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Table of Contents
CASE REPORT
Year : 2015  |  Volume : 9  |  Issue : 1  |  Page : 26-29

A 17-year-old of Nigerian girl with Sydenham chorea: A case report and review of literature


1 Department of Medicine, Neurology Unit, Federal Medical Center, Asaba, Delta State, Nigeria
2 Department of Medicine, Usmanu Danfodio University, Teaching Hospital, Sokoto, Sokoto State, Nigeria

Date of Web Publication21-Aug-2015

Correspondence Address:
Emmanuel Okechukwu Ezunu
Department of Medicine, Neurology Unit, Federal Medical Center, Asaba, Delta State
Nigeria
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0331-3131.163332

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   Abstract 

This is a 17-year-old Nigerian girl who had a history of a sore throat for 8 weeks and persistent fever, associated with central chest pain, abnormal fidgety movement of the body and fleeting joint pain. The diagnosis of Sydenham chorea was made due to the additional finding of positive anti-streptolysin O titer. Sydenham chorea is a neuropsychiatric disorder that is characterized by abnormal body movement, associated with mood and behavioral changes. It is an autoimmune disease that occurs following an infection with M serotype of Group A beta-hemolytic streptococcal infection that is prevalent in our environment. However, Sydenham chorea which is one of its manifestations is still rare in our environment, hence the need for literature review.

Keywords: Literature review, nigerian girl, Sydenham chorea


How to cite this article:
Ezunu EO, Njoku CH. A 17-year-old of Nigerian girl with Sydenham chorea: A case report and review of literature. Ann Nigerian Med 2015;9:26-9

How to cite this URL:
Ezunu EO, Njoku CH. A 17-year-old of Nigerian girl with Sydenham chorea: A case report and review of literature. Ann Nigerian Med [serial online] 2015 [cited 2021 Apr 18];9:26-9. Available from: https://www.anmjournal.com/text.asp?2015/9/1/26/163332


   Introduction Top


Acute rheumatic fever (ARF) is an autoimmune disease that occurs approximately 3 weeks after an untreated Group A beta-hemolytic streptococcal sore throat infection. The rate of ARF infection in the developed world is on the decline probably due to their proper antibiotic usage. [1]

Sydenham chorea is a major criterion of ARF, which results from autoimmune disease following infection with Group A beta-hemolytic streptococci. Its M proteins induce production of anti-neuronal antibodies. These cross-react with the body's own cells in the basal ganglia. [2] It occurs as a result of increased dopaminergic activity in the projections from the substantia nigra- to the Striatum; - resulting in decreased Gamma amino butyric acid (GABA) activity from the striatum to the globus pallidus. [3]

Sydenham chorea occurs among patients between the ages of 5 and 15 years, usually 1-6 months after the onset of a sore throat. [1] It may occur in isolation or following other characteristic features of ARF. [3]

It is an abnormal fidgety movement disorder, which is fast, irregular and uncontrollable. This often disappears with sleep, while exacerbations are noticed with stress, fatigue and excitement. In some patients, it resolves spontaneously within few weeks to many years. Re-occurrences are reported in pregnancy and after use of phenytoin and contraceptives [Table 1]. [3]
Table 1: Clinical classification of Sydenham chorea

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The Management of Sydenham Chorea has four main principles; [4] - The use of antibiotics for the elimination of Streptococcal organism, drugs for symptomatic control of the movement disorder, immunological treatment, and supportive cares.

Treatment with penicillin for at least 10 days is needed to eliminate the organism at the dose of 500 mg BID to TID. While penicillin-allergic patients can use erythromycin given at 40 mg/kg/day in 2-4 divided doses. [4] Long-term prophylaxis ensures for the prevention of chronic squeal of Group A streptococcal infection, but whether this holds true for Sydenham chorea is debatable. [5]

In the past, some practices have been tried for symptomatic control of Sydenham chorea such as purging, bleeding, and hyperthermia without success, [6] until recently with increased understanding of the disease process. The use of drugs that decrease the effect of dopamine and enhance the effect of GABA, such as haloperidol and pimozide, have shown to be effective in controlling the movement disorder. Benzodiazepines have also been found to increase the activities of GABA. [4] Carbamazepine may also be used, as it has been found to cause postsynaptic dopaminergic blockade. [7]

Immunomodulatory therapies are given to control inflammation and modulate the progression of the disease as demonstrated by the use of corticosteroids, intravenous immunoglobulins and plasma exchange. [8] Steroids if indicated is needed for cardiac involvement and can be given as prednisolone 2 mg/kg/day for 2 weeks and then tapered.

Management of Sydenham chorea requires a multidisciplinary approach as the illness is often associated with neuropsychiatric manifestations such as personality changes, obsessive-compulsive behavior, and attention deficit hyperactivity disorder. Psychiatric review with supportive psychotherapy is advised. Educational interventions are needed to protect these children from stigmatization in schools and to ensure continual attendance of hospital visits.

