Epigenetic abnormalities in 15q11-13 imprinted region and UBE3A mutation are the two major mechanisms for molecularly\nconfirmed Angelman Syndrome. However, there is 10% of clinically diagnosed Angelman Syndrome remaining test negative.With\nthe advancement of genomic technology like array comparative genomic hybridization and next generation sequencing methods, it\nis found that some patients of these test negative Angelman-like Syndromes actually have alternative diagnoses. Accurate molecular\ndiagnosis is paramount for genetic counseling and subsequent management. Despite overlapping phenotypes between Angelman\nand Angelman-like Syndrome, there are some subtle but distinct features which could differentiate them clinically. It would provide\nimportant clue during the diagnostic process for clinicians.
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