309 - GM1 Gangliosidosis and Oral Self-Injury: A Case Report

GM1 gangliosidosis is a lysosomal storage disease that progressively destroys neurons in the brain and spinal cord. Degree of severity typically depends on the age of onset and rate of psychomotor deterioration, with Type I (infantile), being the most severe of the three types. The most notable characteristics that individuals with this condition present with include developmental arrest followed by psychomotor deterioration, dystonia, progressive feeding difficulties, and skeletal abnormalities. Oral self-injurious behavior (SIB) has been reported to be common in certain diseases, syndromes, and disorders. Biting is reported to be the most common form of SIB in patients with mental delay. Historical treatment options in patients with compulsive oral SIB have included psychological, pharmacological, intraoral devices, or surgical interventions. In this report, we present a 12-year-old male with GM1 gangliosidosis who presented with a chief concern of chronic oral self-injurious behavior, our initial treatment, and long-term comprehensive care considerations.