Chorea

Following Acute Glomerulonephritis

James

Springate, M.D. Anthony Vetrano, M.D. Steven Cachero, M.D. Vijayan Menon, M.D. Leonard Feld, M.D., Ph.D.

Introduction

Examination revealed a mildly ill-appearing female child who was

rheumatogenic and nephritogenic properties of group A streptococci are generally considered distinct and mutually exclusive. We describe a child who developed Sydenham’s

afebrile and normotensive. No specific physical abnormalities were found. Her urine was tea-colored and dipstick-positive for blood and

chorea two months after an illness consistent with acute post-streptococcal glomerulonephritis.

blood cells and red blood cell casts. A complete blood count and serum electrolyte concentrations were normal. Serum urea nitrogen and creatinine concentrations were 32 mg/dL and 0.9 mg/dL, respectively. An accurate 24-hour urine collection specimen showed a creatinine clearance of 57 mL/min/1.73 m2 and a protein excretion of 263 mg/day. Her erythrocyte sedimentation rate (ESR) (Westergren) was 114 mm/hr; antistreptolysin-O titer, 1:250 Todd units; serum C3 concentration, 31 mg/dL (normal, 90 to 200 mg/dL); and serum C4 concentration, 7 mg/dL (normal, 16 to 39 mg/dL) . A throat culture did not grow group A streptococci. Her

he

Case

Report

This 5-year-old girl presented with a one-week history of increasing malaise, intermittent abdominal pain, and dysuria. Two days before presentation, amoxicillin had been prescribed for a suspected urinary tract infection; the urine culture was subsequently found to be sterile. Persistence of symptoms and the development of gross hematuria prompted her return to medical attention. Her past medical and family history and a review of systems were otherwise unremarkable. She reportedly had no recent throat or skin infections.

From the Department of Pediatrics, State University of New York at Buffalo, and The Children’s Hospital, Buffalo, New York. Address correspondence to: Leonard Feld, M.D., Ph.D., Chief, Division of Nephrology, The Children’s Hospital, 219 Bryant Street, Buffalo, New York 14222

632

protein. Microscopic urinalysis showed numerous dysmorphic red

hospital course was complicated by transient and hypertension oliguria, which responded to fluid restriction and furosemide. She spontaneously improved and was discharged one week later with a diagnosis of probable post-streptococcal glomerulonephritis. No medications were prescribed. When seen in follow-up two weeks later, she had no complaints, and results of her examination were normal. Seven weeks after her

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first hospital admission, she presented with complaints of increasing restlessness, difficulty in walking, and poor handwriting. She showed marked involuntary choreiform movements and a holosystolic apical heart murmur, grade 2/6 in intensity. The remain-

der of her examination was normal. Urinalysis revealed yellow color with small blood and no protein by dipstick. Her ESR was 25 mm/hr, and her antistreptolysin-O titer was 1:100 Todd units. Her total hemolytic

complement activity (CH50) was normal, and serologic tests for systemic lupus erythematosus (SLE) were negative. Echocardiography revealed mitral regurgitation; an electrocardiogram was normal. Her throat culture again grew no group A streptococci. She received phenobarbital and penicillin, improved rapidly, and was discharged with the diagnoses of chorea and mitral insufficiency secondary to rheumatic fever and resolving poststreptococcal glomerulonephritis. One month later, she appeared healthy and had no complaints. Serologic tests for SLE were again negative; serum C3 and C4 connormal. Phenostopped, with no recurrent chorea. Her follow-up now extends over a period of three years. She remains in good health and continues to receive penicillin prophylaxis. Her blood pressure, serum creatinine concentration, centrations

barbital

were

was

and urinalysis results are normal. Her heart murmur is no longer audible, but echocardiography still demonstrates slight mitral

regurgitation.

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Discussion

diseases: glomerulonephritis and rheumatic fever. Her episode of

We believe this child’s unusual is best explained by the coexistence of two post-streptococcal

hypocomplementemic glomerulonephritis resolved spontaneously and was not accompanied by

course

acute

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633

evidence of other organ systemic involvement. Although group A

streptococcus

was

not

isolated, her

antistreptolysin-O titer was initially elevated and subsequently declined, suggesting a recent streptococcal infection. A renal biopsy would have helped confirm this diagnosis but is not standard practice in this setting. Approximately two months after her acute nephritic episode, she developed chorea. Of possible etiologies, only Sydenham’s chorea and lupus-associated chorea seemed tenable.2 Chorea is a rare but well-documented complication of SLE, and it can precede the appearance of other clinical or laboratory evidence for this disease.3 Given the self-limited nature of this child’s nephritis and chorea, her normal serologic test results, and the extended period of asymptomatic follow-up, we feel that SLE is unlikely. In contrast, the association of chorea and mitral insufficiency is highly suggestive of rheumatic fever. 2,4

phritis, followed by a relatively long latency period before the appearance of the chorea. This delay is typical of Sydenham’s chorea, which, unlike other manifestations of rheumatic fever, usually develops

Although glomerulonephritis

several months after streptococcal infection. 2,1 We also regret that, as in other published cases, no organism was available for further study. Analysis of bacterial antigens and host responses in these unusual patients would undoubtedly enhance our understanding of poststreptococcal sequelae. The recent resurgence of rheumatic fever, coupled with closer attention to the possibility of coexistent post-

has been documented in many patients with rheumatic fever, the coexistence of classic, acute post-

streptococcal nephritis, may provide an opportunity to investigate the pathogenesis of these two dis-

streptococcal glomerulonephritis

orders in

and rheumatic fever is considered unusual.’ However, the cases of at least six other children with this association have been reported

634

The clinical features of these patients are summarized in Table 1. Our patient is the only child with chorea as the sole presenting symptom of rheumatic fever. We cannot definitely determine that a single group A streptococcal infection caused both nephritis and rheumatic fever in this child. If this was the case, she presumably had an asymptomatic pharyngitis one to two weeks before her ne-

more

detail.

rheumatic fever and acute Arthritis lonephritis.

2.

3.

4.

Fenichel GM. Clinical Pediatric Neurology. Philadelphia, PA: WB Saunders; 1988:286-291. Arisaka O, Obinata K, Sasaki H, Kaneko K. Chorea as the initial manifestation of

systemic lupus erythematosus. Clin Pediatr. 1984;23:298-300. Ayoub EM, Chun CSY Nonsuppurative complications of group A streptococcal infection. Adv Pediatr Infect Dis. 1990;5:69-92.

5.

Gibney R, Reineck HJ, Bannagan GA, Stein JH. Renal lesions in acute rheumatic

fever.

Ann

6.

Coincidence of acute rheumatic fever and acute post-streptococcal glomeruJ Rheumatol. 1985;12:587lonephritis. 589.

7.

Said R, Hussein M, Hassan A. Simultaneous occurrence of acute post-streptococcal glomerulonephritis and

rheumatic fever. Am J

acute

8.

1986;6:146-148. Kwong YL, Chan KW,

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Nephrol.

Chan MK.

Acute

post-streptococcal glomerulonephritis followed shortly by acute rheumatic fever. Postgrad Med J. 1987;63:209-210.

9.

Doshi H, Brick JE. Rheufever and post-streptococcal

Kujala GA, matic

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Castillejos G, Padilla L, Lerma A, et al.

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