Sandhoff disease
A genetic disorder with symptoms that are very similar to those of Tay-Sachs disease (TSD) and that is characterized by accumulation of fatty material called GM2 ganglioside in the nerve cells of the brain.
Symptoms begin around 6 months of age, with motor weakness, and progress to include difficulties with swallowing and breathing.
Death usually occurs by age 3. Sandhoff disease is an autosomal recessive disorder caused by a mutation to chromosome 5. Unlike TSD, it is most common in the non-Jewish population.
See also Tay-Sachs disease