Njp Volume 42 No 2

Niger J Paed 2015; 42 (2): 165 –166 CASE REPORT Ezenwa BN Sydenham’s chorea in a Nigerian Njokanma F girl: A case report DOI:http://dx.doi.org/10.4314/njp.v42i2.20 Accepted: 17th February 2015 Abstract: Sydenham chorea is a functional disability to the individ- major manifestation of Rheumatic ual requiring appropriate manage- Ezenwa BN ( ) fever (RF). Chorea occurs primar- ment. The rarity of documentation Department of Paediatrics, ily in children and is rare after the of SC in sub-Saharan Africa raises Reddington Hospital, Victoria Island, age of 20 years. Females are more the possibility of missed or misdi- Lagos State, Nigeria Email:[email protected]; often affected and it almost never agnosis. We present the case of a occurs in post-pubertal males. 13 year old girl whose SC was Njokanma F Sydenham’s chorea is character- initially misdiagnosed and con- Department of Paediatrics, ized by emotional lability, unco- clude that though uncommon in Lagos State University Teaching ordinated and erratic movements, Sub-Saharan Africa, SC requires Hospital, Ikeja, Lagos State, Nigeria and muscular weakness. The proper diagnosis and prompt medi- choreiform movements can be a cal attention. source of irritation or a severe Introduction knee and then the left elbow. There was no history of joint swellings. Chorea has been defined as a state of excessive, sponta- Three days before presentation, she developed twitching neous movements, irregularly timed, non-repetitive, and involuntary jerking of her upper limbs and face. The randomly distributed and abrupt in character. These jerky movements worsened whenever she became agi- movements may vary in severity from restlessness with tated and tried to stop them but disappears while asleep. mild intermittent exaggeration of gesture and expres- The abnormal movements made her very anxious and sion, fidgeting movements of the hands, unstable dance- self-conscious. like gait to a continuous flow of disabling, violent move- She was taken to a hospital where a diagnosis of upper ments 1. Sydenham’s chorea was first described by Tho- limb dystonia was made and was commenced on Artane mas Sydenham in 1684 as a clinical syndrome 2 and its (Trihexyphenidyl). She was brought to our facility the link to rheumatic fever was proposed by Stoll in 1780 2. next day because the jerking persisted. Since then, Chorea has been recognized as a major manifestation of acute Rheumatic Fever (ARF) and may General examination was normal except that she looked be the only evidence in approximately 20% of cases 2. anxious with intermittent, involuntary jerks of both up- Chorea occurs primarily in female children. The preva- per limbs and facial twitches. The twitches were subtle lence of chorea in patients with RF varies from 5–36% and the involuntary jerks consisted of fidgeting move- in different reports 3. The incidence in Africa is not pre- ments of the upper limbs which occurred every few min- cisely known but one pooled study has put it at 8.8% 4. utes and were of very low amplitude, though she re- Incidence of ARF is decreasing in almost all the WHO ported occasional involuntary flinging of her arms. She Regions of the world 4, and as such Sydenham’s chorea was lucid and had age-appropriate mentality. Both hands is also decreasing 4. Currently, younger doctors may were not able to sustain a strong grip (milkmaid hand). have never seen a patient with rheumatic chorea and Her knees revealed no signs of inflammation. Both have become unfamiliar with the disease. We report the knees demonstrated crepitus. Her respiratory and cardio- case of a 13-year-old girl, who presented in our clinic vascular systems were normal on physical examination. with acute onset of abnormal involuntary movements Full blood count done at presentation showed slight neu- and whose rheumatic chorea was initially misdiagnosed. trophilia(78%) and elevated erythrocyte sedimentation rate of 43mm/hr (Reference range 0-10mm/hr ). The Case report throat swab culture yielded no growth but anti- streptolysin O-titre (ASOT) was elevated at 420IU OA is a 13 year old girl who presented at the Paediatrics (Reference range <200 I.U). Electrocardiogram, echo- clinic of Reddington Hospital, Lagos, Nigeria with a cardiogram and electroencephalogram showed normal three day history of rhythmic and involuntary jerking of findings. Antineuronal Antibody (ANA) titre was posi- the hands and five months history of joint pains. tive but DNAse B titre was negative. She was well until five months previously when she A diagnosis of Sydenham’s chorea (SC) was made. She developed severe pains on her right knee which was was commenced on Sodium Valproate, Ibuprofen and managed with topical analgesics and bandaging. Three Penicillin V. These medications were given respectively weeks later she experienced similar pains in her left to control the