmegen, The NetherlandsDepartment of neurology, Canisius Wilhelmina Hospital, Nijmegen, The NetherlandsDepartment of neurology, Canisius Wilhelmina Hospital, Nijmegen, The NetherlandsDepartment of neurology, Canisius Wilhelmina Hospital, Nijmegen, The NetherlandsBackground Lambert-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 presentation We 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.Conclusions A 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.https://neurologyopen.bmj.com/content/5/2/e000426.full |
| spellingShingle | Gert W van Dijk AAD VERRIPS Michelle FM ten Brinck Inge WH Verheijen Jaron van de Wardt Frouke AP Nijhuis Dilated fixed pupils and respiratory failure: a rare clinical course of Lambert-Eaton myasthenic syndrome BMJ Neurology Open |
| title | Dilated fixed pupils and respiratory failure: a rare clinical course of Lambert-Eaton myasthenic syndrome |
| title_full | Dilated fixed pupils and respiratory failure: a rare clinical course of Lambert-Eaton myasthenic syndrome |
| title_fullStr | Dilated fixed pupils and respiratory failure: a rare clinical course of Lambert-Eaton myasthenic syndrome |
| title_full_unstemmed | Dilated fixed pupils and respiratory failure: a rare clinical course of Lambert-Eaton myasthenic syndrome |
| title_short | Dilated fixed pupils and respiratory failure: a rare clinical course of Lambert-Eaton myasthenic syndrome |
| title_sort | dilated fixed pupils and respiratory failure a rare clinical course of lambert eaton myasthenic syndrome |
| url | https://neurologyopen.bmj.com/content/5/2/e000426.full |
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