نتایج جستجو برای: chorea

تعداد نتایج: 4171  

2010
Maya Bronfeld Katya Belelovsky Yaara Erez Jenia Bugaysen Izhar Bar-Gad Suzan Gonda

27 Chorea is a basal-ganglia (BG) related hyperkinetic movement disorder characterized by 28 irregular continuous involuntary movements. Chorea and related hyper-behavioral disorders 29 may be induced in behaving primates by local microinjections of the GABA-A antagonist 30 bicuculline in the globus pallidus externus (GPe). We performed multi-electrode extracellular 31 recordings in the GPe and...

2013
Magalie Demar Carine Belzunce Christine Simonnet Alain Renaux Philippe Abboud Antoine Okandze Corrine Marois-Créhan Felix Djossou

performed as described in Stiasny et al. (6) showed a strongly positive titer of 1:320. We excluded hepatitis C virus; West Nile virus infection has not been reported in Poland. Because of his religious beliefs, the patient had not been vaccinated against TBE or yellow fever; he had not traveled abroad and had not been exposed to dengue virus. The differential diagnosis included Lyme disease, b...

Journal: :Electroencephalography and clinical neurophysiology 1966
J H Chandler

One of the more tragic features of Huntington's chorea is the delayed appearance of symptoms until affected mdwiduals are well into, or even past, the age of reproductive capacity. It would be desirable for a number of reasons to be able to identify carriers of the gene responsible for this trait before they reach reproductwe age. Patterson et al. (1948) exammed 26 offspring of diagnosed chorea...

Journal: :American journal of human genetics 2016
Niccolò E Mencacci Erik-Jan Kamsteeg Kosuke Nakashima Lea R'Bibo David S Lynch Bettina Balint Michèl A A P Willemsen Matthew E Adams Sarah Wiethoff Kazunori Suzuki Ceri H Davies Joanne Ng Esther Meyer Liana Veneziano Paola Giunti Deborah Hughes F Lucy Raymond Miryam Carecchio Giovanna Zorzi Nardo Nardocci Chiara Barzaghi Barbara Garavaglia Vincenzo Salpietro John Hardy Alan M Pittman Henry Houlden Manju A Kurian Haruhide Kimura Lisenka E L M Vissers Nicholas W Wood Kailash P Bhatia

Chorea is a hyperkinetic movement disorder resulting from dysfunction of striatal medium spiny neurons (MSNs), which form the main output projections from the basal ganglia. Here, we used whole-exome sequencing to unravel the underlying genetic cause in three unrelated individuals with a very similar and unique clinical presentation of childhood-onset chorea and characteristic brain MRI showing...

2012
Robert Fekete Anthony Davidson Joseph Jankovic

BACKGROUND Tetrabenazine is a monoamine depleter with a well-documented effect against chorea associated with Huntington disease (HD). There is a paucity of data about how reduction in chorea relates to better performance on motor, gait, cognitive, and psychiatric assessments. METHODS We designed an open label tetrabenazine withdrawal study to test performance using validated scales. The foll...

2004
John B. Penney

Neuropeptide immunohistochemistry was used to test several hypotheses of the anatomical bases of chorea and rigidity-kinesia. To test the hypothesis that elevated concentration of striatal somatostatin causes chorea, we visually compared the density of striatal neurons containing somatostatin and neuropeptide Y in brains affected by choreic or rigid-kinetic Huntington's disease (HD). The densit...

Journal: :Acta paediatrica 1999
M Elevli A Celebi T Tombul A S Gökalp

Sydenham's chorea is often encountered in pure form, but it may also occur in association with carditis. Recently, it has been reported that silent, mild valvular regurgitation has been encountered in patients with rheumatic fever manifested by isolated polyarthritis and pure chorea. We studied 22 children with Sydenham's chorea to determine clinical and subclinical heart involvement at the tim...

2017
Ray Dorsey Kevin Biglan Shirley Eberly Peggy Auinger Alicia Brocht Chizoba C. Umeh David Oakes Frederick Marshall Ira Shoulson Samuel Frank

Background: The safety and effectiveness of tetrabenazine in different sub-populations of Huntington disease (HD) is not known. We evaluated its safety in individuals on an antidepressant and its effectiveness in advanced HD. Methods: The TETRA-HD study was a randomized, placebo-controlled, double-blind study of 84 individuals with HD who were randomized 2:1 to receive tetrabenazine (54 partici...

1998
Jina L Janavs Michael J AminoV

Dystonia and chorea are uncommon abnormal movements which can be seen in a wide array of disorders. One quarter of dystonias and essentially all choreas are symptomatic or secondary, the underlying cause being an identifiable neurodegenerative disorder, hereditary metabolic defect, or acquired systemic medical disorder. Dystonia and chorea associated with neurodegenerative or heritable metaboli...

Journal: :Journal of neurology, neurosurgery, and psychiatry 1972
W R McLeod D J Horne

Daily use of 7 g l-tryptophan and 70 mg pyridoxine for 14 days led to no deterioration of objectively measured manual motor skills in a small group of patients with Huntington's chorea.

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