Benign hereditary chorea

Common Name(s)

Benign hereditary chorea

Benign familial chorea is an inherited non-progressive (does not worsen) movement disorder which typically begins in childhood and remains during ones lifetime. It is not associated with mental or cognitive decline or other motor problems as in other conditions that chorea is part of such as Huntington's disease or Parkinson’s disease. The chorea most often involves the lower legs and arms as well as face and trunk. Individuals with benign familial chorea can be expected to live a long, full life with no additional complications associated with it. Diagnosis is based on physical signs and symptoms and family history.

Benign familial chorea is a genetic condition passed through families in an autosomal dominant manner. This means that in order to have the condition, only one copy of the changed gene is needed to produce the symptoms. An affected individual has a 50% chance of passing the gene change and therefore the condition on to each of his or her children. Mutations have found several genes which seem to be associated with this condition. Genetic counseling can provide a better understanding of the underlying cause and recurrence risks. If your child has been diagnosed with benign familial chorea, talk with your doctor about the latest treatment options.

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Advocacy and Support Organizations

 

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Advocacy and Support Organizations

 

Condition Specific Organizations

Following organizations serve the condition "Benign hereditary chorea" for support, advocacy or research.

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General Support Organizations

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Scientific Literature

Articles from the PubMed Database

Research articles describe the outcome of a single study. They are the published results of original research.
The terms "Benign hereditary chorea" returned 8 free, full-text research articles on human participants. First 3 results:

ADCY5 mutations are another cause of benign hereditary chorea.
 

Author(s): Niccolo E Mencacci, Roberto Erro, Sarah Wiethoff, Joshua Hersheson, Mina Ryten, Bettina Balint, Christos Ganos, Maria Stamelou, Niall Quinn, Henry Houlden, Nicholas W Wood, Kailash P Bhatia

Journal: Neurology. 2015 Jul;85(1):80-8.

 

To determine the contribution of ADCY5 mutations in cases with genetically undefined benign hereditary chorea (BHC).

Last Updated: 7 Jul 2015

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Recurrent drop attacks in early childhood as presenting symptom of benign hereditary chorea caused by TITF1 gene mutations.
 

Author(s): Anna Rosati, Beatrice Berti, Federico Melani, Elena Cellini, Elena Procopio, Renzo Guerrini

Journal: Dev Med Child Neurol. 2015 Aug;57(8):777-9.

 

Drop attacks are sudden, spontaneous falls without loss of consciousness, followed by rapid recovery. Causes in children include severe epilepsies, movement disorders, cataplexy, and psychiatric disorders. We describe two children (a 3-year-old female and a 12-year-old male) with ...

Last Updated: 8 Jul 2015

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Benign hereditary chorea: more than meets the eye.
 

Author(s): James Rice

Journal: Dev Med Child Neurol. 2014 Jul;56(7):606-7.

 

Last Updated: 13 Jun 2014

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Reviews from the PubMed Database

Review articles summarize what is currently known about a disease. They discuss research previously published by others.
The terms "Benign hereditary chorea" returned 1 free, full-text review articles on human participants. First 3 results:

A novel de novo mutation of the TITF1/NKX2-1 gene causing ataxia, benign hereditary chorea, hypothyroidism and a pituitary mass in a UK family and review of the literature.
 

Author(s): Liana Veneziano, Michael H Parkinson, Elide Mantuano, Marina Frontali, Kailash P Bhatia, Paola Giunti

Journal: Cerebellum. 2014 Oct;13(5):588-95.

 

Benign hereditary chorea (BHC) is a rare autosomal dominant condition characterized by early onset, non-progressive chorea, usually caused by mutations in the thyroid transcription factor-1 gene (TITF1). We describe a novel mutation arising de novo in a proband presenting in infancy ...

Last Updated: 5 Sep 2014

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Clinical Trial Information This information is provided by ClinicalTrials.gov

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