Lingual Muscle Training in Late-Onset Pompe Disease (LOPD)

Lingual Muscle Training in Late-Onset Pompe Disease (LOPD)

This study is being done to test the effects of Lingual Muscle Therapy (LMT) in patients with Late-Onset Pompe Disease (LOPD) who have tongue weakness. The results of this study will help design future research studies about LMT in LOPD.

Inclusion criteria:

• Age ≥ 12 years
• Lingual weakness as measured by quantitative muscle testing (≤ 5% lower limit of normal for stratified age)
• Confirmed diagnosis of LOPD
• On enzyme replacement therapy for ≥ 26 weeks at pretest
• Able to follow directions for study participation
• Able to complete a home-based LMT regimen
• Access to reliable internet connection
• Access to use of electronic device that allows for FaceTime, Jabber, or WebEx use

Exclusion Criteria:

• Neurodegenerative conditions (e.g. stroke, dementia) or other serious neurologic condition that would prevent meaningful study participation as determined at the discretion of the principle investigator
• Any current or past history of seizures.
• Head and neck cancer or radiation treatment to head/neck
• Inability to tolerate IOPI device tongue bulb
• Inability to give legally effective consent
• Inability to read and understand English