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The co-occurrence of serologically proven myasthenia gravis and Miller Fisher/Guillain Barré overlap syndrome — A case report

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Abstract

The co-occurrence of myasthenia gravis (MG) and Guillain Barré syndrome (GBS) is uncommon with a few reported cases in the literature. There is only one reported case of MG and Miller Fisher variant of GBS. We described an 84 year old Chinese woman with underlying seropositive myasthenia gravis (MG) who presented with ophthalmoplegia, areflexia and acute neuromuscular weakness. She was proved to have co-occurrence of MG and GBS/Miller Fisher overlap syndrome with positive anti-GQ1b antibody. The unusual finding in this patient raises an interesting question on their pathogenesis with the possibility that prior activation of the immune system may predispose the development of autoantibodies against other antigens within the same set of muscles.

Introduction

Myasthenia gravis (MG) and the Guillain Barré syndrome (GBS) are well described autoimmune disorders affecting the neuromuscular junction and peripheral nerve respectively which are now recognized to be heterogeneous with autoantibodies against several different antigens [1], [2], [3], [4], [5], [6]. The co-occurrence of MG and GBS is rare. To date there has been only a few cases in the literature [7], [8], [9], [10], [11], [12]. The association of MG and the Miller Fisher syndrome, a variant of GBS, is even rarer, with one case reported [13]. Interestingly, both have remarkably similar clinical features at presentation and neuromuscular transmission abnormalities have been recognized in Miller Fisher syndrome [14], [15], [16], [17].

We report an elderly woman, known to have MG, presenting acutely with ophthalmoplegia and diplopia, bulbar, limb and respiratory muscle weakness, proving to be an acute Miller Fisher/GBS overlap syndrome.

Section snippets

Case report

An 84 year old Chinese lady diagnosed to have ocular myasthenia gravis six years ago and was on pyridostigmine. Five days prior to admission, she developed upper respiratory tract infection followed by ptosis, diplopia, swallowing difficulty and slurring of speech. Her symptoms rapidly deteriorated, with severe weakness of all four limbs and respiratory distress. She was admitted into Intensive Care Unit (ICU) for mechanical ventilation. While on ventilator, she remained conscious and alert.

Discussion

The history and clinical presentation suggested an exacerbation of myasthenia gravis. This diagnosis of MG was supported by repetitive nerve stimulation tests which showed significant decremental response and positive ACh-R antibody. The diagnosis of MG was further confirmed with three Ach-R autoantibodies; namely the ACh-R modulating, binding and blocking autoantibodies.

However, the edrophonium tests performed was negative. Furthermore the NCT had evidence for acute demyelinating peripheral

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