Adult onset, also known as Late Onset Tay-Sachs disease (LOTS) and Late Onset Sandhoff disease, is a very rare form of Tay-Sachs and Sandhoff disease that usually occurs in individuals in their 20s and early 30s.
Adult onset progresses more slowly than other forms. It is characterized by unsteadiness of gait and progressive neurological deterioration.
The symptoms of LOTS, which present in early adulthood, include speech and swallowing difficulties, unsteadiness of gait, spasticity, cognitive decline, and psychiatric illness. The cherry red spot common to infantile forms is almost never seen in LOTS.
As it is very difficult to identify, when an individual is finally given the diagnosis they often look back to their childhood and notice symptoms that were not obvious at the time. A common early sign is not being athletic and/or speech difficulties or a stutter as a child or teenager. One early indicator that can lead to a LOTS diagnosis is a mental health problem, although this will just be the beginning of a long road to the final diagnosis. About 40% of affected adults experience mental health disorders, such as bipolar disorder or psychotic episodes.
Like all forms of Tay-Sachs and Sandhoff diseases, there is a loss of skills, although in LOTS this is much slower than in the infantile and juvenile forms of the diseases. Over a prolonged period of time, adults with LOTS slowly decline and require more mobility assistance. Although many experience speech and swallowing difficulties, only a few will eventually require a feeding tube.
As LOTS is very hard to diagnose, individuals can experience a lengthy delay in finding out that they suffer from it. It is very common for LOTS to be misdiagnosed as other more common neurological diseases.