Chorea minor

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Classification according to ICD-10
I02.0 Rheumatic chorea with heart involvement
I02.9 Rheumatic chorea without heart involvement
ICD-10 online (WHO version 2019)

The chorea minor (Sydenham) is one with hyperkinesia (uncontrollable flash outgoing movement) of the hands, throat and facial muscles and simultaneous hypotonia (muscle weakness) and hyporeflexia (weakening of reflexes) associated neurological disease.

It is a possible form of manifestation of rheumatic fever (see Jones criteria ), hence the synonyms chorea rheumatica and chorea infectiosa .

It occurs as a secondary disease a few weeks to months after an infection with β-haemolytic group A streptococci , mostly after a tonsillitis ( angina tonsillaris ) or pharyngitis , scarlet fever or erysipelas .

It mainly affects girls between the ages of six and 13. In very rare cases, adults up to 40 years of age can also be affected.

Thomas Sydenham

Historical

The English doctor Thomas Sydenham (1624–1689) is considered to be the first to describe this disease. About 200 years later, the New York physician George Huntington (1850-1916) discovered a non-infectious hereditary variant of chorea (also known as "St. Vitus's Dance"), which occurs mainly in older people over 50 years of age, and delimited it as chorea major .

root cause

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It is an autoimmune disease in which antibodies - which were originally formed against the streptococci - cross-react and suddenly attack certain endogenous cells of the basal ganglia in the brain that are responsible for the inhibitory dosage and fine-tuning of movements. Essentially, the striatum is damaged, but the striatum is not irreversibly destroyed, as is the case with Huntington's disease.

The same mechanism is also the basis of rheumatic endocarditis (also a possible form of manifestation of rheumatic fever), with the only difference that in this case the antibodies are not directed against the basal ganglion cells, but against tropomyosin and myosin, i.e. against components of the heart muscles.

This then leads to inflammatory reactions with impairment of the functionality of these movement-inhibiting basal ganglia. The movement-promoting basal ganglia ( substantia nigra and pallidum ) are now partially disinhibited, and the typical excessive movement patterns occur.

The clinical appearance is logically more pronounced the more cells are damaged and the greater the resulting inflammation has become.

In Parkinson's disease , on the other hand, the cells of a movement-promoting basal ganglion ( substantia nigra ) perish, which leads to the triad of akinesia (lack of movement), rigor (muscle stiffness) and resting tremor (muscle tremors) paired with hypertension and hyperreflexia of the muscles; thus to symptoms that are exactly the opposite of those of chorea.

Chorea and Parkinson's disease are both based on damage to or destruction of one of the two antagonizing basal ganglia. Their complex, coordinated interaction of mutual inhibition and reinforcement is then disturbed and there is either too much or too little movement. The two diseases are, so to speak, the two sides of the same coin.

Definition of the term chorea

Choreatic movement disorders belong to the large group of extrapyramidal hyperkinesias , to which u. a. also the tremor , the dystonia , the ballism or the tics in Tourette's syndrome belong. All extrapyramidal hyperkinesias are based on a malfunction of certain parts of the basal ganglia.

The Chorea (Greek for dance) is not a disease, but a purely descriptive term for a symptom, the number of very different causes may underlie. The end result, however, is always a functional disorder of the striatum, which then leads to very characteristic movement disorders . So there is:

Symptoms

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The neurological symptoms are similar to those of the hereditary disease chorea major (Huntington's).

  • Hyperkinesia (= short-term uncontrollable + uncoordinated lightning-like muscle twitches) of the distal (= body remote) extremities. (especially the hands → fast moving movements):
    • the children become increasingly noticeable in movements of the hands that require precise coordination of antagonizing muscle groups
      • at school they stand out for their deteriorating typeface. At the beginning this can be misinterpreted by teachers as a kind of childish defiant reaction or displeasure.
      • At home they are noticeable for their unusually frequent "clumsiness", in that they drop objects more often than before, suddenly cannot handle the cutlery properly, etc.
    • the " facial muscles " (grimacing)
    • the " throat muscles " (difficulty speaking and swallowing due to uncoordinated muscle twitching → The patients then have a clenched sounding, choppy way of speaking ( dysarthria ). They can choke more often ( dysphagia ) with the risk of aspiration pneumonia .)
    • and the " tongue muscles " ("chameleon tongue " or "flycatcher tongue" → involuntary stretching out and rapid retraction of the tongue).

Something similar happens with another disease, the so-called ballism , but here coarse, worm-shaped movements of the proximal (= body-close) muscle groups, ie the shoulder and upper arm muscles, are typical. The main difference is that now it is not the striatum but the second important basal ganglion (the nucleus subthalamicus ) that prevents movement . Apparently, the striatum and the subthalamic nucleus divide the muscles in the sense of near and far from the body. This then results in different clinical pictures, which is of course very helpful in diagnosis.

The movements increase with emotional stress or in stressful situations and decrease again with relaxation. They are not even there in sleep.
The little patients find them very stressful and embarrassing, which is why they try to cover them up as well as possible. They also succeed in doing this in part by incorporating the hyperkinesia into seemingly normal voluntary movements (e.g. by stroking their hair, wetting their lips with their tongue, or by dancing their hands and smiling ).
  • Muscle hypotension (= weakness of the muscles) and hyporeflexia. (weakened muscle reflexes):
At the same time, there is also muscle weakness with hyporeflexia, which can be diagnosed using certain clinical examination methods (strength and reflex tests). In severe cases, this makes standing or walking impossible.
  • Mental disorders :
    • such as attention disorders , tiredness and apathy at school,
    • but also restlessness and irritability .
    • Also psychosis are described, but these are very rare.

