PubMed Health⌕ Search
Home/Archive/PubMed

PubMed · 12122808

The Gordon-Hey reflex.

G W Bruyn·P Voswinckel
View original source ↗Export metadata ↓

Abstract

Nowadays, a neurologist, even in many years of practice, rarely sees a patient with Sydenham's chorea. Things were quite different during the period +/- 1850 - +/- 1950, when 'chorea minor' was the subject of hundreds of publications. In those days, the practising neurologist stood a good chance of coming across patients with such a degree of muscular hypotonia (a characteristic feature of severe Sydenham's) that he was inclined to infer the presence of paralysis. Those instances used to be denoted as chorea mollis or chorea paralytica. According to textbooks then considered to be authoritative, any doubt regarding the diagnosis of Sydenham's chorea was largely removed if one succeeded in eliciting Gordon-Hey's reflex. This eponym turns out to be as fascinating as the question of its still largely unclarified pathophysiology. In spite of that, mention of the reflex has wholly disappeared from the textbooks of neurology since circa forty years.

Explore related subjects

ChoreaEponymsGermanyHistory, 19th CenturyHistory, 20th CenturyHumansReflex, AbnormalUnited KingdomBiographyHistorical ArticleJournal ArticlePortrait

Keep this discovery

Explore connections, maps & timelines

BibTeXRIS

G W Bruyn, P Voswinckel. 2002. The Gordon-Hey reflex.. https://doi.org/10.1076/jhin.11.2.174.15187

Cite the original work for its findings. Save a collection to share your selection of sources.

KEEP EXPLORING

Related citations

PubMed2006

Effect of donepezil on motor and cognitive function in Huntington disease.

Striatal cholinergic dysfunction may be important in Huntington disease (HD). We studied whether donepezil improves chorea, cognition, and quality of life (QoL) in HD. Thirty patients were randomly assigned to treatment with donepezil or placebo. At the doses studied, donepezil did not improve chorea, cognition, or QoL. Adverse events were similar between both groups. Based on this small sample study, donepezil was not an effective treatment for HD.

Chorea↗
PubMed2006

[The chorea of Zezé].

The authors analyse the correspondence between Eça de Queiroz, a well-known Portuguese writer, and his wife, in 1900, describing the neurological disease of their second son, José Maria d'Eça de Queiroz, know as Zezé. In those letters, the writer mentions chorea as his son diagnosis, explaining in detail its clinical picture. In addition, the writer reveals the medical thoughts of the time about the aetiology, treatment and natural history of Sydenham's Chorea.

Chorea↗
PubMed2006

Myofibrillogenesis regulator 1 gene (MR-1) mutation in an Omani family with paroxysmal nonkinesigenic dyskinesia.

Two recurrent missense mutations (c.20C>T: A7V; c.26C>T: A9V) in the gene encoding the myofibrillogenesis regulator 1 (MR-1) have been shown to cause autosomal dominant paroxysmal nonkinesigenic dyskinesia (PNKD) in 13 families of primarily European ancestry. Here we report an Omani PNKD family with seven affected family members and autosomal dominant inheritance. Our linkage analysis provided consistent positional evidence that the MR-1 gene could be the responsible disease gene. Sequence analysis identified a MR-1 missense mutation (c.20C>T; A7V) in the affected family members, whereas it was not present in five unaffected family members and 129 population controls. Taking into account that previous haplotype analyses did not reveal evidence for common founders among several PNKD families, our present findings strengthen three implications: (1) autosomal dominant PNKD seems to be a homogenous disorder, for which the MR-1 gene is the major disease gene; (2) mainly two recurrent MR-1 missense mutations (57% V7, 43% V9) account for the genetic variance of familial PNKD; (3) it supports current evidence that some of the recurrent MR-1 mutations may have arisen independently by de novo mutation at functionally convergent key sites of the brain-specific MR-1L isoform.

Chorea↗