Abstract

Abstract Biotinidase deficiency is an inherited metabolic disorder that can present with seizures, hypotonia, and breathing issues in the newborn period. The BTD gene makes the enzyme biotinidase that aids the body in reusing biotin, a B vitamin that is essential for breaking down fats, proteins, and carbohydrates. If left untreated, developmental delay, vision and hearing loss, ataxia, skin rashes, and alopecia may develop. Treatment involves lifelong management with biotin supplements. This case report indicates that bruxism could be a dental manifestation in patients with biotinidase deficiency owing to its psychological and psychosomatic effects.

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