Importantly, the tongue muscles appear to be particularly susceptible to pathology in Pompe disease. For example, Carlier et al.(2011) evaluated MRI images of Pompe patients, and concludedthat while the majority of facial muscles were unaffected in Pompe,the tongue was always affected. Specifically, T1 weighted imagesshowed “massive fat content” in the tongue, but the facial muscleswere “systematically spared”. Moreover, the appearance of tonguepathology was unrelated to overall disease severity (i.e., the tongue