How to Identify Patients Whose Generalized Myasthenia Gravis (gMG) Symptoms Are Not Well-controlled

Key Takeaways:

• Spot uncontrolled gMG. Look for persistent weakness, functional decline, and reduced quality of life beyond overt myasthenic crises.
• Leverage tools like MG-ADL and MG-QOL15 to objectively track symptom impact and guide treatment.
• A comprehensive approach is key. Address adherence, triggers, and comorbidities for better patient outcomes.

Recognizing when generalized myasthenia gravis (gMG) symptoms are not well-controlled is vital for optimizing patient outcomes. While a range of treatments is available, achieving symptom control can be challenging. Healthcare providers (HCPs) must be adept at identifying signs of inadequate control to intervene effectively.^1,2

Identifying suboptimal symptom control

Suboptimal symptom control in gMG patients often manifests in several key ways, extending beyond overt myasthenia crises. HCPs should look for:²

Persistent or worsening weakness

Patients whose gMG is not well-controlled may report persistent weakness in muscles affecting eye movement (diplopia, ptosis), swallowing (dysphagia), speech (dysarthria), or limb strength, even with ongoing treatment. A noticeable increase in the severity or frequency of these symptoms, or the emergence of new areas of weakness, indicates a need for re-evaluation.^1-3

Increased functional impairment

Evaluate the patient's ability to perform daily activities. Are they experiencing greater difficulty with tasks that were previously manageable, such as eating, speaking on the phone, walking distances, or climbing stairs?²

Tools like the Myasthenia Gravis Activities of Daily Living (MG-ADL) scale can be invaluable for quantifying functional impact and tracking changes over time. A higher score or an increase in score on this patient-reported outcome measure suggests worsening control.⁴

Frequent or prolonged fluctuations

While some fluctuation is characteristic of gMG, poorly controlled patients often experience more dramatic and unpredictable shifts in symptom severity throughout the day or week. They may report "good" days followed by several "bad" days, or a rapid onset of severe weakness after minimal exertion.²

Impact on quality of life

Beyond physical symptoms, assess the patient's overall quality of life. Are they experiencing increased anxiety, depression, or social isolation due to their symptoms?^2-4

The MG-QOL15 questionnaire can provide a standardized measure of this impact and help identify patients whose emotional and social well-being is compromised.^2-4

Increased need for rescue medications or emergency department visits

A red flag for uncontrolled gMG is an increased reliance on pyridostigmine or other symptomatic treatments beyond their usual prescribed regimen, or more frequent visits to urgent care or emergency departments for symptom exacerbations or myasthenic crises.^1,2

Signs of myasthenic crisis

A myasthenic crisis is the ultimate indicator of uncontrolled gMG and requires immediate intervention. A myasthenic crisis is a life-threatening MG complication characterized by severe muscle weakness affecting the respiratory muscles that can lead to respiratory failure. HCPs must be vigilant for acute respiratory distress, severe dysphagia leading to aspiration risk, and profound generalized weakness.¹

Other considerations for HCPs

Several factors are closely intertwined with identifying and managing uncontrolled gMG:

Adherence to treatment

Non-adherence to prescribed medications is a common reason for suboptimal symptom control. HCPs should routinely ask about medication adherence and address any barriers the patient may face, such as side effects, cost, or misunderstanding of the regimen.^1,2

Triggers for exacerbation

Educate patients about common gMG exacerbation triggers, which can precipitate symptom worsening. These include infections (especially respiratory), stress, certain medications, surgery, and pregnancy. Identifying and mitigating these triggers is crucial for maintaining control.⁵/p>

Comorbidities

gMG patients often have coexisting autoimmune diseases, such as thyroid disorders or rheumatoid arthritis, which can complicate symptom management and require concurrent treatment. A holistic approach considering all comorbidities is essential.^1,5

Timely referral to specialists

When gMG symptoms are not well-controlled, collaboration with or referral to a neurologist specializing in neuromuscular disorders is critical. These specialists have expertise in advanced diagnostic techniques and treatment modalities, including immunomodulatory therapies and surgical options like thymectomy.^1,5

Patient education and empowerment

Empowering patients with knowledge about their condition, symptom monitoring, and when to seek medical attention can greatly improve self-management and lead to earlier recognition of worsening control. Encourage patients to keep a symptom diary to track fluctuations and identify patterns.^2,3