Elsevier

Brain and Development

Volume 35, Issue 9, October 2013, Pages 865-869
Brain and Development

Original article
Combined Guillain–Barré syndrome and myasthenia gravis

https://doi.org/10.1016/j.braindev.2012.10.016Get rights and content

Abstract

Background: Guillain–Barré syndrome and myasthenia gravis both lead to muscle weakness but the two combined is uncommon. Detection of these entities can help identify forms of autoimmune neuromuscular diseases that may respond to immunotherapy. This report sought to characterize the clinical features of these two entities when combined. Methods: This report is of a case of combined Guillain–Barré syndrome and myasthenia gravis. The clinical features were analyzed and correlated to those published in English literature from 1960 to 2012. Ten reports and 12 cases, including the present case, were reviewed. Results: There were 12 patients (4 women and 8 men), aged 17 to 84 years, with combined Guillain–Barré syndrome and myasthenia gravis. Four had post-infectious Guillain–Barré syndrome followed by the development of myasthenia gravis concurrently or concomitantly within one month. All cases had symptoms of ptosis and areflexia. The other common presentations were limb weakness, oculobulbar weakness, and respiratory involvement. Functional outcome was mentioned in 10 patients and seven had good outcome (Hughes scale ⩽2). Conclusion: Detection of ptosis with or without ophthalmoplegia, distribution of limb weakness, and reflex can help in recognizing combined Guillain–Barré syndrome and myasthenia gravis. The early recognition of this combination of peripheral nervous and neuro-muscular junction inflammation is important for initial treatment and prognosis.

Introduction

Guillain–Barré syndrome (GBS) and myasthenia gravis (MG) are different autoimmune disorders affecting the peripheral nerves and neuro-muscular junction, respectively. GBS is an acute, post-infectious autoimmune disease of peripheral nerves that cause rapidly progressive weakness, areflexia, and in severe cases, early respiratory insufficiency. Adult onset MG is a neuro-muscular transmission disorder involving the formation of antibodies to acetylcholine receptors. It is usually associated with thymoma or thymic hyperplasia and pathologic fatigability with fluctuating weakness, such as limb and respiratory muscle weakness, is the cardinal symptom. Where MG presents before 19 years of age, it is termed juvenile MG and it divided into pre-pubertal, peri-pubertal and post-pubertal. Although juvenile MG shares many features with the more common adult onset MG, there are many important differences in its gender prevalence, severity, etiology and treatment. Pre-pubertal juvenile MG is more likely to an equal male: female ratio, manifestation of ocular myasthenia, less frequent AChR antibodies and a higher probability of achieving remission. Peri- or post-pubertal patients presenting with juvenile MG share more similarities with adult onset MG. [1].

Interestingly, both diseases share several similar clinical features on presentation, including muscle weakness and respiratory failure. However, the combination of these two autoimmune conditions in the same patient is very rare. To date, there have been only a few case reports on combined GBS and adult onset MG in literature [2], [3], [4], [5], [6], [7], [8], [9], [10], [11], [12] and there are no childhood-onset MG patients. Here is a case of combined GBS and juvenile MG. The clinical features of cases published in English literature were analyzed through a systematic literature review.

Section snippets

Case report

A 17-year-old man presented at the emergency department with dysarthria and dysphagia, followed by fever and flu-like symptoms 7 days before consult. His symptoms rapidly deteriorated to dyspnea and respiratory distress within hours. He was admitted to the pediatric intensive care unit for mechanical ventilation. On neurologic examination, he was conscious and alert, and had a moderate flaccid tetraparesis with absence of deep reflexes on all four limbs. Signs of cranial nerve involvement such

Methods

Literature was reviewed through a PubMed search of the period 1960–2012 using key words related to Guillain–Barré syndrome and myasthenia gravis. Patients with combined GBS and MG were identified and their clinical data as gender, past history, preceding illness, clinical presentation, CSF finding, GBS type, presence of AChR antibody, mediastinal mass, treatment, and outcome were reviewed. Descriptive statistics were used to determine the characteristics of these entities and their respective

Results

Ten reports on 11 cases of combined GBS and MG, and the current patient report were reviewed (Table 1). The 12 patients (4 women and 8 men) were aged between 17 years and 84 years. Four had post-infectious GBS followed by the development of MG concomitantly within one month. Eight patients had both diseases at different time points, including four patients with a past history of MG who later developed GBS and four patients with a past history of GBS who later developed MG.

The type of GBS was

Discussion

Both GBS and MG are autoimmune diseases caused by auto-antibodies against peripheral nerves and the neuro-muscular junction, respectively. However, the combination of both diseases is rare, with very few case reports [2], [3], [4], [5], [6], [7], [8], [9], [10], [11], [12]. The age of the patients reviewed from the literature is from 29 to 84 years, and they should be classified as adult onset MG. There are no childhood-onset MG patients. In our case, the patient is 17 years old, which would be

Conclusions

The very few case reports in literature may reflect the relative difficulty in diagnosing both GBS and MG in one patient with severe symptoms, as both diseases may present with similar symptoms. Especially when GBS presents as progressive muscle weakness with areflexia, ptosis without ophthalmoplegia, fluctuating weakness, and fatigued muscle strength that characterize concurrent MG can be easily overlooked. Detection of ptosis with or without ophthalmoplegia, distribution of limb weakness, and

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1

These authors contributed equally to this work.

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