Which syndrome is associated with the absence of deep tendon reflexes and smooth appearance of the tongue?

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The correct answer is associated with Familial Dysautonomia, which is a genetic disorder affecting the autonomic nervous system and is characterized by the absence of deep tendon reflexes and a smooth appearance of the tongue. This syndrome arises due to a mutation in the IKBKAP gene, leading to dysfunction in sensory and autonomic neurons. Individuals with Familial Dysautonomia often exhibit symptoms related to impaired peripheral nervous system function, such as an absence of deep tendon reflexes. The smooth appearance of the tongue is a notable physical manifestation of the condition, often due to the absence of papillae.

Other syndromes listed do not present with these specific features. For instance, Fryns Syndrome is primarily characterized by congenital abnormalities and does not typically include the absence of deep tendon reflexes or a smooth tongue. Goldenhar Syndrome is mainly associated with facial and eye abnormalities, while Incontinentia Pigmenti involves skin, hair, teeth, and nervous system manifestations but does not feature the specific neurological symptoms described. Thus, Familial Dysautonomia is the only option that correctly matches the symptoms presented in the question.

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