One of the major criteria for Rheumatic fever but can sometimes be seen in isolation. See Jelly Jumps page.

Classically, involuntary, non-rhythmic movements, associated with emotional lability. Often misdiagnosed initially eg psychogenic [Mary King, ADC 2015]. Adults can get it rarely – tends to be relapse of childhood disease, female hormones seem to a trigger (eg pregnancy, oral or other contraceptives).

Chorea is a particular kind of movement – varies from smooth writhing (athetosis) to rapid, high amplitude jerks (ballism). Typical signs are repeated pouting of lips, milk maid sign (ask to squeeze fingers in hand), hyperextension of wrists, piano playing movements. Fine motor control usually lost, due to these extra movements. Gait disturbance common, can look like hip hop dancing! Ask to stick tongue out (unable to maintain – “motor impersistence”). Movements disappear in sleep. Can be hard to differentiate sometimes from stereotypies and tics, and of course these things are not uncommon so might co-exist.

Can be one side of the body predominantly (hemichorea). Underlying the involuntary movements is often a loss of tone, which may not become obvious until treatment started to suppress the chorea.

In severe cases, the loss of tone and weakness predominate (chorea paralyticum).

Variable severity. May just be some instability on walking, some difficulty with hand writing. Or unable to walk, talk, feed yourself.

Emotional lability well described, mild anxiety and poor attention less common – although developing a new disability without any cognitive impairment may explain some of it.

Pregnancy is associated with first onset but also relapse!

High risk of cardiac involvement, as type of rheumatic fever – 71% of cases in Turkish study, of which nearly half silent (no findings on clinical examination). Significant risk of long term morbidity, even if chorea never comes back, so always echo and give penicillin prophylaxis.

Diagnosis

Essentially clinical, with supportive evidence of recent streptococcal infection (ASO titre, throat swab). But recognised that infection can be up to 6 months before, or too mild to really notice.

Other tests are directed at differential diagnosis – lumbar puncture, MRI brain etc. Putaminal enlargement described.

Although there is evidence of anti-neuronal antibodies directed against the basal ganglia (eg anti D2R, see Church 2003), these are not specific or sensitive (see Sugar 2003, same time as Church) so not used in clinical practice. “Cunningham panel” is private test, see PANDAS.

Management

There is a UFMG rating scale for SC, from Brazilian Universidade Federal de Minas Gerais (UFMG), for research purposes but only looks at motor function.

Occupational and physiotherapy useful for maintaining function and muscle tone.

Treatment with valproate is effective for controlling symptoms but doesn’t speed up recovery. Haloperidol used previously but prob more side effects. Case reports to support carbamazepine and levetiracetam.

One RCT supporting steroids from Paz, Brazil 2006, 22 cases of SC, remission reduced to 54 days from 119 days. Various other reports of use of oral or IV steroids from Israel, Italy [2012, 2017], Brazil [Cardoso 2005], immunoglobulin [Holland, 2016, South Africa 2016]. Some improvement can be seen within a few days of IV steroids. In Italy, prednisolone reduced average duration of symptoms from 9 weeks to 4 weeks, and these were severe cases [Fusco 2012]. South African group found less neuropsychiatric complications at 6 months with IVIG treatment (IVIG preferred due to fear of TB reactivation). [Review by Deans and Singer, 2017]

A course of penicillin is usually given at diagnosis, to definitively clear any remaining strep but no evidence this really achieves anything and active infection probably long gone. Penicillin prophylaxis, on the other hand, essential if you have other features of rheumatic fever. If isolated chorea, arguable whether cardiac risk justifies or not.

Recurrence

Recurrence seen in 16-40%. More likely if poor compliance with penicillin prophylaxis, of course. Sometimes associated with rise in ASO or other evidence of new streptococcal infection but certainly not always the case. No obvious clinical parameter that might predict those at risk of recurrence. More likely if failure to remit in initial 6 months, can recur with pregnancy.

Higher recurrence rates seen in longest follow up – can recur up to 10 years after the initial episode, so might be underestimated by series with shorter follow up.

