1/9. An investigation into diet treatment for adults with previously untreated phenylketonuria and severe intellectual disability.There is evidence in the literature which suggests that adults with previously untreated phenylketonuria (PKU) benefit from a low phenylalanine diet. A prospective study providing a phenylalanine-restricted diet to five subjects with severe intellectual disability arising from untreated PKU is reported. Physical, social and behavioural measures were used to monitor the effects of the diet Four out of the five subjects derived considerable benefit. It is concluded that the restricted diet is worth trying in most individuals with previously untreated PKU, and that possible benefits are in the areas of concentration, alertness, mood, irritability and adaptive behaviour.- - - - - - - - - - ranking = 1keywords = behaviour (Clic here for more details about this article) |
2/9. phenylalanine-restricted diet should be life long. A case report on long-term follow-up of an adolescent with untreated phenylketonuria.The aim of this brief report is to stimulate a debate and to advocate life-long treatment with phenylalanine restricted diet to persons with phenylketonuria. We present a case of an adolescent male who today is a 65 year old with moderate mental retardation. He was previously observed as having severe challenging and self-injurious behaviour, sleeping disorder, hyperactivity, and masturbation. In the last year before reassessment he displayed loss of weight, muscle weakness resulting in wheelchair placement, upper respiratory infections, and fungal skin infections. After reassessment in 1998 the patient was started on treatment with a phenylalanine-restricted diet supplemented with vitamin B12, iron, and folic acid. At follow-up one year later the patient had regained his weight and was walking around freely. The challenging and self-injurious behaviour, and the masturbation, had ceased, and he was participating in social activities which he had previously not been able to participate in. At follow-up four years later he was still at this level of accomplishment. In general, this patient's quality of life had increased. We therefore recommend life-long treatment with phenylalanine restricted diet for persons with previously treated, and also for those with untreated, phenylketonuria.- - - - - - - - - - ranking = 1keywords = behaviour (Clic here for more details about this article) |
3/9. The use of a low phenylalanine diet with amino acid supplement in the treatment of behavioural problems in a severely mentally retarded adult female with phenylketonuria.If phenylketonuria is untreated in infancy, it causes irreversible brain damage. No dietary treatment can alter this brain damage. However, it has been shown that dietary treatment can reduce behaviour disturbances often associated with the condition. In this study, a subject's behaviour was monitored over 18 weeks while on a low phenylalanine diet. Results demonstrate a significant reduction in the level of her disturbed behaviour.- - - - - - - - - - ranking = 3.5keywords = behaviour (Clic here for more details about this article) |
4/9. agoraphobia in phenylketonuria.We describe agoraphobia as a complication of phenylketonuria (PKU) in young adults. The five patients have classic PKU and received phenylalanine-restricted diet only in childhood. Only one has normal intelligence. All but one were also depressed. All were anxious. Three of the five had initiated the phenylalanine-restricted diet after 3 months of age. Two returned to the phenylalanine-restricted diet with dramatic reduction of symptoms. The frequency of manifestations of agoraphobia was also examined in 50 young women with PKU enrolled in a longitudinal study of psychosocial factors in maternal PKU, 47 of their acquaintances and 49 women with diabetes. All were administered a test of agoraphobic-avoidant behaviour. The women with PKU appeared to be more prone to social withdrawal and fear of leaving home. Twenty per cent were within the agoraphobia range of the Mobility Inventory. Those still on diet and those with non-PKU hyperphenylalaninaemia reported less avoidant behaviour than those who had terminated the diet in childhood. These results suggest that young adults with PKU are at risk for agoraphobia but that return to the phenylalanine-restricted diet may be an effective treatment.- - - - - - - - - - ranking = 1keywords = behaviour (Clic here for more details about this article) |
5/9. Phenylketonuria and anorexia nervosa.A girl with phenylketonuria and mild mental retardation developed a behaviour disorder and anorexia nervosa at the age of 14. The anorexic symptoms disappeared at the age of 20, but the behaviour disorder worsened, leading to compulsory detention in a psychiatric hospital. serum phenylalanine levels may have influenced the course of the disorder.- - - - - - - - - - ranking = 1keywords = behaviour (Clic here for more details about this article) |
6/9. Phenylketonuria revisited: treatment of adults with behavioural manifestations.Dietary treatment of three PKU patients with irreversible changes is described and elevated.- - - - - - - - - - ranking = 2keywords = behaviour (Clic here for more details about this article) |
7/9. Use of a restricted protein diet in the treatment of behaviour disorder in a severely mentally retarded adult female phenylketonuric patient.A 54-year-old profoundly mentally retarded female patient with phenylketonuria and a severe behaviour problem was treated with a restricted protein and high energy diet. After several weeks of dietary intervention the patient's behaviour improved significantly.- - - - - - - - - - ranking = 3keywords = behaviour (Clic here for more details about this article) |
8/9. The use of a low phenylalanine diet in response to the challenging behaviour of a man with untreated phenylketonuria and profound learning disabilities.If phenylketonuria (PKU) is not treated from an early age with a reduced phenylalanine diet, irreversible brain damage may occur. Although dietary intervention can do nothing to alter this impairment once it occurs, there is some evidence to suggest that a low phenylalanine diet may help to reduce the level of behavioural disturbances frequently experienced by people with untreated PKU. Using this evidence, dietary intervention was used in response to the challenging behaviour of a 30-year-old man with profound learning disabilities. Initial improvements were observed, but overall the results were inconclusive. Possible confounding factors are discussed. An unusual reaction to neuroleptic medication whilst on the diet is also documented.- - - - - - - - - - ranking = 3keywords = behaviour (Clic here for more details about this article) |
9/9. Benefits of normalizing plasma phenylalanine: impact on behaviour and health. A case report.An elderly man with mental retardation who had never received dietary treatment for his phenylketonuria was placed on a phenylalanine-restricted diet. Social skills and walking gait improved and a new interest in the objects in his environment developed spontaneously. A 2-year analysis of diet, blood plasma phenylalanine levels and behavioural state indicated that small differences in phenylalanine intake impacted his well-being. Of significant note, leg tremor and spasm that precipitated severe self-injury were only reversible when plasma blood phenylalanine concentrations were titrated to near normal ranges and daily phenylalanine intake was strictly controlled. This case may offer a potential explanation for some of the late treatment failures that have been reported and suggest new avenues to explore in the late treatment of PKU.- - - - - - - - - - ranking = 2.5keywords = behaviour (Clic here for more details about this article) |