Juvenile Ocular Myasthenia Gravis: A Case Report

Ferry Yulianto,Dewi Sutriani Mahalini,Made Paramita Wijayati,Anak Agung Mas Putrawati Triningrat,I Gusti Ngurah Made Suwarba

doi:10.11648/j.cnn.20200404.14

Ferry Yulianto, Dewi Sutriani Mahalini + Show 3 more

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https://doi.org/10.11648/j.cnn.20200404.14

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Abstract

Myasthenia gravis is a chronic autoimmune disease of neuromuscular blockade, characterized by rapid fatigability of striated muscle. Estimated incidence has been reported at 1/200,000 to 1/1,000,000 worldwide, but its incidence in Indonesia is not clearly recorded. We report a rare case of juvenile ocular myasthenia gravis which is considered rare in child population. The objective of this case report is to describe the proper assessment of its clinical presentation. Patient is an eleven years old boy who had ptosis and opthalmoplegia on both sides for three weeks duration. Its complaint was exaggerated with sustained gaze on daily activities. Ptosis was triggered quickly after being induced by fixated upgaze without blinking and improved after ice pack was put on his eyelids for five minutes. However, his vision was preserved and no slurred speech, dysphagia or limb weakness on physical examination. Neostigmine test showed positive result and rapid nerve stimulation test also revealed withsignificant decrement on the affected eyelid muscles. No thymoma was seen on chest CT scan and thyroid function test was also normal. Hence the diagnosis was made and pyridostigmine was started for its medication. This report present a rare case of juvenile ocular myasthenia gravis. Early recognizing by some diagnostic modalities confirms its diagnosis so that treatment could be started to control the muscle weariness and improving patient's quality of life.

Highlights

Myasthenia gravis is a chronic autoimmune disease of neuromuscular blockade, characterized clinically by rapid fatigability of striated muscle, extraocular and palpebral muscles and those of swallowing [1]
The immune-mediated nature of Myasthenia Gravis was suspected as early as 1960, when Simpson speculated that it was an autoimmune disease with antibodies directed against skeletal muscle Acetylcholine Receptor (AChR) [3]
Our patient has just started the pubertal phase and has the typical mainly ocular manifestation which with no involvement of limb weakness. This case report presents a myasthenia gravis and its clinical manifestation occurred in childhood which considered rare in pediatric population

Summary

Introduction

Myasthenia gravis is a chronic autoimmune disease of neuromuscular blockade, characterized clinically by rapid fatigability of striated muscle, extraocular and palpebral muscles and those of swallowing [1]. Prepubertal patients are more likely to have ocular only myasthenia, whereas a majority of postpubertal patients with myasthenia will have generalized symptoms [5] In this case, our patient has just started the pubertal phase and has the typical mainly ocular manifestation which with no involvement of limb weakness. Our patient has just started the pubertal phase and has the typical mainly ocular manifestation which with no involvement of limb weakness This case report presents a myasthenia gravis and its clinical manifestation occurred in childhood which considered rare in pediatric population. It’s early subtle manifestation is not conspicuously seen makes it challenging to be diagnosed in early phase since some of this kind of disease may progress

Case Report

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