Article Text

Download PDFPDF
Behavioural and cognitive dysfunction across basal ganglia disorders
  1. Andrea Eugenio Cavanna1,2
  1. 1Department of Neuropsychiatry, University of Birmingham and BSMHFT, Birmingham, UK
  2. 2Sobell Department of Motor Neuroscience and Movement Disorders, Institute of Neurology, UCL, London, UK
  1. Correspondence to Dr Andrea Eugenio Cavanna, Consultant in Behavioural Neurology, Department of Neuropsychiatry, The Barberry National Centre for Mental Health, 25 Vincent Drive, Birmingham B15 2FG, UK; andrea.cavanna{at}

Statistics from

Request Permissions

If you wish to reuse any or all of this article please use the link below which will take you to the Copyright Clearance Center’s RightsLink service. You will be able to get a quick price and instant permission to reuse the content in many different ways.

Gras and colleagues present the results of an interesting and accurate study on the clinical phenomenology and long-term prognosis of a large series of 28 patients with genetically confirmed benign hereditary chorea (BHC).1 BHC is a rare genetic condition with an autosomal dominant pattern of inheritance. The defects have been identified in mutations affecting the TIFT1 gene (now named NKX2-1), which encodes the thyroid transcription factor 1, while the clinical phenotype has traditionally been defined by the triad of chorea, hypothyroidism and lung disease (‘brain-thyroid-lung syndrome’). Specifically, the clinical picture of BHC has long been thought to be characterised by childhood …

View Full Text


  • Linked article 302505.

  • Competing interests None.

  • Provenance and peer review Commissioned; internally peer reviewed.

Linked Articles