AU - Garg, Kabir AU - Kar, Sujita TI - Mental health perspectives of Hunter syndrome: Case reports of two biological siblings PT - CASE DP - 2016 Jul 1 TA - Medical Journal of Dr. D.Y. Patil University PG - 534-536 VI - 9 IP - 4 4099- https://journals.lww.com/mjdy/pages/default.aspx/article.asp?issn=0975-2870;year=2016;volume=9;issue=4;spage=534;epage=536;aulast=Garg;type=0 4100- https://journals.lww.com/mjdy/pages/default.aspx/article.asp?issn=0975-2870;year=2016;volume=9;issue=4;spage=534;epage=536;aulast=Garg AB - Hunter syndrome is a rare X-linked recessive disorder caused by deficiency of the lysosomal enzyme iduronate-2-sulphatase, leading to progressive accumulation of a substance called glycosaminoglycans in nearly all cell types, tissues, and organs. Hunter syndrome presents with facial dysmorphism, airway diseases, skeletal defects, cardiomyopathies, and neuropsychiatric manifestations. Mental subnormality is a cardinal feature in Hunter syndrome. This is a progressive cognitive decline that is not amenable to enzyme replacement therapy. Due to progressive cognitive decline, training the children to improve the adaptive functioning is a challenge that creates immense stress for the caregivers. Patients with Hunter syndrome should undergo serial assessment of intellectual ability and may be trained accordingly.