CASE REPORT

Niger J Paed 2015; 42 (2): 165 –166

Ezenwa BN

Njokanma F

Sydenham’s chorea in a Nigerian

girl: A case report

DOI:http://dx.doi.org/10.4314/njp.v42i2.20

Accepted: 17th February 2015

Abstract: Sydenham chorea is a

major manifestation of Rheumatic

fever (RF). Chorea occurs primar-

ily in children and is rare after the

age of 20 years. Females are more

often affected and it almost never

occurs in post-pubertal males.

Sydenham’s chorea is character-

ized by emotional lability, unco-

ordinated and erratic movements,

and muscular weakness. The

choreiform movements can be a

source of irritation or a severe

functional disability to the individ-

ual requiring appropriate manage-

ment. The rarity of documentation

of SC in sub-Saharan Africa raises

the possibility of missed or misdi-

agnosis. We present the case of a

13 year old girl whose SC was

initially misdiagnosed and con-

clude that though uncommon in

Sub-Saharan Africa, SC requires

proper diagnosis and prompt medi-

cal attention.

Ezenwa BN⁽

)

Department of Paediatrics,

Reddington Hospital, Victoria Island,

Lagos State, Nigeria

Email:beatriceezenwa@yahoo.com;

Njokanma F

Department of Paediatrics,

Lagos State University Teaching

Hospital, Ikeja, Lagos State, Nigeria

Introduction

knee and then the left elbow. There was no history of

joint swellings.

Chorea has been defined as a state of excessive, sponta-

neous movements, irregularly timed, non-repetitive,

randomly distributed and abrupt in character. These

movements may vary in severity from restlessness with

mild intermittent exaggeration of gesture and expres-

sion, fidgeting movements of the hands, unstable dance-

like gait to a continuous flow of disabling, violent move-

Three days before presentation, she developed twitching

and involuntary jerking of her upper limbs and face. The

jerky movements worsened whenever she became agi-

tated and tried to stop them but disappears while asleep.

The abnormal movements made her very anxious and

self-conscious.

She was taken to a hospital where a diagnosis of upper

limb dystonia was made and was commenced on Artane

(Trihexyphenidyl). She was brought to our facility the

next day because the jerking persisted.

ments . Sydenham’s chorea was first described by Tho-

mas Sydenham in 1684 as a clinical syndrome and its

link to rheumatic fever was proposed by Stoll in 1780 .

Since then, Chorea has been recognized as a major

manifestation of acute Rheumatic Fever (ARF) and may

be the only evidence in approximately 20% of cases .

General examination was normal except that she looked

anxious with intermittent, involuntary jerks of both up-

per limbs and facial twitches. The twitches were subtle

and the involuntary jerks consisted of fidgeting move-

ments of the upper limbs which occurred every few min-

utes and were of very low amplitude, though she re-

ported occasional involuntary flinging of her arms. She

was lucid and had age-appropriate mentality. Both hands

were not able to sustain a strong grip (milkmaid hand).

Her knees revealed no signs of inflammation. Both

knees demonstrated crepitus. Her respiratory and cardio-

vascular systems were normal on physical examination.

Full blood count done at presentation showed slight neu-

trophilia(78%) and elevated erythrocyte sedimentation

rate of 43mm/hr (Reference range 0-10mm/hr ). The

throat swab culture yielded no growth but anti-

streptolysin O-titre (ASOT) was elevated at 420IU

(Reference range <200 I.U). Electrocardiogram, echo-

cardiogram and electroencephalogram showed normal

findings. Antineuronal Antibody (ANA) titre was posi-

tive but DNAse B titre was negative.

Chorea occurs primarily in female children. The preva-

lence of chorea in patients with RF varies from 5–36%

in different reports . The incidence in Africa is not pre-

cisely known but one pooled study has put it at 8.8% .

Incidence of ARF is decreasing in almost all the WHO

Regions of the wo₄rld , and as such Sydenham’s chorea

is also decreasing . Currently, younger doctors may

have never seen a patient with rheumatic chorea and

have become unfamiliar with the disease. We report the

case of a 13-year-old girl, who presented in our clinic

with acute onset of abnormal involuntary movements

and whose rheumatic chorea was initially misdiagnosed.

Case report

OA is a 13 year old girl who presented at the Paediatrics

clinic of Reddington Hospital, Lagos, Nigeria with a

three day history of rhythmic and involuntary jerking of

the hands and five months history of joint pains.

She was well until five months previously when she

developed severe pains on her right knee which was

managed with topical analgesics and bandaging. Three

weeks later she experienced similar pains in her left

A diagnosis of Sydenham’s chorea (SC) was made. She

was commenced on Sodium Valproate, Ibuprofen and

Penicillin V. These medications were given respectively

to control the involuntary movements, reduce pain and

inflammation of the joints and eliminate any streptococ-

cal organism remaining in her system. The patient’s

condition improved within 48 hours. By the second

week, the involuntary movements had disappeared. The

patient has resumed normal activities and has been fol-

lowed up in our clinics for about 8 months. No recur-

rence was reported.

disease of adult onset with an unremitting autosomal

dominant movement disorder and dementia; unlike in

Huntington disease, neuroimaging in Sydenham's chorea

is normal and other family members are usually unaf-

fected. Our patient manifested the main features of SC

as listed by WHO viz: involuntary movements, hypoto-

nia, and muscular weakness. Chorea can be generalized

or unilateral . Patients with SC may also have psychiat-

ric symptoms such as depression, anxiety, personality

Discussion

changes, etc Whether the psychological manifestations

are secondary to the movement disorder or an integral

part of the disease is not clear. It is also common for

some patients with SC to present with other manifesta-

Reliable data on the incidence of SC and RF are scarce.