involuntary movements, reduce pain and 166 inflammation of the joints and eliminate any streptococ- disease of adult onset with an unremitting autosomal cal organism remaining in her system. The patient’s dominant movement disorder and dementia; unlike in condition improved within 48 hours. By the second Huntington disease, neuroimaging in Sydenham's chorea week, the involuntary movements had disappeared. The is normal and other family members are usually unaf- patient has resumed normal activities and has been fol- fected. Our patient manifested the main features of SC lowed up in our clinics for about 8 months. No recur- as listed by WHO 2 viz: involuntary movements, hypoto- rence was reported. nia, and muscular weakness. Chorea can be generalized or unilateral 2. Patients with SC may also have psychiat- ric symptoms such as depression, anxiety, personality Discussion changes, etc 7 Whether the psychological manifestations are secondary to the movement disorder or an integral Reliable data on the incidence of SC and RF are scarce. part of the disease is not clear. It is also common for The incidence of SC in Africa is not known but one some patients with SC to present with other manifesta- pooled study has put it at 8.8% 4. No known data exists tions of RF 2. Our patient had chronic arthralgia but had for SC in Nigeria but Okoroma et al 5 in Enugu reported no clinical evidence of carditis. This may not be unusual it as being rare in their study. The diagnosis of SC de- as carditis has been documented to occur in only about pends on a high index of suspicion. Depending on the 40-50% of patients with ARF at the time of diagnosis 9. experience of the health provider the diagnosis may be Averagely, rheumatic chorea resolves spontaneously entirely missed due to lack of awareness of the clinical within 3-6 months but may last longer if untreated. With entity. Our patient presented early to a peripheral hospi- treatment our patient’s symptoms resolved within two tal where the diagnosis was missed delaying manage- weeks. Fast resolution with treatment has also been re- ment of her rheumatic chorea. ported by other researchers 4,6,7 . About 20% of patients Considering the high contribution of RHD to acquired may experience recurrences and relapses, usually within heart disease in Sub-Saharan Africa 6,7 , the low incidence 2 years after the initial attack 7 and this underscores the of SC appears paradoxical and the explanation is not need for adequate follow up. clear. Studies of ARF outbreak in the USA during the Treatment of SC is typically limited to supportive care, 1990’s attributed SC to highly virulent mucoid strains of palliative medications, to eliminate streptococci and group A Streptococci 8. Though, it may be attractive to treat movement disorders, and long-term follow up. 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 Conclusion done on the virulence and immunogenicity of the strains of group A Streptoccoci in our environment that cause Sydenham’s chorea is a disabling manifestation of rheu- ARF to establish that. matic fever, and its rarity in sub-Saharan Africa may have contributed to cases being misdiagnosed or even According to Jones criteria 4 chorea alone is sufficient missed. It requires proper diagnosis as prompt and ap- for diagnosis of RF 4 provided other causes of chorea had propriate medications can bring quick resolution to been excluded. Other possible causes of childhood cho- symptoms. There is need for continual awareness crea- rea include cerebrovascular accidents, collagen vascular tion on manifestations of ARF and its proper manage- diseases, drug intoxication, hyperthyroidism, Wilson's ments. disease, Huntington's disease, abetalipoproteinemia, Lesch-Nyhan syndrome and hormonal imbalances 2. Conflict of interest: None Unlike SC, Huntington chorea is generally a Funding : None References 1. Barbeau A, Duvoisin RC, Gersten- 3. Bisno A. Non-cardiac manifesta- 7. Walker KG, Wilmshurst JM. An brand F, Lakke JP, Marsden CD, tions of rheumatic fever. In: Narula update on the treatment of Syden- Stern G. Classification of ex- et al., editors. Rheumatic fever. ham’s chorea: the evidence for trapyramidal disorders. Proposal Washington, DC, American Regis- established and evolving interven- for an international classification try of Pathology, 1994:245–256. tions. Ther Adv Neurol Disord and glossary of terms. J Neurol 4. Michael DS, Tracey RH. The 2010; 3(5):301-309. Sci . 1981;51(2):311-27. worldwide epidemiology of acute 8. Woo CLF, Liu KT,Young BWY . 2. WHO Expert Consultation on rheumatic fever and rheumatic Acute Rheumatic Fever Presenting Rheumatic Fever and Rheumatic heart disease. Clinical Epidemiol- with Sydenham's chorea. HK J Hear Disease. Rheumatic fever ogy 2011;3:67–84. Paediatr (new series) 2003;8:198- and rheumatic heart disease: report 5.

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