title: Late diagnosis of neurodegenerative disease in children: anosognosia by proxy.
abstract: Anosognosia is a term now generally defined as a failure to recognize the existence of disease processes, particularly those with a neurological basis. Denial of illness has been recognized in a large number of disorders, and is generally thought to have not only a central nervous system basis, but to also be influenced by the psychological processes of denial. This disorder has been reported in the adult population, and there are initial suggestions that it exists in children/adolescents. We propose to extend the concept of anosognosia to the caregivers of children who suffer significant dementia, and extended degeneration in neuropsychological and neurological functioning. We term this syndrome anosognosia by proxy. Three case examples are presented in which parents, teachers, and health care professionals observed extended deterioration in a child/adolescent's level of functioning prior to seeking appropriate diagnostic evaluations. Possible explanations for this process are advanced, including a combination of insidious onset coupled with the intimate nature of the child/parent relationship. The need for professional, teacher, and parent education on the nature of childhood degenerative neurological disorders is emphasized.
authors.affiliation: Department of Pediatrics, Oregon Health & Science University, Portland, OR 97201-3098, USA. butlerr@ohsu.edu
authors.forename: Robert W
authors.initials: RW
authors.lastname: Butler
authors.forename: Roger
authors.initials: R
authors.lastname: Light
mesh_terms: D000293:Adolescent; D001364:Awareness; D002648:Child; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D007361:Intelligence Tests; D008297:Male; D001523:Mental Disorders / Q000209:etiology / Q000523:psychology*; D019636:Neurodegenerative Diseases / Q000150:complications / Q000175:diagnosis / Q000523:psychology; D009483:Neuropsychological Tests; D010290:Parents; D010552:Personality Assessment; D019727:Proxy / Q000523:psychology*; D011569:Psychiatric Status Rating Scales