To report three cases of Juvenile Myasthenia Gravis (JMG) and investigate current guidelines for diagnosis and treatment.

JMG is a rare disorder with an estimated occurrence of 1.2-1.6 cases/million-person years. Diagnosis and management of JMG poses unique challenges compared to adults.

In a one-month period on our child neurology inpatient service, we diagnosed and managed three patients with JMG. Features of the clinical presentations and outcomes serve to illustrate important differences between JMG and patients with adult MG. We present a JMG-specific approach to diagnostic evaluation and management.

Patients included three females ages 13 months, 3 years, and 15 years old. Two presented with generalized weakness and one with isolated ocular symptoms. Diagnostic testing revealed seronegative generalized MG, seronegative ocular MG and AChR-positive generalized MG (with positive EMG findings) respectively. All three patients received pyridostigmine and corticosteroids – one with transient worsening after steroid initiation. Two patients received IVIG. All three patients had symptom improvement after treatment.

JMG is a relatively unstudied disorder given its low prevalence in children compared to adults. Here we report a limited case series of three patients with suspected JMG who presented to our hospital within one month, which is an unusual frequency for this rare disease.  There are no standardized guidelines specific for JMG, with most current recommendations involving adaptation of published adult MG guidelines. Compared with adult MG, the diagnosis and management of JMG poses unique challenges including broader differential diagnosis in children, increased incidence of seronegative JMG prepuberty, difficulties with patient compliance for electrodiagnostic testing, and differences in management strategies.  We report this series of patients as a means of enhancing awareness of the disorder and to emphasize the differences between JMG and adult MG. As standardized guidelines for JMG are lacking, we propose a specific protocol for JMG diagnosis and management.