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LAMBERT-EATON MYASTHENIC SYNDROME IN A TERTIARY NEUROLOGY CENTRE
  1. Mark Ellul1,2,
  2. Michael Bonello2,
  3. Udo Wieshmann2
  1. 1 University of Liverpool
  2. 2 The Walton Centre

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.

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