A nursing challenge: adult-onset Tay-Sachs disease.
Adult-onset GM2 gangliosidosis (AOG), also labelled Adult-Onset Tay-Sachs disease, is a slowly progressing disease caused by a gradual accumulation of the GM2 ganglioside in neurons due to defective hexosaminidase A. Recent research findings and clinical experiences suggest that AOG may be more widespread than previously believed. Moreover, the diagnosis of AOG is often delayed because patients present with psychotic symptoms that mimic dementia, schizophrenia, mania, and depression. Because AOG patients typically respond poorly to psychiatric drug therapy and the symptomatology is so diverse, nurses must design and implement nursing care that ensures safety, structure, and comfort.
Hamilton
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College of Nursing, Medical University of South Carolina, Charleston 29425.
D000328:Adult; D005260:Female; D006801:Humans; D010347:Patient Care Planning / Q000592:standards*; D011568:Psychiatric Nursing / Q000379:methods*; D013661:Tay-Sachs Disease / Q000175:diagnosis / Q000451:nursing* / Q000503:physiopathology