The Differential Diagnosis of Chorea

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Huntington's Disease, Huntington's Disease Look‐Alikes‎, and Benign Hereditary Chorea: What's New?

In general, however, recognizing the effectiveness of other medications, the potential complications of IVIG and plasma exchange, their high cost, and limited trials, these treatments remain investigational. Selective serotonin reuptake inhibitors are effective for obsessive-compulsive behaviors, benzodiazepines are used for anxiety, and in some patients antidepressants are required.

The recommendation of the World Health Organization is the regular use of secondary prophylaxis with penicillin G benzathine 1.

Allergic patients are treated with oral sulfa drugs, such as sulfadiazine, mg every 6 hours. In contrast to existing beliefs that SC is a self-limited condition, it has been suggested that in up to half of patients, chorea continues to be active 2 years after the acute onset. Further, in many cases, recurrences occur despite the use of penicillin prophylaxis. In multiple instances, a return in symptoms is not associated with a GABHS infection or change in antineuronal antibodies.

Chorea | Radiology Reference Article |

Sydenham chorea is proposed to be a group A beta-hemolytic streptococcal GABHS -induced autoimmune disorder secondary to polyreactive antibodies against streptococci that also recognize neuronal extracellular surface or intracellular antigens. After binding to the normal protein, through the process of molecular mimicry, clinical symptoms may be secondary to altered cell signaling, cellular toxicity, or other mechanisms. Support for an antineuronal antibody proposal in SC is based in part on the documentation of serum autoreactive antibodies against human basal ganglia or rat striatum.

In acute rheumatic fever, cross reactive antibodies against the streptococcal group A carbohydrate antigen N-acetyl-beta-D-glucosamine GlcNAc and cardiac muscle have been associated with rheumatic carditis. In studies using monoclonal antibodies derived from acute SC patients, cross reactivity has been identified between GlcNAc and mammalian lysoganglioside GM1, tubulin, and the dopamine D1 and D2 receptors.

Despite the aforementioned, the microinjection of SC antibodies into rodent striatum does not alter motor behavior or cause detectable cellular changes. Pediatr Neurol.

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A review of practical aspects of Sydenham chorea with diagnostic and treatment flow charts. Cardoso, F. Curr Treat Options Neurol. Quality review of multiple treatment options in Sydenham chorea. Ther Adv Neurol Disord. Thorough review of therapeutics in Sydenham chorea, with excellent summarization of major treatment studies.

Pediat Neurol.

Huntington's Chorea patient

Case review of children treated with corticosteroids. All rights reserved. No sponsor or advertiser has participated in, approved or paid for the content provided by Decision Support in Medicine LLC. Login Register. What are the typical findings for this disease? Reduced muscle tone, tics, grimacing, clumsiness, dysarthria, weakness. Table I. Differential diagnosis: The following conditions may present with findings similar to those of SC: Systemic lupus erythematosus Primary antiphospholipid antibody syndrome Acute encephalitides Drug intoxication Wilson disease Familial chorea Metabolic hyperglycemia Endocrine hyperthyroidism What caused this disease to develop at this time?

What laboratory studies should you request to help confirm the diagnosis? How should you interpret the results? Specific laboratory tests should include: Throat culture Anti-streptolysin O titer Anti DNAse B titer Antinuclear antibody studies Anticardiolipin antibody Antiphospholipid tests Thyroid function tests Renal function tests Hepatic function tests Ceruloplasmin concentration Erythrocyte sedimentation rate C-reactive protein Spinal fluid analysis EEG can be considered Evaluations for other aspects of rheumatic fever i.

Interpretation of laboratory tests: Laboratory studies assist in eliminating alternative causes of chorea, but do not confirm the diagnosis of SC. A single sample is insufficient evidence to determine whether the titer is rising or declining. Cerebrospinal fluid analysis is often normal. EEG is usually normal. There is no specific biological marker for SC.

  • Differential Diagnosis of Chorea | SpringerLink.
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Would imaging studies be helpful? If so, which ones? Imaging is primarily recommended to rule out other causes of chorea.

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If you are able to confirm that the patient has Sydenham chorea, what treatment should be initiated? Primary Therapy: Despite the fact that most patients do not have active GABHS infections at the onset of chorea, it is generally recommended that penicillin therapy be given day course of oral penicillin V or benzathine penicillin G injection. Treatment of chorea: No large, placebo controlled, randomized, blinded studies have been performed, and most recommendations are based on case reports.

Treatment of neuropsychiatric signs: Psychiatric signs are usually short-lived. Prophylaxis of rheumatic fever: The recommendation of the World Health Organization is the regular use of secondary prophylaxis with penicillin G benzathine 1. What are the adverse effects associated with each treatment option?

What causes this disease and how frequent is it? The differential diagnosis of chorea. Pract Neurol. Cardoso F. Chorea: non-genetic causes. Pubmed citation. Promoted articles advertising. Edit article Share article View revision history Report problem with Article.


URL of Article. Article information. System: Central Nervous System. Synonyms or Alternate Spellings: Choreas. Support Radiopaedia and see fewer ads. Loading more images Close Please Note: You can also scroll through stacks with your mouse wheel or the keyboard arrow keys. Loading Stack - 0 images remaining.

By System:. Patient Cases.