A 62 year old diabetic and hypertensive male presented with sudden onset generalized chorea. Investigations revealed uncontrolled diabetes with absent ketones and normal serum osmolality. Achievement of euglycemia with insulin therapy abolished the involuntary movements completely within a day. The direct effect of hyperglycemia causing striatal neuronal dysfunction could be the pathogenesis of...

An 11-year-old boy presented to our clinic with abnormal involuntary jerky movements of the limbs for 3 months. Examination revealed fidgetiness with frequent rapid, irregular, and aimless involuntary movements of all 4 limbs, hypotonia, frequent facial grimacing, and dysarthria (Movie I in the online-only Data Supplement). The movements used to disappear with sleep. He was unable to sustain hi...

The pharmacological treatment of Huntington's chorea has been based on the use of neuroleptic drugs, on the hypothesis that there is a functional dopaminergic hyperactivity in this illness (Agid, 1975). More recently, after the observation of reduced cholinergic and GABAminergic activity at the striatal level in brains of choreic subjects (McGeer et al., 1973; Stahl and Swanson, 1974; Bird and ...

no abstract

Huntington's disease [HD] is a progressive neurodegenerative condition characterized by movement disorder, cognitive impairment, and behavioral symptoms. It inherited as an autosomal-dominant trait normally manifests in mid-adulthood. HD common India parts of Central Asia, with prevalence rate 4–8 per 100 000 most European populations. Juvenile onset affects around 5–10% cases, signs appearing ...

Sydenham's chorea (SC) is a disorder of the central nervous system (CNS) characterized by sudden, involuntary, arrhythmic, clonic, and purposeless movements. SC appears to provide a model for understanding various neuropsychiatric dysfunctions. Its relationship with attentional deficits, obsessive-compulsive symptoms (OCS) as well as movement disorders provides support for the hypothesis of the...

Chorea is one of the non-thrombotic neurological complications associated with antiphospholipid antibodies (aPL) and it is the most common motor disorder associated with autoimmune disease both in adult and paediatric populations. It is characterized by involuntary, aimless, rapid, non-stereotyped movements involving mainly face and limb muscles; psychiatric symptoms such as emotional lability ...

The uptake of dopamine (DA) by platelet rich plasma was assayed in II patients with Huntington's chorea (H. C). The results confirmed the previous observation that platelets from H.C. patients take up, at equilibrium, more dopamine than do platelets from normal control subjects. The mean difference was 50% higher at DA substrate concentrations of 0.11 mM. However, attempts to confirm the higher...

A genetic linkage study between benign hereditary chorea and the locus D4S10 using the DNA probe G8 has shown two recombinations in five small families. There were negative lod scores at recombination fractions that show conclusive evidence of linkage in 16 larger British Huntington's disease families. We suggest that although benign hereditary chorea and Huntington's disease may have some clin...