"St. Vitus Dance" (Chorea Sancti Viti)

St. Vitus - emergency helper for cramps, epilepsy, rabies, St. Vitus dance, bed-wetting

In the Middle Ages, patients with chorea (Greek for dance) were thought to be “ obsessed with dance madness” due to their symptoms . Since at that time there was no medical explanation for this "strange behavior" of the sick, one believed in an obsession with an evil spirit .

In fact, one could have got the impression that someone would take control of the affected person's muscles for a few seconds (up to minutes) and play jokes with them “at will”. The patients could have felt “remote-controlled”, so to speak.

The St. Vitus (St. Vitus) healed in the 4th century the son of the Roman emperor Diocletian from such an obsession. Since Diocletian had him executed afterwards anyway (because he did not want to renounce the Christian faith), and he is said to have worked many other miracles beforehand , he was later canonized. Since then he has been considered a patron saint of Catholics - more precisely he is one of the so-called Fourteen Helpers in Need - who is still v. a. in the case of neurological diseases such as epilepsy , cramps or St. Vitus's dance. Especially in the Middle Ages, those affected prayed to him and made a pilgrimage to the place where his bones were kept in order to find redemption.

For this reason, the names "were for the chorea chorea " and chorea sancti viti (= "Dance of St. Vitus") use.

Diagnosis

The diagnosis can be made based on the following features:

Since the minor chorea is only a manifestation of the rheumatic fever, one must also look for any other signs of the rheumatic events that may be present. In principle, all of the other Jones criteria can also be present.

therapy

The therapy is the same as for rheumatic fever:

  • Acute therapy:
    • bed rest
    • high-dose penicillin administration over 10 days to eliminate the remaining streptococci, which further maintain the autoimmune process.
The tried and tested penicillin is completely sufficient for group A streptococci, since no resistance has so far occurred. In case of penicillin intolerance (allergies, ...) you can switch to erythromycin .
  • Anti-inflammatory:
    • Cortisone for a few weeks to combat the inflammatory reactions in the brain tissue caused by the antibody reaction. (anti-rheumatic therapy)
    • Salicylates such as B. Aspirin for 6-12 weeks
This is the crucial step in therapy. Only under the anti-inflammatory therapy do the choreic symptoms slowly decrease.
  • Therapy of psychological symptoms: if necessary
    • by creating a calm environment,
    • with sedatives (= tranquilizers ), such as B. Diazepam (→ Since the symptoms increase when excited, calming medication is sometimes very helpful!),
    • or in the very rare case of a psychosis with neuroleptics such. B. Haloperidol .

prophylaxis

  • Relapse prevention:
    • Benzathine penicillin G (intramuscularly injected depot penicillin with a half-life of 30 days)
This lower-dosed monthly penicillin is usually given over a period of 5 years to avoid a relapse as far as possible. With involvement of the heart valves (rheumatic endocarditis) also longer, mostly up to adulthood.
The benefit of prophylaxis is indisputable, since without penicillin prophylaxis, recurrences can occur in up to 50% of cases, but the duration of treatment is not entirely undisputed. The views range from the administration only in special hazardous situations to the administration within a period of 5 years to lifelong penicillin prophylaxis.
  • Elimination / remediation of the origin of the streptococcal infection:
It is very important to find a possibly existing source of chronic streptococcal infection (e.g. carious teeth, enlarged tonsils) and to eliminate it (dental restoration, tonsillectomy ), as otherwise there may be another relapse . It then does not have to manifest itself again as chorea, but it could e.g. B. also show as a very dangerous rheumatic endocarditis (heart valve inflammation) or polyarthritis (see Jones criteria ).

forecast

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The therapy and especially the prognosis of chorea minor and major differ considerably from one another.

  • Huntington's disease is currently incurable. The symptoms continue to increase, the patients lose control of their muscles and they become bedridden. Years later, the disease always ends with the death of the patient - often due to respiratory insufficiency (respiratory paralysis) or severe aspiration pneumonia .
  • In contrast, with appropriate therapy (see above), the minor chorea heals completely after about eight to twelve weeks in 90% of cases; later relapses are possible despite prophylaxis.

Comparison of minor chorea (Sydenham) - major chorea (Huntington)

Chorea minor (Sydenham) Chorea major (Huntington's)
Age Children aged 3–13,
rarely up to 40 years 		3–75 years, mostly from 30 years
gender predominantly female
root cause Autoimmune disease after past streptococcal infection Hereditary disease
autosomal dominant (short arm of chromosome 4)
Pathogenesis Cross-reacting antibodies against proteins of the basal ganglia (especially striatum) Deposition of non-physiological proteins in the basal ganglia (especially the striatum)
therapy Treatment of the cause (infection) with
penicillin G for 10 days of
recurrence prevention 		no causal treatment possible
neuroleptics (tiaprid) against the hyperkinesia
genetic counseling, since 50% of children will become ill
Complications residual disorders remain in 10% of cases almost always dementia in the later stages of the disease. (may be absent in the rare late onset of the disease (> 50))
forecast reversible
heals without consequences in 90% of cases 		irreversibly
chronically progressive, usually ends fatally after 12-15 years

See also

Older literature

  • Ludwig Heilmeyer , Wolfgang Müller: The rheumatic diseases. In: Ludwig Heilmeyer (ed.): Textbook of internal medicine. Springer-Verlag, Berlin / Göttingen / Heidelberg 1955; 2nd edition ibid. 1961, pp. 309–351, here: p. 317: Die Chorea minor ( St. Vitus Dance ).

Individual evidence

  1. ↑ Thomas Sydenham, in: Schedula monitoria de Novae febris ingressu. 1686.
  2. ↑ Gerd Herold and colleagues: Internal Medicine 2020. Self-published, Cologne 2020, ISBN 978-3-9814660-9-6 , p. 164.