LAMBERT-EATON MYASTHENIC SYNDROME IN A TERTIARY NEUROLOGY CENTRE. Issue 12 (15th November 2016)

• Record Type: Journal Article
• Authors: Ellul, Mark; Bonello, Michael; Wieshmann, Udo
• Is Part Of: Journal of neurology, neurosurgery and psychiatry. Volume 87:Issue 12(2016)
• Page Start: e1
• Page End: e1
• Publication Date: 2016-11-15
• Subjects: Neurology -- Periodicals; Nervous system -- Surgery -- Periodicals; Psychiatry -- Periodicals; 616.8
• DOI: 10.1136/jnnp-2016-315106.131
• Languages: English
• ISSNs: 0022-3050

Abstract

Abstract : Introduction: Lambert-Eaton myasthenic syndrome (LEMS) is a rare but well-characterised neuromuscular disorder. 50–60% of cases are associated with small cell lung cancer (SCLC). The Dutch-English LEMS tumour association prediction (DELTA-P) score was developed to guide malignancy screening in LEMS. Aim: To review the clinical features, investigations, management and clinical course of LEMS patients at the Walton Centre over a 20 year period. Methods: Using pharmacy records, we identified LEMS patients treated with 3, 4-diaminopyridine (3, 4-DAP). We then reviewed medical records, imaging and laboratory results. Results: Of 16 patients analysed, 8 had SCLC and one had thymoma. All had lower limb weakness at presentation. DELTA-P score discriminated between SCLC-LEMS and NT-LEMS (p=0.04), as did smoking history (p=0.03). Bulbar symptoms, autonomic symptoms, age at onset and weight loss alone did not discriminate between SCLC-LEMS and NT-LEMS. Most cancers were diagnosed on the initial computed tomography (CT) or positron emission tomography (PET)-CT. Most patients responded to treatment with either 3, 4-DAP, intravenous immunoglobulin or steroids. Conclusions: Our survey confirms that the DELTA-P score is a useful clinical tool to stratify screening for malignancy in patients with LEMS, but suggests that isolated clinical features, such as bulbar or autonomic symptoms, are not necessarily indicative of SCLC-LEMS.