What neurological sign is commonly associated with Tay-Sachs disease?

Tay-Sachs disease is a genetic disorder caused by a deficiency of an enzyme called Hex-A, which leads to the accumulation of GM2 gangliosides, particularly in the nerve cells of the brain. The most common neurological sign associated with Tay-Sachs disease is hyperreflexia, which refers to an exaggerated response to stimuli in the muscle reflexes.

As the disease progresses, children often exhibit increased muscle tone and brisk reflexes. The pathological changes in the central nervous system due to the buildup of harmful substances lead to neurological deterioration, contributing to these exaggerated reflex responses. Over time, while motor function declines, the hyperreflexia can be noted in conjunction with other neurological deficits.

In this context, hyperreflexia is a key clinical finding that supports the diagnosis of Tay-Sachs, as it is characteristic of the upper motor neuron involvement seen in the disease. This reflects the overall impact of the disease on neurodevelopment and motor function, highlighting the importance of recognizing this neurological sign during evaluation.