Disease relevance of Chorea

• An expanded polyglutamine domain in huntingtin underlies the pathogenic events in Huntington disease (HD), characterized by chorea, dementia and severe weight loss, culminating in death [1].
• Letter: Asphyxiation, bulimia, and insulin levels in Huntington disease (chorea) [2].
• Huntington's disease (HD) is an autosomal, dominantly inherited neurodegenerative disorder that is characterized by abnormal involuntary movements (chorea), intellectual impairment and selective neuronal loss [3].
• The disease manifests at approximately 3-4 years and is characterized by both pyramidal and extrapyramidal abnormalities, including chorea, dystonia, ataxia, gait instability, spasticity, seizures, mutism, and intellectual impairment [4].
• The most common types of levodopa-induced dyskinesias in patients with Parkinson's disease (PD) are chorea and dystonia, and often the two types are intermixed [5].

Psychiatry related information on Chorea

• Huntington's disease (HD) is an autosomal dominant neurological disorder characterized by progressive chorea, cognitive impairment and emotional disturbance [6].
• Baclofen therapy may be associated with chorea in Alzheimer's disease [7].
• Among HD patients, both 24-hour EE/SMR and spontaneous physical activity correlated with the severity of chorea, but SMR and total energy expenditure did not [8].
• The Sydenham's chorea group had a higher frequency of major depressive disorder, tic disorders, and attention deficit hyperactivity disorder (ADHD) than both the comparison and rheumatic fever groups [9].
• Pemoline induced chorea and Gilles de la Tourette's syndrome [10].

High impact information on Chorea

• Phenytoin and choreic movements [11].
• Convalescent sera and sera from other streptococcal diseases in the absence of chorea did not activate the kinase [12].
• Chorea monoclonal antibodies showed specificity for mammalian lysoganglioside and N-acetyl-beta-D-glucosamine (GlcNAc), the dominant epitope of the group A streptococcal (GAS) carbohydrate [12].
• These results are relevant to the clinical cases of chorea associated with elevated concentrations of estrogen, which occur in pregnancy and during oral contraceptive use [13].
• Kainic acid lesions of the striatum dissociate amphetamine and apomorphine stereotypy: similarities to Huntingdon's chorea [14].

Chemical compound and disease context of Chorea

• Letter: Amantadine in chorea [15].
• Reversible chorea due to ranitidine and cimetidine [16].
• In Huntington's disease (HD), CAG repeats extend a glutamine tract in huntingtin to initiate the dominant loss of striatal neurons and chorea [17].
• We studied the temporal patterns of chorea and plasma levodopa profiles in 30 patients with Parkinson's disease whose motor fluctuations were difficult to characterize and treat on the basis of observation alone [18].
• One of 4 patients who received haloperidol had a decrease in the severity of chorea [19].

Biological context of Chorea

• BACKGROUND: In juvenile Huntington disease (HD), dystonia as well as parkinsonism and eye movement abnormalities may be the predominant motor signs rather than chorea [20].
• Primary defects of mitochondrial DNA leading to respiratory chain dysfunction have been described in association with dystonia, chorea and parkinsonism [21].
• It is proposed that MJD, and possibly the other more common expansion mutation diseases such as Friedrich's Ataxia and Huntingdon's Chorea, are multifactorial diseases caused by a hitherto unrecognised autosomal dominant inherited failure to regulate Mn/Mg metabolism in populations living in high Mn/low Mg ecosystems [22].
• We describe a 6 year old girl with chorea following cardiac surgery, the first such report in Japan. The radical operation for total anomalous pulmonary venous return was carried out at the age of 11 months under hypothermia [23].

Anatomical context of Chorea

• These results are consistent with the hypothesis that chorea results from preferential loss of striatal neurons projecting to the lateral globus pallidus and that rigid-akinetic HD is a consequence of the additional loss of striatal neurons projecting to the medial segment of the pallidum [24].
• The severe loss of D2-receptor-bearing striatal neuron, with concomitant loss of dopaminergic projections from the nigra to the posterior putamen, is consistent with both chorea and extrapyramidal rigidity being features of patients with neuroacanthocytosis [25].
• Concentrations of gamma-aminobutyric acid, choline acetyltransferase activity and spiroperidol binding in the basal ganglia did not correlate with the generation of choreic movements [26].
• The global atrophy of cerebral cortex and white matter observed in all degrees of HD may account for the cognitive and neuropsychiatric impairments which often precede the onset of chorea [27].
• When HD patients were divided into more (i.e., HD with chorea; n=4) and less impaired (i.e., without chorea; n=6) groups, both showed significant decreases in left anterior cerebral artery flow velocity during visual spatial executive cognition testing compared with control subjects [28].

Gene context of Chorea

• Increased serum concentrations of monokine induced by interferon-gamma/CXCL9 and interferon-gamma-inducible protein 10/CXCL-10 in Sydenham's chorea patients [29].
• The frequencies of the HLA-DRB1 and HLA-DQB1 alleles in patients with pure chorea were not significantly different from those observed in controls [30].
• We evaluated 91 patients with RF for HLA-A, HLA-B, and HLA-DR antigens; of these, 33 had pure chorea, 26 pure carditis, 16 pure arthritis, and 16 carditis plus arthritis [30].
• Considering that chorea in this patient might be due to the disruption of a gene at either of the 4p15.32 or 4q33 breakpoints, CLCN3 was considered as a candidate gene [31].
• MRI brain showed multiple infarcts in MELAS, hyperintensities in putaminal areas in chorea and bilateral cerebellar atrophy in MERRF [32].

Analytical, diagnostic and therapeutic context of Chorea

• Five days after treatment with haloperidol and penicillin, the chorea began to improve slowly, and completely resolved in 2 months [33].
• Steroid-responsive chorea in childhood following cardiac transplantation [34].
• Common neural mechanisms in experimental chorea and hemiballismus in the monkey. Evidence from 2-deoxyglucose autoradiography [35].
• We report the failure of bilateral globus pallidus internus deep brain stimulation to improve chorea in a patient with chorea-acanthocytosis [36].
• Randomized Double-Blind Study With Prednisone in Sydenham's Chorea [37].

References