Refractory myasthenia gravis, dysphagia and malnutrition: A case report to suggest disease-specific nutritional issues

Abstract 

Objective

We describe a case of refractory myasthenia gravis with bulbar involvement and the nutritional treatment solutions proposed to treat the associated dysphagia and malnutrition.

Methods

A 39-y-old woman with refractory myasthenia gravis was referred to our clinical nutrition unit for deteriorating dysphagia and progressive malnutrition.

Results

The first-line nutritional approach consisted of dietary counseling and thickened meals. Unfortunately, no adequate oral intake was achieved and an enteral nutrition treatment was proposed. A nasogastric tube was removed after a few days due to local pain and poor quality of life. Despite consistent weight loss and overt malnutrition, the patient refused percutaneous endoscopic gastrostomy placement. Neurologic symptoms did not show any improvement but unexpectedly the patient's weight started to increase to previous values. Anamnestic recall revealed that the patient learned by herself how to position the nasogastric tube that is now temporarily used for formula infusion coinciding with neurologic poussés.

Conclusions

Current guidelines consider chronic neurologic diseases with associated dysphagia, where refractory myesthania gravis has also been considered, a unique category. Chronic neurogenic dysphagia with high risk of aspiration, long-term inability to obtain adequate oral intakes, and malnutrition are established indications for percutaneous endoscopic gastrostomy placement. However, patients may need different forms of nutritional intervention during the course of their illness and choices and indications should contemplate ethical reasons, clinical benefits, minimal risks, and acceptable quality of life. Minimally invasive intermittent enteral nutrition might be considered a possible clue for nutritional management of exacerbating dysphagia.

Keywords: Myasthenia gravis, Dysphagia, Malnutrition, Enteral feeding, Percutaneous endoscopic gastrostomy, Quality of life