Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0013362 (dysarthria)
 3,768 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Sydenham's chorea (chorea minor, St. Vitus dance, rheumatic encephalitis), described by Thomas Sydenham in 1686, is considered one of the major manifestations of rheumatic fever (1, 2, 3, 4). Clinically it is characterized by involuntary movements, hypotonia, dysarthria, emotional disorders, and less frequently, by other neurological manifestations such as weakness, headache, seizures and sensory abnormalities (1,4). The motor disorders may be generalized or unilateral, in this case constituting a hemichorea (3). Chorea may present associated to other rheumatic fever manifestations during an acute episode, or in isolated form, characterizing the so-called "pure" chorea (5, 6, 7). Its etiology and pathophysiological mechanisms are still unclear, although its relation with a previous pathophysiological group A Beta-hemolytic streptococcus infection is well established (8). There is also evidence of the participation of immunological mechanisms in its pathogenesis, such as the finding of serum anti-nucleus caudatus and anti-subthalamic antibodies (9) and increase in IgG levels in cerebrospinal fluid of patients with chorea (10). In developed countries due to the reduction in rheumatic fever incidence and decrease in frequency of chorea as its manifestation (3, 11), the latter has become rare. However, in developing countries rheumatic fever remains a public health problem. In Brazil, in the last years an increase in the incidence of chorea has been observed as part of the clinical picture of rheumatic fever (12). The present study reports the clinical and laboratory findings of 187 cases of Sydenham's chorea followed-up during the period of January 1980 to December 1990 in two university centers in the city of Sao Paulo, Brazil.
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PMID:Sydenham chorea: clinical and laboratory findings. Analysis of 187 cases. 134 Oct 4

Eleven children with Sydenham's chorea (8 girls and 3 boys, mean age = 8.4 +/- 2.2 [SD] years) underwent comprehensive physical, neuropsychologic, and psychiatric examination. The chorea was manifested as dysarthria, gait disturbances, and frequent adventitious movements of the face, neck, trunk, and extremities. Antineuronal antibodies were present in 10 of 11 children. All children exhibited concomitant psychologic dysfunction, specifically obsessive-compulsive symptomatology, increased emotional lability, motoric hyperactivity, irritability, distractibility, and age-regressed behavior. Obsessive-compulsive symptoms were observed in 9 (82%) children, 4 of whom met diagnostic criteria for obsessive-compulsive disorder. These behavioral symptoms began several days to weeks before the chorea was observed, and they waxed and waned in severity along with the motoric abnormalities. These results suggest that psychologic, particularly obsessive-compulsive, symptoms are accompanying manifestations of Sydenham's chorea which may require medical attention.
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PMID:Sydenham's chorea: physical and psychological symptoms of St Vitus dance. 846 54

Tetrabenazine (TBZ) is widely used to treat hyperkinetic movement disorders in adults; however, published experience with the drug in children is limited. Common side effects of TBZ include drowsiness, sedation, weakness, Parkinsonism, depression, and acute akathisia, all of which are reversible with decreased doses. We report here a 7-year-old girl with rheumatic chorea who developed acute akinesia of all four limbs and dysarthria due to TBZ therapy. Withdrawal of the drug led to rapid improvement within 18 hours.
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PMID:Quadriparesis and dysarthria due to tetrabenazine therapy in a child with rheumatic chorea. 2202 10

Isolated motor disturbances in the paediatric population are uncommon presentations to the emergency department. Choreiform movements have a broad differential diagnosis and may present insidiously with progressive worsening of asymmetric clumsiness, hypotonia and dysarthria. The incidence of Sydenham's chorea (SC) caused by acute rheumatic fever (ARF) is very rare in developed countries. We report a previously healthy, vaccinated 9-year old male who presented to our ED with intermittent and progressive right sided clumsiness for four weeks. Physical examination findings showed dysdiadokinesis and dysmetric movements of the right side, which varied in intensity and were less pronounced on serial re-examination during the same ED visit. Basic bloodwork, MRI and MRA/V showed no abnormalities, and the patient was discharged home with urgent neurology follow-up. He re-presented to our ED four days later with worsening gait and inability to hold a pencil at school. He was subsequently diagnosed with chorea by the neurology team. The cause of chorea was later determined to be SC, and the patient's throat swab came back positive for group A-beta hemolytic strep (GAS) infection. We explore current literature regarding the various presentations of ARF, differential considerations in acute chorea, and diagnostic studies needed to determine the etiology of acute chorea. With the low incidence of chorea in developed nations, this diagnosis can be easily overlooked. We highlight the importance of this diagnosis, as well as primary and secondary treatment in ARF.
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PMID:You can dance if you want to: A case of Sydenham's chorea. 3147 78