Juvenile Myasthenia Gravis and Amblyopia

Ellenhorn, Naomi; Lucchese, Neil; Greenwald, Mark J.

doi:10.1016/0002-9394(86)90598-2

Cited by 12 publications

(4 citation statements)

References 16 publications

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“…These symptoms cause particular problems in children as, if severe, they may cause persistent amblyopia [11]. Most children also develop generalised muscle weakness, which presents as painless fatigability of the bulbar and limb musculature, with resultant dysphonia, dysphagia, and proximal limb weakness.…”

Section: Epidemiology and Clinical Featuresmentioning

confidence: 99%

Juvenile Myasthenia Gravis: A Paediatric Perspective

Finnis

Jayawant

2011

Autoimmune Diseases

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Myasthenia gravis (MG) is an autoimmune disease in which antibodies are directed against the postsynaptic membrane of the neuromuscular junction, resulting in muscle weakness and fatigability. Juvenile myasthenia gravis (JMG) is a rare condition of childhood and has many clinical features that are distinct from adult MG. Prepubertal children in particular have a higher prevalence of isolated ocular symptoms, lower frequency of acetylcholine receptor antibodies, and a higher probability of achieving remission. Diagnosis in young children can be complicated by the need to differentiate from congenital myasthenic syndromes, which do not have an autoimmune basis. Treatment commonly includes anticholinesterases, corticosteroids with or without steroid-sparing agents, and newer immune modulating agents. Plasma exchange and intravenous immunoglobulin (IVIG) are effective in preparation for surgery and in treatment of myasthenic crisis. Thymectomy increases remission rates. Diagnosis and management of children with JMG should take account of their developmental needs, natural history of the condition, and side-effect profiles of treatment options.

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Section: Epidemiology and Clinical Featuresmentioning

confidence: 99%

Juvenile Myasthenia Gravis: A Paediatric Perspective

Finnis

Jayawant

2011

Autoimmune Diseases

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“…Altogether with other ophthalmic sign and symptoms such as opthalmoplegia and strabismus, can be provoked with sustained upgaze for few minutes [12]. These symptoms, if severe, they may cause persistent amblyopia [13]. Weakness is often fluctuating and usually becomes more projected through the day and improves with rest.…”

Section: Discussionmentioning

confidence: 99%

Juvenile Ocular Myasthenia Gravis: A Case Report

Yulianto¹,

Suwarba²,

Mahalini³

et al. 2020

CNN

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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.

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“…Regarding strabismus, lasting exotropia, not esotropia, might cause amblyopia. [14][15][16][17][18] This is because the child tends not to use the externally located eye.…”

Section: Symptomsmentioning

confidence: 99%

Myasthenia gravis in children; Issues and challenges

Nomura

2019

Clinical & Exp Neuroim

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Autoimmune myasthenia gravis (MG) occurs in children, as well as in adults. Childhood myasthenia gravis is characterized by its distinct clinical features. Epidemiological studies have shown the high frequency of onset before the age of 5 years in Japan. Similar results have been reported from China. Clinical types are divided into pure ocular, latent generalized and generalized. In general, childhood myasthenia gravis is milder, mainly with ocular symptoms. Anti-acetylcholine receptor antibody is often negative (seronegative). The responsiveness to treatment is generally good, and almost 90% of patients can achieve complete remission. Early diagnosis and adequate treatments are important.

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Juvenile Myasthenia Gravis and Amblyopia

Cited by 12 publications

References 16 publications

Juvenile Myasthenia Gravis: A Paediatric Perspective

Juvenile Myasthenia Gravis: A Paediatric Perspective

Juvenile Ocular Myasthenia Gravis: A Case Report

Myasthenia gravis in children; Issues and challenges

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