Various neurological complications occur in primary or secondary antiphospholipid syndrome (APS) consisting of cerebrovascular attacks, ocular events, dementia, seizure, chorea, and transverse myelopathy that are all related to the titer of antiphospholipid antibodies (aPL). We report a patient with chorea and retinal vessel occlusion as manifestations of systemic lupus erythematosus (SLE) and ...

Functional imaging studies in patients with Sydenhams chorea have suggested brain perfusional abnormalities. In this study, we aimed to investigate the cerebral perfusion patterns of the cortical/subcortical structures by using Tc-99m hexamethylpropylenamine oxime single photon emission computed tomography in children with Sydenhams chorea, accompanied with magnetic resonance imaging and crania...

Huntington disease has been linked to increased dopaminergic neurotransmission in the striatum, and clinical studies have demonstrated that the associated chorea can be treated with dopamine antagonist or dopamine-depleting drugs. The origin of this hyperdopaminergic status is unknown. Because substantia nigra pars compacta and the ventral tegmental area are the main sources of striatal dopamin...

Homovanilic acid (HVA) was determined in the lumbar CSF of 12 patients with Huntington's disease and 12 with Sydenham's chorea before and after probenecid administration. The means of HVA concentration (basal and after probenecid) were lower in those with Huntington's disease than in controls, and were even lower in a sub-group characterised by increased tone and slowness of voluntary movement....

Investigation of motor function in a group of 17 patients with Huntington's disease reveals that, in addition to the chorea that many patients exhibit, defects in voluntary motor performance also are evident. Fast simple wrist flexion movements to 15 degrees or 60 degrees were slower, and individual movements showed greater variability than seen in normal subjects. This bradykinesia was most pr...

BACKGROUND Movement disorders in Huntington's disease are often medically refractive. The aim of the trial was assessment of procedure safety of deep brain stimulation, equality of internal- and external-pallidal stimulation and efficacy followed-up for 6 months in a prospective pilot trial. METHODS In a controlled double-blind phase six patients (four chorea-dominant, two Westphal-variant) w...

Huntington’s chorea is a genetic disorder characterized by cognitive, motor and psychiatric impairments. It is caused by a dominant mutation on chromosome 4 featuring the multiplication of a portion of the gene in which the codon CAG occurs. CAG codes for the amino acid, glutamine and so the Huntingtin protein (htt) features an excessive expression of glutamine. The abnormal protein htt is pron...

In cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL), clinical presentation with movement disorders such as dystonia and progressive supranuclear palsy-phenotype are rarely reported. None of the CADASIL cases, to our knowledge, has been reported with chorea. Herein, we describe a Korean woman with CADASIL who had presented with chorea. F-fl uor...