Sydenham's Chorea

[Skyllark]

has anyone dealt with Sydenham's Chorea?

[lupus-support1 Administrator]

Sydenham's chorea is a neurological disorder characterized by rapid, jerky, irregular, and involuntary movements (chorea), especially of the face and limbs. Additional symptoms may include muscle weakness, slurred speech, headaches, and seizures. Children with Sydenham's chorea often have emotional or behavioral problems such as obsessive-compulsive disorder, distractibility, irritability, and inappropriate outbursts of laughing or crying. Sydenham's chorea mostly affects children and adolescents and usually follows a Streptococcal infection by anywhere form 1-8 months.

Sydenham's chorea is one of the major clinical signs of acute rheumatic fever. The uncontrolled movements are often worse during periods of stress, fatigue, or excitement. In some cases, only one side of the body is affected. Sydenham's chorea usually resolves within 3 weeks to 3 months. However, symptoms may last longer in some cases.

In most cases, patients with Sydenham's chorea recover fully with no treatment. Drugs that have been used to treat patients with significant movement problems include corticosteroids, valproic acid, diazepam, chlorpromazine, and carbamazepine. In patients who do not respond to these drugs, haloperidol or pimozide may be used. In addition, patients with Sydenham's chorea are usually treated with antibiotics to prevent another Streptococcal infection and to minimize the risk of rheumatic heart disease. Sydenham's chorea usually resolves within 12-15 months, though symptoms may persist for two years or more. Up to 30% of patients experience a recurrence of Sydenham's chorea within a few years. Some believe that treatment with an antibiotic reduces the risk of recurrence.

rarediseases.info.nih.gov/d...

[Skyllark in reply to lupus-support1]

yep, I know this. My wife is the one with CAPS... has been dealing with Syndenhams Chorea for over 5 years now... does plasma Plasmapheresis or — plasma exchange for over that time. There is a trial to use another medication to stop it but it is really expensive ($40k), and controlled by the cancer association here in Canada... and also being used in the USA. It is called Rituximab, We and our specialists here have requested the cancer society to used it and have been denied each time, even though it has been proven to be successful. Well we can use it, just have to pay the $40k, and my own work place will not cover it unless she follows under the normal uses for that treatment. I have even added up the cost of doing plasma exchange over the past 5 years or so, and OHIP has payed out more than 8 times that already.

[lupus-support1 Administrator in reply to Skyllark]

I am very sorry to read this. I know Rituximab as I have this prescribed but don’t have to pay, which doesn’t help you or your wife.I should imagine your having some pretty difficult feelings about your wife’s situation & how powerless you may feel.

This forum is as much for you as your wife so you don’t feel so alone.

If you want to talk, we are here for you too.

With good wishes,

Ros

[KellyInTexas Administrator]

Sorry skylark, I have not heard of this specifically. I was happy for the info lupus- support 1 included.

I am aware there can the chorea involved with APS. Dr Hughes noted this very early in his work.

I hope you and neurologist figure this out together.

[MaryF Administrator in reply to KellyInTexas]

Also called St Vitus' Dance is as lupus-support1 often related to Strep, Prof Hughes also writes about it a fair bit, my own daughter had hints of this after Scarlet Fever when very small: sciencedirect.com/topics/me... MaryF