1/9. Remotivation therapy and Huntington's disease.Huntington's disease (HD) is a genetic, autosomal dominant, neurodegenerative disorder for which there is no known cure. Because remotivation therapy (RmT) has been effective in other neurological conditions and because of the lack of current efficacious treatments for HD, RmT may be a beneficial therapy for persons suffering from the disease. By cultivating a more fertile environment, RmT leads to increased self-awareness, self-esteem, and an improved quality of life, even in late-stage HD. Two recent studies using animal models suggest that exposure of transgenic HD mice to a stimulating, enriched environment helped to prevent the loss of cerebral volume and delay the onset of motor disorders. Six case studies are presented that demonstrate improved physical, mental, and social functioning in persons with HD when a more stimulating environment is provided.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
2/9. Case vignette: genetic secrets.Mrs. Thomas, age 50, became concerned after experiencing intermittent uncontrollable jerky body movements. She contacted her family physician and, following a full diagnostic evaluation, learned that she is experiencing early symptons of Huntington's disease. This illness is a degenerative disease of the central nervous system, which will ultimately lead-to physical incapacity, dementia, and death. The disease is known to be transmitted genetically as an autosomal dominant trait, with the first onset of symptoms usually occurring in middle age. For example, the child of an affected person has a 50% chance of inheriting the gene, and thus the illness, and a 50% chance of passing it on to their children. Mrs. Thomas is unaware of any history of the disease among others in her family; however, her father was an adopted child, who died in an automobile accident at the age of 37 and may well have been a carrier of the gene. It is highly likely that other members of her family, including her siblings and children, may be carriers of the gene and ultimately transmit it to their children before clinical symptoms of the disease develop. Her own son married last year, and her two brothers have children of childbearing age. Genetic screening and counseling are available for those at risk for Huntington's disease; however, Mrs. Thomas does not want to discuss her diagnosis with family members, fearing that they may blame her and that she may lose her job and friends if the information becomes public knowledge. What advice would you give to the health-care providers caring for Mrs. Thomas regarding the assorted rights, duties, and obligations surrounding this situation?- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
3/9. Cognitive-behavioural therapy with a Huntington's gene positive patient.The treatment of choice for depression or anxiety after genetic testing is the use of medication. The present paper reports a case of a lady who had a positive test result for Huntington's disease (HD). After the predictive test her mood declined and she experienced symptoms of anxiety. Cognitive-behavioural therapy (CBT) enabled her to deal effectively with her negative automatic thoughts (NATs) and interpret situations more realistically. Therapy was successful in reducing her level of physical, behavioural and affective symptoms and in increasing her sense of control. These gains were maintained at 3 and 6 months in spite of the death of her mother. The paper discussed the potential value of cognitive-behavioural theory and therapy in similar cases.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
4/9. Monozygotic twins discordant for huntington disease after 7 years.BACKGROUND: huntington disease (HD) has only rarely been identified in identical twins. All described twins have had disease onset within 1 year of each other, suggesting that disease onset is determined solely by genetic influences. OBJECTIVE: To describe a unique set of monozygotic twins in whom clinical HD onset is at least 7 years apart. DESIGN: A 71-year-old woman was diagnosed as having HD based on medical history, physical examination results consistent with HD, and a CAG trinucleotide repeat number of 39 in the HD gene on chromosome 4. Her onset was 6 years earlier. Her genetically confirmed identical twin, carrying the same number of CAG repeats, was neurologically healthy when examined the next year. Only the HD-manifest twin had chronic bronchitis, rheumatoid arthritis, type 2 diabetes mellitus, and chronic anemia. Both had hypertension. CONCLUSIONS: To our knowledge, this is the first report of monozygotic twins discordant for HD by more than 2 years. The onset of HD symptoms in a patient with 39 triplet repeats at least 7 years earlier than her identical twin suggests the possibility that the disease may be initiated (or delayed) by environmental factors. We have identified increased cigarette use and longer exposure to various industrial toxins as potential explanations for the earlier onset in one twin.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
5/9. hypnosis and the treatment of Huntington's disease.Huntington's Disease is an inherited fatal disorder of the central nervous system. literature on the hypnotic treatment of this disease is extremely sparse. We treated two patients with Huntington's Disease using a wide variety of hypnotic interventions. One patient was treated for a number of years and the other for 10 consecutive sessions. We discuss the continuously interacting cycle between the primary physical symptoms caused by the disease and the secondary psychological symptoms. Hypnotic techniques and daily self-hypnosis appeared to ameliorate both physical and psychological difficulties, thereby enhancing the quality of life that remained for the patients. Systematic research into the effectiveness of hypnosis with symptoms of Huntington's Disease needs to be undertaken in the future.- - - - - - - - - - ranking = 2keywords = physical (Clic here for more details about this article) |
6/9. An abused psychotic preadolescent at risk for Huntington's disease.An abused 10-year-old girl with a family history of Huntington's disease developed incapacitating abdominal pain with concomitant behavioral symptomatology suggestive of dementia. The pseudoneurologic nature of her symptoms was clarified through exhaustive evaluation and did not appear to be that of early-onset Huntington's disease. Assessment included pediatric, psychiatric, neurologic, and gynecologic examination; extensive radiologic and laboratory tests; and chronobiology studies. Successful treatment necessitated the integration of numerous therapeutic modalities including dynamically oriented psychotherapy, psychopharmacologic intervention, physical therapy, behavior modification, and electroconvulsive therapy.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
7/9. Hereditary progressive chorea without dementia.A family with hereditary non-Huntington's chorea is presented. Transmission was autosomal dominant with variable penetrance. chorea commenced in childhood and affected predominantly the head, face and upper limbs. dysarthria appeared later, followed in two family members by elements of an axial dystonia. There was no intellectual impairment. Unlike previously described families, symptoms progressed steadily up to the eighth decade, causing considerable physical disability.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
8/9. social work with victims of Huntington's disease.Huntington's Disease (HD) is a progressive, hereditary neurological disease that affects cognitive, behavioral, and physical functioning. It has a severe impact on the family, the members of which are affected by the patient as well as the vulnerability of children at risk. Social workers can provide support and anticipatory guidance to patients and their families. adult children can benefit from genetic counseling regarding their own at risk status. Community involvement in voluntary organizations is also beneficial.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
9/9. Educational intervention for a Huntington's disease caregiver.A lack of knowledge has been linked to increased stress for caregivers. Educational programs are an inexpensive intervention with potential to improve caregivers' provision of care and to reduce the associated stress. The present description is of a Huntington's disease caregiver's participation in a training program for at-home care of a physically dependent, demented person. Care knowledge was deficient prior to participation and increased with participation. Collaterally, confidence in providing assistance increased and stressfulness of care fell. While increased knowledge remained at follow-up, confidence and stressfulness returned to pre-intervention levels. Measures of affect mirrored the pattern of change in confidence and stressfulness. This suggests that education increased knowledge but this gain was not associated with longer term improvement in the caregiver's affect, stressfulness of continuing to provide care, and confidence in providing care. Implications for such programs are discussed.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |