Histopathology of acute cell loss in Huntington's chorea brain
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Abstract:
Histopathological study of 130 cases of Huntington's chorea revealed in 102 cases microscopic foci of acute cell loss associated with an inflammatory reaction in otherwise normal areas of the affected regions of the brain. Hemi-striata of 12 consecutive cases all showed these areas of acute death when examined by serial section. The neuroanatomic topography and microscopic size of the findings were not found in 53 control cases and appear specific for this disease. It is suggested that these lesions represent the sites of active atrophy within the brain.Keywords:
Histopathology
Background:Anosognosia, or unawareness of illness of deficits, has been observed in Huntington's disease (HD) in relation to motor and cognitive signs and symptoms. Most studies of awareness in HD have used self-report questionnaire methodology rather than asking patients to report on their symptom s in real-time. The two studies in which patients were asked about their chorea in real-time had small sample sizes and only examined patients early in disease progression. Objective:To examine awareness of chorea in real-time in HD patients across a broad range of disease progression. Methods:Fifty HD patients across motor and cognitive impairment severity were asked if they noticed any involuntary movements after completing a simple working memory task used to elicit chorea. A movement disorders specialist rated the presence or absence of chorea while the patients completed the task. Disagreement between the patient and movement disorders specialist's ratings was considered to be an indicator of unawareness. Results:Approximately 46% of patients who exhibited chorea did not report chorea. Eighty-eight percent of participants who acknowledged chorea did not report chorea in all parts of the body that chorea was observed. Conclusions:HD patients demonstrate unawareness of chorea across cognitive and motor sign severity.
Movement Disorders
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To describe the epidemiology and clinical features of Sydenham's chorea in the Aboriginal population of northern Australia a review was conducted of 158 episodes in 108 people: 106 were Aborigines, 79 were female, and the mean age was 10.9 years at first episode. Chorea occurred in 28% of cases of acute rheumatic fever, carditis occurred in 25% of episodes of chorea, and arthritis in 8%. Patients with carditis or arthritis tended to have raised acute phase reactants and streptococcal serology. Two episodes lasted at least 30 months. Mean time to first recurrence of chorea was 2.1 years compared with 1.2 years to second recurrence. Established rheumatic heart disease developed in 58% of cases and was more likely in those presenting with acute carditis, although most people who developed rheumatic heart disease did not have evidence of acute carditis with chorea. Differences in the patterns of chorea and other manifestations of acute rheumatic fever in different populations may hold clues to its pathogenesis. Long term adherence to secondary prophylaxis is crucial following all episodes of acute rheumatic fever, including chorea, to prevent recurrence.
Carditis
Acute rheumatic fever
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Chorea is the hallmark motor feature of Huntington disease (HD) and can negatively impact daily functioning and health-related quality of life (HRQoL).The objective of this study was to evaluate how chorea impacts HRQoL and overall functioning among HD patients participating on the PatientsLikeMe website ( www.PatientsLikeMe.com ).A survey was provided to HD participants and/or their caregivers via PatientsLikeMe (9 February 2017-22 March 2017), comprising multiple-choice and open-ended questions designed to assess how chorea impacts HRQoL and overall functioning, and the importance of treating chorea. The HDQLIFE measurement system was used to evaluate patient-reported outcomes of chorea and compare Anxiety and Stigma scores in participants with high chorea versus those with low chorea [HDQLIFE Chorea scores ≥ 60 (n = 45) vs. < 60 (n = 38)].A total of 115 participants (n = 35 caregivers; n = 80 individuals with HD) were included in this study. Among those experiencing chorea (n = 83, 74% of respondents), 66% indicated it was 'Very Important' to manage chorea; however, only 47% agreed that their current medication regimen helped manage their movements. In general, respondents reported that chorea negatively affected HRQoL [HDQLIFE Chorea mean score (standard deviation): 59.3 (6.1)]. Consistent with this, significantly higher Anxiety (P = 0.0423) and stigma (P < 0.0001) scores were observed among respondents with high chorea than in those with low chorea.These results highlight the negative impact of chorea on HRQoL and overall functioning in individuals with HD. Better chorea treatment options are needed to successfully manage symptoms and to help improve HRQoL in these individuals, and patient experiences of anxiety and stigma should be considered in treatment plans.
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The therapeutic activity of natrium dipropilaceticum in two cases of Huntington chorea and in one case of vascular chorea, has been valued. DPA has putted into evidence a scarce activity in Huntington chorea cases; on the contrary it has showed a high activity for the vascular chorea ones.
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SUMMARY Huntington's disease (HD) is an inherited, neurodegenerative disorder characterized by progressive motor dysfunction, abnormal involuntary movements, emotional disturbances and cognitive decline. There is currently no treatment to modify the progression of HD. Until disease modifying agents are established, symptomatic treatment remains the cornerstone of management. Treating chorea and other motor symptoms may improve the quality of life of sufferers. Multiple interventions have been studied for the treatment of chorea, but tetrabenazine is the only US FDA-approved drug indicated for the treatment of chorea associated with HD. In this article, medications available for the treatment of chorea will be summarized and investigational interventions for the management of chorea will also be briefly reviewed. Although chorea only constitutes part of HD, the movements can be disabling, injurious or bothersome.
Tetrabenazine
Movement Disorders
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Chorea is a hyperkinetic involuntary movement disorder characterized by a random pattern of irregular muscle jerks. This movement may involve any parts of the body. Emotional stress or voluntary movements may exacervate chorea and sleep abolish it. Luys body and striatum are the most important anatomical sites to evoke chorea. The lesion of inner segment of pallidum or ventrolateral thalamus may abolish chorea. Measurements of neurotransmitter changes of Huntington's disease show diminution of striatal GABA neurons and preserving nigrostriatal dopamine neurons. Dopamine antagonists can reduce chorea because doperminergic hyperactivity contribute to exacervate chorea. Precise pathophysiological mechanism of chorea is controversial, therefore its classification is not established. On the clinical point of view, classification according to heredity is useful to make diagnosis because hereditary diseases are easily confirmed by family history or specific biochemical markers. There are two groups of underlying diseases of non hereditary chorea. One is unilateral chorea usually due to contralateral hemispheric lesions to chorea. Another is bilateral chorea usually due to degenerative, metabolic or toxic brain diseases. Recent identification of abnormal DNA structure (trinucleotide repeat) in Huntington's disease may greatly contribute to classify underlying diseases of chorea.
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Tetrabenazine
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Chorea is defined as a syndrome characterized by brief, abrupt involuntary movements resulting from a continuous flow of random muscle contractions. There are genetic and non-genetic causes of chorea. The most common genetic cause of chorea is Huntington's disease (HD). Non-genetic forms of chorea include vascular choreas, auto-immune choreas, metabolic and toxic choreas, and drug-induced choreas. This chapter provides an overview of clinical features, pathogenesis and management of HD, other important genetic causes of chorea, Sydenham's chorea, other autoimmune choreas and vascular choreas.
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The causes of chorea are numerous and it is convenient to distinguish hereditary chorea and acquired syndromes with chorea. Huntington's disease represents the most typical disorder with chorea and the clinical diagnosis must be considered if there is chorea intellectual decline and autosomal dominant inheritance. The availability of molecular analysis for diagnostic confirmation shows that the clinical diagnosis in experienced centers is very accurate. Predictive testing involves several ethical problems, especially related to the severity of the disease and its absence of treatment. The other causes of hereditary chorea are less frequent and specific clinical contexts are evoqual of acquired choreas.
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The nationally-recognized Susquehanna
Chorale will delight audiences of all
ages with a diverse mix of classic and
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performances have been described
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aim to make the audience feel,
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working at an extremely high
musical level.AÂAÂAÂAÂAÂAÂAÂAÂAÂAÂAÂAÂAÂAÂAÂAÂAÂAÂAÂAÂAÂAÂAÂAÂAÂAÂAÂAÂAÂAÂAÂA¢AÂAÂAÂAÂAÂAÂAÂAÂAÂAÂAÂAÂAÂAÂAÂAÂAÂAÂAÂAÂAÂAÂAÂAÂAÂAÂAÂAÂAÂAÂAÂAÂAÂAÂAÂAÂAÂAÂAÂAÂAÂAÂAÂAÂAÂAÂAÂAÂAÂAÂAÂAÂAÂAÂAÂAÂAÂAÂAÂAÂAÂAÂAÂA Experience choral
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