A family with hereditary non-Huntington's chorea is presented. Transmission was autosomal dominant with variable penetrance. Chorea commenced in childhood and affected predominantly the head, face and upper limbs. Dysarthria appeared later, followed in two family members by elements of an axial dystonia. There was no intellectual impairment. Unlike previously described families, symptoms progre...

Twenty-four members of a family with benign familial chorea underwent testing for evidence of intellectual impairment. Lower verbal intelligence was found in affected individuals compared to unaffected family members, as were deficits in verbal abstract concept formation. These results challenge the notion that benign familial chorea uniformly spares the intellect in all kindreds.

The possibility that Huntington's chorea might be due to a genetically determined biochemical derangement has been raised on a number of occasions. In 1963,Kenyon and Hardy suggested that abnormalities of magnesium metabolism might account for some of the features of Huntington's chorea and in a group of patients investigated the cellular magnesium levels using the Szvenk-Fekete titration metho...

OBJECTIVE Sydenham's chorea (SC) is thought to be an autoimmune disorder. MRI is generally used to exclude other causes of chorea. There are no typically defined MRI features of SC. In this study we aimed to determine clinical and neuroimaging findings of SC. METHODS In this study 17 patients with acute SC were retrospectively evaluated. Sydenham's chorea was diagnosed according to the 1992 r...

The EEGs are reported on a group of 95 patients with Huntington's chorea. Thirty one showed little activity of any kind, and in particular no alpha rhythm above 10 μV in amplitude was seen. Only those records which still met these criteria when re-examined were included in the `low voltage' category. EEGs in this category occurred significantly more frequently in institutionalized patients and ...

CONTEXT Sydenham's chorea affects almost 30% of patients with acute rheumatic fever. It is more frequent in females and is rare in the first decade of life, and genetic vulnerability underlies it. Because of easy access to antibiotics, it is now rare in so-called developed countries. CASE REPORT A 6-year-old boy with a family history of Huntington's disease, who was the only child of an unscr...

There are currently no effective pharmacological agents available to stop or prevent the progression of Huntington's disease (HD), a rare hereditary neurodegenerative disorder. In addition to psychiatric symptoms and cognitive impairments, HD causes progressive motor disturbances, in particular choreiform movements, which are characterized by unwanted contractions of the facial muscles, trunk a...