With the widespread availability of antibiotics for streptococcal A infection, Sydenham chorea has become rare. We present the first documented case of Sydenham Chorea in the North West of Nigeria.


   Case Report Top


We present a 17-year-old girl, who was admitted to Medical Outpatient Clinic at a University Teaching Hospital in Nigeria. She had 2 months history of sore throat and high grade continuous fever, 4 weeks history of central chest pain, 2 weeks history of marked abnormal movement of the body involving the limbs, the head and neck; with patient being assisted to do most of her routine daily activities. The abnormal movement disappears during sleep, with associated generalized throbbing headache, odynophagia, and fleeting joint pain. No history of the passage of dark stool, palpitation, neck stiffness, ingestion of medication, trauma, malar rash or family history of similar illness. Examination revealed a young girl with rapid uncontrollable jerky movement affecting the limbs, head, and neck. There was no history of loss of consciousness or irrational behavior. There was apical pansystolic murmur radiating to the axillary area, otherwise the cardiovascular system was normal. Abdominal and respiratory systems were not remarkable. The two-dimensional-echo findings showed normal myocardial wall motion. There was mildly dilated left atrium, mildly thickened posterior mitral valve leaflet and diminished valve excursion. No pericardial effusion, no vegetation. Doppler studies revealed mitral regurgitation and mitral stenosis (mild). Chest X-ray showed normal sized heart with mitralization of the left cardiac border and left atrial enlargement with a cardiothoracic ratio of 0.4. Electrocardiogram showed nonspecific ST/T changes, anti-streptolysin O antibody titer was >1/200. Full blood count result revealed a packed cell volume of 33%, white blood cell count of 7.8 × 10 9 /L. Differentials showed neutrophil of 78%, lymphocyte of 15% and monocyte of 7%. The level of platelet count was 308 × 10 9 /L and Fasting blood sugar was 6.3 mmol/l. Serum electrolyte, urinalysis, venereal disease research laboratory, and Widal test were normal.

Based on the presence of chest pain, fleeting joint pain, abnormal jerky, fidgety, purposeless movement of the body and evidence of post-streptococcal infection, the diagnosis of Sydenham chorea was made. The following drugs were commenced-tablets penicillin 500 mg q. d. s., tablets aspirin 600 mg t. d. s., tablets diazepam 10 mg t. d. s., tablets Vitamin E 200 units daily and tablets Vitamin C 200 mg t. d. s. Symptoms resolved within 2 weeks of admission. She was discharged on monthly benzathine penicillin prophylaxis and antioxidants, but she was eventually lost to follow-up.


   Discussion Top


Sydenham chorea is one of the major criteria for ARF. The prevalence of ARF in the developed world is on the decline [3] as compared with developing countries. The incidence rates of first episodes of ARF in indigenous people of the Northern Territory of Australia were highest in 5- to 14-year-old age groups (228 and 162) per 100,000 in girls and boys, respectively.

Aron et al. [9] in 1964 reviewed 50 patients with Sydenham chorea. He followed them up for more than two decades after their first attack; 34% of patients were found to have rheumatic valvular heart disease developing within this period. This finding was low, as it is currently documented that about 50 % of those with Sydenham chorea developed rheumatic heart disease within a 13-year study period. [1] This may not be unconnected with current diagnostic criteria which have solved the problem of misdiagnosis of ARF. [10] This may also explain why ARF may have occurred unnoticed in the past in this same patient. The presence of carditis may explain a recurrence of ARF before onset of chorea. This supports the findings from Fadahunsi, [11] where most of the patients studied presented with carditis with only a few presenting with Sydenham chorea with carditis at recurrence. This may also be due to the fact that patient may not have sought for medical intervention at that time. Jonathan et al. [10] in a study of 108 people with 158 episodes of chorea in Australia over 20 years, documented that average mean age of first presentation of chorea was 10.9 (range 3.9-28.6) years with female preponderance (female:male ratio was 2.7:1). He found out that acute phase reactants were increased in patients with other rheumatic presentation compared with those who had Sydenham chorea only. [12] Our patient had rheumatic manifestations with chorea, but acute phase reactants were not evaluated fully to validate these findings. They also found out that mean anti-streptolysin O titers were substantially (although not significantly) higher in patients with other rheumatic manifestation than those with pure chorea. Though actual levels of anti-streptolysin O titer were not estimated in this study, our patient's chorea resolved earlier than theirs. One girl first chorea lasted for 32 months compared with 2 weeks for our patient. One would have loved to know whether racial differences accounted for these findings. Okoroma et al. [13] after studying 66 Nigerian Children with Rheumatic fever concluded that in 98% of cases, the first presenting major manifestation of ARF is carditis. [13] While joint (polyarthritis, polyarthralgia, and monoarthritis) involvement was observed in 77% of cases as the first presenting symptom in Australia, [1] when compared with carditis 27.5% and Sydenham chorea 19.5%. The broadened criteria of including polyarthritis, polyarthralgia, and monoarthralgia as a major diagnostic criteria may have accounted for the increase. [14] All patients with Sydenham chorea should have secondary antibiotic prophylaxis and followed up for a period of 10 years. This is due to the fact that recurrence rate is highest in the 1 st year and decreases to zero by the 10 th year. [14] However, most patients are lost to follow in this part of the world, like in the index case. This poses a serious challenge in developing countries. Health education is needed in understanding the condition of these children, which will promote tolerance and ensure that they are not unduly exposed to teasing, bullying and other forms of psychological abuse. [4] They should also be encouraged to take their secondary prophylaxis seriously to prevent the chronic sequelae of rheumatic heart disease.