Usually recurrence is just chorea, even if you had other features of rheumatic fever to being with. Just two reports of heart disease worsening after recurrence of chorea [Israel and Thailand]. The Thailand study also had 2 cases where carditis, which had improved after initial diagnosis, came back again. Some suggest that perhaps recurrent chorea is a different disease altogether. [Israel, Arch Neurol. 2004; Turkey, PMID 27209549]

Prognosis

10% reported long term tremor in one study (10 years follow up). Long term neuropsychiatric difficulties increasingly recognised (49 studies so far, {Michael Morton and Nadine Mushet 2016 PMID 25926089] esp Obsessive-compulsive disorder but also Attention-deficit-hyperactivity disorder, affective disorders, tic disorders, executive function disturbances, psychotic features, language impairment.

Heart involvement improves in about a third of cases (whether silent or not).[PMID 22734303]

Differential

• PANDAS (Pediatric Autoimmune Neuropsychiatric Disorder Associated with Streptococcal infections) – ICD criteria.
• Tics, Tourettes, stereotypies
• Benign hereditary chorea (BHC) – rare. In infants low muscle tone, chorea, lung infections, and respiratory distress. In older children, delayed motor and walking milestones, myoclonus, dystonia (esp upper limb), motor tics, and vocal tics. The chorea often improves with time, in some cases myoclonus persists or worsens. Some have learning and behaviour problems, thyroid problems and recurring chest infections. Caused by mutations in the NKX2-1 gene (autosomal dominant)
• Bilateral striatal necrosis is a rare condition where similar symptoms but chronic and permanent. Various causes, has been seen in association with streptococcus. Has been described in a case of Sydenham’s where symptoms recurred and then persisted, so not clear whether coincidence or it wasn’t really Sydenham’s in the first place.

Patient/family support at Sydenham’s Chorea Association.

[Review article Oosterveer, NL Ped Neuro 2010]

=inflammation of the meninges. Clinically neck stiffness/pain, headache, photophobia. Almost always vomiting. Usually infective, so usually fever too. Can be viral, bacterial or tuberculosis.

This clinical picture gets confused with the diagnosis of meningococcal disease. Meningococcus (gram negative diplococcus, very distinctive under the microscope) commonly causes meningitis but tends to cause a relatively mild disease with good outcome. It can also cause sepsis that is rapid onset and often fatal, meningitis is rarely a feature of this disease (indeed, having meningitis at the same time is a good prognostic feature).

Diagnosis is by lumbar puncture. Bugs often seen under microscope, which will usually give organism too, else rapid antigen tests available. White cells will be high (often in thousands if bacterial), protein high (can be over 2 if bacterial). Neutrophil predominance suggests bacterial but this is not v reliable esp in babies. Low glucose v suggestive of bacterial.

Can be complicated by raised intracranial pressure and seizures.

Organisms

In neonates, mostly Group B streptococcus, else gram negative bacilli. Listeria can present with sepsis or meningitis in young infants (90% under 30 days).

In older infants and children, mostly meningococcal disease, else pneumococcal or haemophilus. All declining rapidly as a result of immunisation, currently conjugate Hib, PCV-13 and MenACWY plus 4CMenB.

Treatment

Antibiotics to kill bugs. Steroids to reduce damage.

Ceftriaxone is ideal, broad spectrum, good CSF penetration, once daily. But listeria resistant, and gets chelated by calcium so contraindicated if likely HDU/ICU care where calcium infusions often necessary. Also contraindicated in preterm infants under 41/40 corrected, and in neonates esp jaundice, acidosis, hypoalbuminaemia.

For listeria, amoxicillin or ampicillin for 21 days in total, plus gentamicin for at least the first 7 days.

Dexamethasone has been shown to reduce complications eg deafness. Regimen is 0.15 mg/kg (max 10 mg) qds x 4 days. Only given to children ≥ 3 months old. Ideally given before or with first dose antibiotics – NICE says within 12 hours, assuming positive LP viz frankly purulent CSF, or CSF WCC > 1000/μl, or raised CSF WCC and protein > 1 g/L, or bacteria on Gram stain. Steroids should not be used in developing countries.

TB meningitis is a whole different ball game. See NICE NG33 before administering steroids.

Complications

Hydrocephalus, epilepsy, deafness. Particularly seen with Pneumococcal disease.

Recent evidence highlights that meningitis in early childhood is associated with higher depressive and anxiety symptoms, psychological and behavioural problems, and increased risk of psychotic experiences. Not just that, higher risk of ADHD, and lower IQ on average. Follow up therefore very important for young babies, and probably appropriate to warn families.