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Lambert Eaton Syndrome: A Unique Presentation of the Disease With Isolated Respiratory Muscle Weakness FREE TO VIEW

Urooj Fatima, MD; Tasneem Shah, MD; Paras Dedhia, MD; Muhammad Ebrahim Khan, MD; Sean Devine, MD
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Guthrie/Robert Packer Hospital, Sayre, PA

Chest. 2013;144(4_MeetingAbstracts):326A. doi:10.1378/chest.1704135
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SESSION TITLE: Critical Care Student/Resident Case Report Posters I

SESSION TYPE: Medical Student/Resident Case Report

PRESENTED ON: Tuesday, October 29, 2013 at 01:30 PM - 02:30 PM

INTRODUCTION: Lambert Eaton syndrome is a disorder of neuromuscular junction transmission in which the voltage gated calcium channel antibody (VGCCA) Interfere with the normal calcium influx required for the acetylcholine release. It has a strong association with malignancy especially small cell lung cancer and the diagnosis is usually made on the clinical grounds and confirmed by the presence of VGCCA

CASE PRESENTATION: A 69-year-old female with a significant past medical history of hypertension and chronic venous insufficiency presented with worsening shortness of breath, fatigue, cough, and dizziness for the last several months. Subsequently, patient developed acute hypercarbic respiratory failure, and she was intubated for this. She failed multiple weaning trials with rapid shallow breathing index of 700 and subsequently underwent tracheostomy for the inability to be weaned off the ventilator. In a patient with no prior underlying chronic lung disease, acute onset of hypercarbic respiratory failure and inability to wean off the ventilator was thought to be secondary to the neuromuscular etiology. On the neurological exam, patient did not have any significant abnormality except for diplopia. The result of the electromyography and the nerve conduction velocity was within normal range without any evidence of peripheral neuropathy. To evaluate further acetylcholine receptor antibody and VGCCA were send and she was found to have high titer of VGCCA of greater then 23000. Thereafter patient was started on intravenous immunoglobulin infusion (IVIG) with substantial improvement in the symptoms. Our patient’s presentation is unique as she presented with isolated respiratory muscle weakness as the main manifestation of the Lambert Eaton syndrome in the absence of any underlying malignancy. There was a dramatic response leading to increase in strength with the IVIG infusion.

DISCUSSION: Most patients with Lambert Eaton syndrome present with slowly progressive proximal muscle weakness with depressed deep tendon reflexes and autonomic symptoms. However, most patients do not have significant respiratory muscle weakness, with the respiratory failure seen in the later stages of the disease.

CONCLUSIONS: This case highlights the importance of recognizing Lambert Eaton syndrome as the cause of respiratory failure in patients even without any other manifestation of the disease and further strengthens the evidence of the efficacy of the use of IVIG in this condition.

Reference #1: An improved diagnostic assay for Lambert Eaton myasthenic syndrome .J Neurol Neurosurg Psychiatry. 1995;58 (1):85.

Reference #2: The Lambert Eaton myasthenic syndrome 1988-2008: a clinical picture in 97 patient s. J Neuroimmunol.2008;201-202:153

Reference #3: J Neurol.2005 May; 252 suppl 1:114-8

DISCLOSURE: The following authors have nothing to disclose: Urooj Fatima, Tasneem Shah, Paras Dedhia, Muhammad Ebrahim Khan, Sean Devine

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