In conclusion, Sydenham chorea predominantly affects females, particularly in adolescence. The condition is rare even in the Northern Nigeria where Group A beta streptococcal infection and its other manifestations is still a problem. There may be racial differences in the presentation which may require further studies. ARF may be missed; therefore, the use of current diagnostic criteria is advised to improve in the detection of the disease. Advocacy for the prevention of ARF is recommended in order to eradicate its chronic sequelae.

 
   References Top

1.
Lawrence JG, Carapetis JR, Griffiths K, Edwards K, Condon JR. Acute rheumatic fever and rheumatic heart disease: Incidence and progression in the Northern Territory of Australia, 1997 to 2010. Circulation 2013;128:492-501.  Back to cited text no. 1
    
2.
Clur SA. Frequency and severity of rheumatic heart disease in the catchment area of Gauteng hospitals, 1993-1995. S Afr Med J 2006; 96:233-7.  Back to cited text no. 2
    
3.
Royden JH, Richard AB, Netter FH. Netter′s Neurolog, Chorea - International Students. Ch. 53. Published by Icon Learning System LLC, a Subsidiary of MediMedia USA, Inc.; 2005. p. 453-9.  Back to cited text no. 3
    
4.
Walker KG, Wilmshurst JM. An update on the treatment of Sydenham′s chorea: The evidence for established and evolving interventions. Ther Adv Neurol Disord 2010;3:301-9.  Back to cited text no. 4
    
5.
Cilliers AM. Rheumatic fever and its management. BMJ 2006;333: 1153-6.  Back to cited text no. 5
    
6.
Gordon N. Sydenham′s Chorea, and its complications affecting the nervous system. Brain Dev 2009;31:11-4.  Back to cited text no. 6
    
7.
Genel F, Arslanoglu S, Uran N, Saylan B. Sydenham′s chorea: Clinical findings and comparison of the efficacies of sodium valproate and carbamazepine regimens. Brain Dev 2002;24:73-6.  Back to cited text no. 7
    
8.
Garvey MA, Snider LA, Leitman SF, Werden R, Swedo SE. Treatment of Sydenham′s chorea with intravenous immunoglobulin, plasma exchange, or prednisone. J Child Neurol 2005;20:424-9.  Back to cited text no. 8
    
9.
Aron AM, Freeman JM, Cater S. The natural history of Sydenham chorea; review of literature and long term evaluation with emphasis on cardiac sequel. Am J Med 1964;38:83-95.  Back to cited text no. 9
    
10.
Jonathan CR, Alex B, Graeme M, Warren W, Marc R, Remenyi B, et al. The Australian guideline for prevention, diagnosis and management of acute rheumatic fever and rheumatic heart disease. Full guidelines. 2 nd ed. RHD Australia (ARF/RHD Writing group), National Heart Foundation of Australia and the Cardiac Society of Australia and New Zealand: Menzies School of Health Research; 2012.  Back to cited text no. 10
    
11.
Fadahunsi HO. Rheumatic fever in Nigerian children: A prospective study. Ann Trop Paediatr 1981;1:115-8.  Back to cited text no. 11
    
12.
Carapetis JR, Currie BJ. Rheumatic chorea in northern Australia: A clinical and epidemiological study. Arch Dis Child 1999;80:353-8.  Back to cited text no. 12
    
13.
Okoroma EO, Ihenacho IN, Anyanwu CH. Rheumatic fever in Nigerian children. A prospective study of 66 patients. Am J Dis Child 1981;135:236-8.  Back to cited text no. 13
    
14.
Ider LA, Leitman SF, Werden R, Swedo SE. Treatment of Sydenham′s chorea with intravenous immunoglobulins, plasma exchange, or prednisone. J Child Neurol 2005;20:424-9.  Back to cited text no. 14
    



 
 
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