Pediatric Autoimmune Ocular Myasthenia Gravis: Evaluation of Presentation and Treatment Outcomes in a Large Cohort

Fisher, Kristen; Gill, Jason; Todd, Hannah; Yang, Michael B.; Lopez, Michael A.; Abid, Farida; Lotze, Timothy; Shah, Veeral S.

doi:10.1016/j.pediatrneurol.2021.01.009

Cited by 6 publications

(7 citation statements)

References 24 publications

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“…The extraocular muscle is the most common muscle involved in MG. Cases in which only ocular muscles are involved are called ocular MG. If other skeletal muscles are affected, then it is termed generalised MG. Around 10%–35% of juvenile MG is ocular MG 12. Our case also had ocular MG in the form of ptosis.…”

Section: Discussionmentioning

confidence: 49%

Acute liver failure due to hepatitis A virus presented with Guillain-Barré syndrome and ocular myasthenia gravis

KS,

Kumar,

et al. 2023

BMJ Case Rep

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Hepatitis A is one of the most common causes of acute viral hepatitis in children. Immunological manifestations involving the nervous system are rare with hepatitis A infection. We report a case of a toddler who presented with acute liver failure secondary to hepatitis A infection. The child showed clinical and laboratory improvement initially with conservative management. However, after the initial improvement, she developed acute-onset ptosis along with areflexia. Serological and neurophysiological tests revealed the occurrence of ocular variant Guillain-Barré syndrome and ocular myasthenia gravis, which was only partially responsive to treatment (intravenous immunoglobulin and pyridostigmine). A sudden clinical deterioration was noted after the onset of ptosis. She succumbed on day 40 of hospitalisation due to hospital-acquired infection along with the primary hepatic pathology. This is a rare coincidental presentation of acute viral hepatitis A infection with autoimmune manifestations.

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Section: Discussionmentioning

confidence: 49%

Acute liver failure due to hepatitis A virus presented with Guillain-Barré syndrome and ocular myasthenia gravis

KS,

Kumar,

et al. 2023

BMJ Case Rep

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“…According to the regimen in our hospital since 2010, if drug treatment is effective and there is no recurrence, treatment is expected to be completed in approximately 1.5 years to 2 years. However, as has been reported [7][8][9], for some patients, the effects of the drugs are insufficient, or the patient has repeated recurrences, necessitating a more extended period of drug administration. Therefore, the duration from initial treatment to a patient reaching either no symptom or only minimal symptoms of functional limitations from MG varies, and this variability was also observed in our data.…”

Section: Discussionmentioning

confidence: 94%

“…Anticholinesterases, corticosteroids, and immunosuppressants are used for treatment, and the treatment period is often prolonged when corticosteroids or immunosuppressants are used [8]. Moreover, most patients experience fluctuating symptoms or recurrence [7,9], requiring long-term treatment.…”

Section: Introductionmentioning

confidence: 99%

“…This may be owing to rare diseases and long treatment periods, and there have been few detailed reports regarding the course of treatment and duration of medication [6,11]. Several reports have suggested that when corticosteroids are used for a long duration, switching to immunosuppressants should be considered to reduce the side effects [8,12,13]. However, the appropriate administration period for corticosteroids and immunosuppressants has not yet been established.…”

Section: Introductionmentioning

confidence: 99%

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Clinical Characteristics and Treatment of Juvenile Myasthenia Gravis—A Single-Center Experience

Maeda,

Shimomura,

Tokunaga

et al. 2024

Children

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Juvenile myasthenia gravis (MG) is a rare autoimmune neuromuscular disease, often treated with anticholinesterases, corticosteroids, and immunosuppressants. However, optimal treatment durations remain unclear. This study investigated the clinical characteristics and treatment of juvenile MG, including medication duration. The administration period for all drugs, immunosuppressants, and prednisolone at doses greater than 0.35 mg/kg daily was extracted retrospectively from medical records. Nineteen participants (8 boys, 11 girls) aged 8 months to 14 years (median, 2.5 years) at onset were identified. Fourteen patients (73.7%) had ocular MG and five (26.3%) had generalized MG. Drug treatment was conducted in 18 cases; however, 7 patients did not complete the treatment. Among the patients who completed drug treatment, the duration of treatment ranged from 11 to 100 months (median, 47 months). In the six patients treated with continuous administration of prednisolone or immunosuppressants, the treatment duration ranged from 33 to 99 months (median, 56 months). No severe adverse effects requiring hospitalization were reported. The patients treated with prednisolone or immunosuppressants required at least 33 months of treatment. These results will help develop protocols for juvenile MG treatment.

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“…1 Ocular only presentations are more common, though severe refractory generalized MG presentations also occur. 2 An observational examination is key to the diagnosis and follow-up of pediatric MG patients in the clinic setting. 3 Treatment options are limited by side effect and growth considerations, as well as lack of approved MG medications in the pediatric population.…”

mentioning

confidence: 99%

Pediatric Myasthenia Gravis

Tiongson

2023

rrnmf

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Pediatric myasthenia gravis (MG) is a relatively rare, but very treatable condition. Prognosis in pediatric myasthenia gravis is favorable for minimal manifestation status (MMS) or remission when compared to adults. Ocular only presentations are more common, though severe refractory generalized MG presentations also occur. An observational examination is key to the diagnosis and follow-up of pediatric MG patients in the clinic setting. Treatment options are limited by side effect and growth considerations, as well as lack of approved MG medications in the pediatric population. Multidisciplinary care should be considered for pediatric MG, similar to other neuromuscular conditions seen in specialty care settings.

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Pediatric Autoimmune Ocular Myasthenia Gravis: Evaluation of Presentation and Treatment Outcomes in a Large Cohort

Cited by 6 publications

References 24 publications

Acute liver failure due to hepatitis A virus presented with Guillain-Barré syndrome and ocular myasthenia gravis

Acute liver failure due to hepatitis A virus presented with Guillain-Barré syndrome and ocular myasthenia gravis

Clinical Characteristics and Treatment of Juvenile Myasthenia Gravis—A Single-Center Experience

Pediatric Myasthenia Gravis

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