The incidence of SC in Africa is not known but one

pooled study has put it at 8.8% . No known data exists

tions of RF . Our patient had chronic arthralgia but had

for SC in Nigeria but Okoroma et al in Enugu reported

no clinical evidence of carditis. This may not be unusual

as carditis has been documented to occur in only about

it as being rare in their study. The diagnosis of SC de-

pends on a high index of suspicion. Depending on the

experience of the health provider the diagnosis may be

entirely missed due to lack of awareness of the clinical

entity. Our patient presented early to a peripheral hospi-

tal where the diagnosis was missed delaying manage-

ment of her rheumatic chorea.

40-50% of patients with ARF at the time of diagnosis .

Averagely, rheumatic chorea resolves spontaneously

within 3-6 months but may last longer if untreated. With

treatment our patient’s symptoms resolved within two

weeks. Fast resolution with t_,₆r_,e₇atment has also been re-

ported by other researchers . About 20% of patients

Considering the high contribution of RHD to acquired

may experience recurrences and relapses, usually within

2 years after the initial attack and this underscores the

need for adequate follow up.

Treatment of SC is typically limited to supportive care,

palliative medications, to eliminate streptococci and

treat movement disorders, and long-term follow up.

heart disease in Sub-Saharan Africa , the low incidence

of SC appears paradoxical and the explanation is not

clear. Studies of ARF outbreak in the USA during the

990’s attributed SC to highly virulent mucoid strains of

group A Streptococci . Though, it may be attractive to

speculate that the strains of group A Streptoccoci in our

environment are less virulent and therefore have lower

propensity for causing SC, further studies need to be

done on the virulence and immunogenicity of the strains

of group A Streptoccoci in our environment that cause

ARF to establish that.

Conclusion

Sydenham’s chorea is a disabling manifestation of rheu-

matic fever, and its rarity in sub-Saharan Africa may

have contributed to cases being misdiagnosed or even

missed. It requires proper diagnosis as prompt and ap-

propriate medications can bring quick resolution to

symptoms. There is need for continual awareness crea-

tion on manifestations of ARF and its proper manage-

ments.

According to Jones criteria chorea alone is sufficient

for diagnosis of RF provided other causes of chorea had

been excluded. Other possible causes of childhood cho-

rea include cerebrovascular accidents, collagen vascular

diseases, drug intoxication, hyperthyroidism, Wilson's

disease, Huntington's disease, abetalipoproteinemia,

Lesch-Nyhan syndrome and hormonal imbalances .

Conflict of interest: None

Funding: None

Unlike SC, Huntington chorea is generally a

References

Barbeau A, Duvoisin RC, Gersten-

brand F, Lakke JP, Marsden CD,

Stern G. Classification of ex-

trapyramidal disorders. Proposal

for an international classification

and glossary of terms. J Neurol

Sci. 1981;51(2):311-27.

WHO Expert Consultation on

Rheumatic Fever and Rheumatic

Hear Disease. Rheumatic fever

and rheumatic heart disease: report

of a WHO Expert Consultation,

Geneva, 29 Oct – 1 Nov 2001.

Geneva: WHO, 2004 (WHO Tech-

nical Report Series No.923).

3. Bisno A. Non-cardiac manifesta-

7. Walker KG, Wilmshurst JM. An

update on the treatment of Syden-

ham’s chorea: the evidence for

established and evolving interven-

tions. Ther Adv Neurol Disord

2010; 3(5):301-309.

8. Woo CLF, Liu KT,Young BWY.

Acute Rheumatic Fever Presenting

with Sydenham's chorea. HK J

Paediatr (new series) 2003;8:198-

202.

9. Busari O; Opadijo G; Fasae

A.Acute Rheumatic Fever: A Pub-

lic Health Concern in Resource-

Poor Settings Archives Medical

Review Journal 2013; 22(2):153-

169

tions of rheumatic fever. In: Narula

et al., editors. Rheumatic fever.

Washington, DC, American Regis-

try of Pathology, 1994:245–256.

4. Michael DS, Tracey RH. The

worldwide epidemiology of acute

rheumatic fever and rheumatic

heart disease. Clinical Epidemiol-

ogy 2011;3:67–84.

5. Okoroma EO, Ihenacho IN, An-

yanwu CH. Rheumatic fever in

Nigerian children. A prospective

study of 66 patients. Am J Dis

Child. 1981; 135: 236–238.

Carapetis JR, Currie BJ. Rheu-

matic chorea in northern Australia:

a clinical and epidemiological study.

Arch Dis Child 1999; 80:353-8.