Unusual Association of Non-paraneoplastic Variant of Lambert-Eaton Myasthenic Syndrome with Predominant B-cell Inflammatory Myopathy

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Article Type:
Case Report (دارای رتبه معتبر)
Abstract:
Introduction

 Myasthenia gravis disease (MGD) and inflammatory myopathy (IM) are commonly reported in the literature and usually appear with thymic pathology. Lambert-Eton myasthenic syndrome (LEMS) associated with IM is extremely rare.

Case Presentation

 We report a 42-year-old female patient who presented with proximal muscle weakness of the upper and lower limbs, normal creatinine kinase (CK) level, and positive acetylcholine and voltage-gated calcium channel receptor antibodies. There were no oculobulbar symptoms and no history of thymoma, and the electrophysiological tests were unremarkable. Muscle biopsy revealed focal perimysial and perivascular inflammation, predominantly B-cell lymphocytes, in a non-necrotizing muscle.

Conclusions

 LEMS associated with IM, particularly B-cell inflammation, has never been reported in the absence of cancer history. Clinical investigations and myopathological features can help establish the diagnosis.

Language:
English
Published:
Archives of Neuroscience, Volume:10 Issue: 1, Jan 2023
Page:
7
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