Cases reported "Huntington Disease"

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1/6. Treating a mandibular condylar fracture in a patient with Huntington's disease.

    This case illustrates the use of a microplate in the open reduction and fixation of an intracapsular mandibular condylar fracture in a patient who was unable to tolerate a closed reduction because of the nature of her chronic illness. The advantages of this method of fracture fixation include early function and elimination of intermaxillary fixation, which benefited our patient. This technique should be reserved for patients for whom conservative measures are not appropriate.
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2/6. Thursdays with Marion: the therapist's tale.

    We who work with people with chronic and/or terminal illnesses are often confounded about what we ought to do and what we can do for those who suffer. This paper presents a case study of my work with Marion who began therapy after her presymptomatic testing for HD to address her depression and anxiety. The approach we took is a narrative one. The impact of illness on existing family stories is described followed by an examination of the premises of a narrative model. The case description demonstrates the collaborative nature of the therapy. Some thoughts on the role of the therapist in this model are offered.
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3/6. 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.
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4/6. kluver-bucy syndrome in Huntington's chorea.

    In this paper the first case of kluver-bucy syndrome (KBS) in Huntington's chorea is reported. The patient, a 46-year-old man with advanced Huntington's disease, displayed prosopagnosia, oral tendencies, emotional changes, hypersexual behavior, and hyperphagia associated with severe dementia. haloperidol in moderate doses controlled both the KBS and the chorea, suggesting a possible role for the dopaminergic system in the pathogenesis of KBS in Huntington's disease. The presence of profound dementia in our patient supports the previous assertion that human cases of KBS are invariably associated with severe cognitive dysfunction. Since KBS was established as an entity, a great deal of attention has been directed to its neuroanatomical basis. However, due to the multidetermined nature of human behavior, the role of physiological, psychological, and environmental factors should also be taken into consideration with regard to the pathogenesis of this syndrome.
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5/6. Familial psychiatric presentation of Huntington's disease.

    Symptoms of schizophrenia may be encountered in Huntington's disease (HD) but usually when the full clinical syndrome is apparent; prechoreic psychosis is relatively uncommon. We describe a family where all four members affected with HD presented first with a severe psychiatric syndrome, which in three cases was schizophreniform in nature. Two other living members with no current signs of motor disorder have received psychiatric treatment, one for schizophrenia. Concurrence of psychosis and Huntington's disease in this family is unlikely to have occurred by chance, suggesting that there is some feature in this family which gives rise to the psychotic presentation. Families such as this may contribute to the investigation of genetic factors associated with psychiatric illnesses.
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6/6. Which diagnostic procedures in the elderly? The case of late-onset Huntington's disease.

    The typical adult-onset form of Huntington's disease (HD) is a clinical condition in which the latest advances of genetic research can be usefully applied during the course of the diagnostic process; not so clear are the guidelines for the much less frequent late-onset variant. We have recently seen three patients in their late sixties who had been misdiagnosed for up to 10 years due to the apparently isolated, mild, and slowly progressive nature of their hyperkinetic movements or cognitive disorders. Only after the results of dna sequencing on a blood sample became available could the appropriate diagnosis of late-onset HD be reached. By contrast, neuroimaging studies lacked sufficient sensitivity and specificity. Appropriate neurogeriatric assessment in these cases should follow specific guidelines and should always include selected high-technology procedures.
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