RESUMO
Fasting ketoacidosis is especially an underdiagnosed problem in patients with neuro-muscular disease with severely depleted muscular mass. During periods of prolonged fasting, low levels of insulin and high levels of glucagon induce lipolysis in the peripheral fat tissue. This will result in elevated free fatty acids levels in de blood and increased ketogenesis in the liver. These ketones pass into the blood, leading to a ketoacidosis. Patients with low muscular mass are more susceptible to develop ketoacidosis due to lower energy reserves and reduced glycogen stores on the one hand, and a reduced uptake of ketones by their low muscular mass on the other hand. During periods of increased metabolism and in the absence of adequate caloric intake, this can easily lead to severe ketoacidosis. An adequate oral caloric intake is essential in the prevention and treatment of fasting ketoacidosis.