An 18-Year-Old Female Was Admitted to the Hospital With Uncontrollable Writhing Movements

Introduction

An 18-year-old female was admitted to the hospital with uncontrollable writhing movements.

HISTORY OF PRESENT ILLNESS: Three weeks prior to admission, the patient began experiencing bilateral knee pain. She also had "biting" abdominal pain and a sensation of "stinging nettles" in the neck. Abnormal movements of the extremities and torso then ensued. The family described bizarre mental behavior. For the 3 days before admission, the patient had been unable to speak or feed. Fever and cough were also reported.

The patient had visited an outside institution, where she was treated for malaria, typhoid, and gonorrhea.

REVIEW OF SYSTEMS: Several weeks previous to the onset of symptoms, the patient had suffered from a sore throat.

PAST MEDICAL HISTORY: None

MEDICATIONS: None

FAMILY HISTORY: No similar illnesses

SOCIAL HISTORY: The patient lives in a rural Kenyan Maasai community. She is married without children. She does not use tobacco or alcohol. There was no history of animal bites.

PHYSICAL EXAMINATION: The vital signs were blood pressure 110/70 mm Hg, pulse 80/minute, respiratory rate 20/minute, temperature 37.5° Celsius, and oxygen saturation 90% breathing ambient air. Most striking was the presence of severe choreiform movements of the whole body Video 1. She was coughing frequently. The oropharynx was clear without tonsillar enlargement or thrush. The heart displayed a regular rate and rhythm without murmurs, rubs, or gallops. The lungs were clear to auscultation. Abdominal examination revealed no tenderness or organomegaly. There was no rash, nodules, edema, joint swelling, or evidence of active arthritis. The patient was awake, alert, and attempted to follow simple commands but was unable to speak. When asked for a verbal reply, she simply did not respond. The cranial nerves appeared grossly intact. Strength, sensation, and reflexes could not be formally evaluated due to the chorea, but no gross abnormalities were detected.

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LABORATORY EVALUATION: Hemoglobin 17.1, hematocrit 51.9, white blood cell count 10,600 with 67% polymorphonuclear cells and 33% lymphocytes, platelet count 454,000, and MCV 88.8. A smear for malaria parasites was negative, as was a VDRL. The erythrocyte sedimentation rate was 43. A chest x-ray was normal.

What is the most likely diagnosis? What is the next step in management?

Diagnosis

The combination of a preceding sore throat, significant joint pain, and choreiform movements in a rural Kenyan patient strongly suggests the diagnosis of acute rheumatic fever.

Although every American medical student is required to memorize the classic Jones criteria for the diagnosis of rheumatic fever, today this disease remains a problem predominantly outside the West. In the tropics, rheumatic heart disease, a sequela of rheumatic fever, is the most common cause of acquired cardiac ailments.[1] An estimated 10-20 million new cases per year occur in the developing world.[2] The "molecular mimicry" mechanism of rheumatic carditis -- cross-reaction of antibodies directed against group A streptococcus with cardiac myosin -- is also well-known. Only streptococcal pharyngeal infection leads to this complication. Prompt antibiotic therapy, while not shortening the duration of upper respiratory symptoms, does abort the onset of the autoimmune process.

The clinical manifestations and diagnosis of rheumatic fever are closely related. The clinical presence of 2 major or 1 major and 2 minor Jones criteria, along with evidence of recent streptococcal infection, is required to establish the diagnosis.[3]

The 5 major Jones criteria are:

1. Arthritis: Most typically, the leg joints are involved first, followed by other large joints such as the elbows and wrists. This polyarthritis is said to "migrate" as the pain moves from joint to joint. Often, arthralgia is present rather than frank arthritis. Aspiration of the affected joint reveals inflammatory synovial fluid.

2. Carditis: Pancarditis may present with chest pain and symptoms of congestive heart failure. Chest radiography often shows cardiac dilatation. Mitral regurgitation (the Carey-Coombs murmur) is the most common valvular lesion.

3. Subcutaneous nodules: These lesions usually only appear in patients who also experience carditis. The nodules are firm, painless, and mostly overly bony prominences. Numbering anywhere from 1 to several dozen, the lesions usually resolve in 1 to 2 weeks.

4. Erythema marginatum: This manifestation, usually occurring early in the disease course, is named for the observation that the rash appears to be spreading outward as the central skin returns to normal. It is faint and pink, most often found on the trunk.

5. Sydenham chorea: Also know as "St. Vitus' dance," this disorder can occur months after the onset of rheumatic fever, and relapses years later are not unheard of. It is the most common form of childhood chorea. The pathophysiology may be similar to the molecular mimicry of rheumatic carditis, with cross-reacting antibodies attacking structures in the basal ganglia and caudate nucleus. The presence of antineuronal antibodies correlates with disease activity, a finding that has implications for therapy.

The uncontrollable and nonrhythmic movements may be accompanied by psychological disturbances, such as labile mood and psychosis. The patient may have been nonverbal secondary to these psychiatric effects (although she could physically attempt to follow commands) or because of the frequent tongue and facial twitching.

Chorea occurs as a consequence of acute rheumatic fever more often in children, but episodes do occur among adults. Females are more commonly affected than males. Although this patient is somewhat older than the typical case of Sydenham chorea, Kijabe Hospital's status as a referral institution serving a very wide region means atypical presentations of common diseases are encountered frequently. It is said that in Africa, when you hear hoof beats, think of zebras.

The 3 minor Jones criteria include:

1. Arthralgia: Bilateral knee pain without obvious arthritis was present in this case. The elevated erythrocyte sedimentation rate pointed to an inflammatory state.

2. Fever: The family reported a history of fever, and the initial axillary temperature was 37.5° Celsius.

3. Previous rheumatic fever or rheumatic heart disease.

In this case, 1 major (chorea) and 2 minor (arthralgia and fever) Jones criteria were present. However, formal diagnosis of rheumatic fever also requires evidence of preceding streptococcal infection, such as rising antistreptolysin O (ASO) titers, positive throat culture, or recent scarlet fever. ASO titers are not available at our institution. There was no history of scarlet fever or previous rheumatic fever. Bacterial culture is available but expensive, and there were no signs or symptoms of pharyngitis upon presentation to Kijabe Hospital. As with many diagnoses in our setting, the conclusion in this case was "almost certain but presumptive." The constellation of signs and symptoms in an area of high prevalence, and in the absence of another etiology, strongly supports the verdict of acute rheumatic fever.

Differential Diagnosis

Other possibilities should be entertained, both for the entire clinical picture and for the chorea. Symmetric arthritis in a young woman should bring to mind rheumatoid arthritis, but this disease would not explain the chorea. Subacute bacterial endocarditis can cause fever, an inflammatory state, and central nervous system manifestations from emboli; however, this patient's symptoms resolved without therapy for endocarditis. Systemic lupus erythematosus, an uncommon disorder in Africa, can cause joint pain and chorea. The other causes of chorea are many and include congenital disorders (Huntington's disease, Wilson's disease, benign hereditary chorea, cerebral palsy, Lesch-Nyhan syndrome, neuroacanthocytosis, and dentatorubropallidoluysian atrophy), kernicterus, the antiphospholipid antibody syndrome, steroids (including oral contraceptives), paraneoplastic syndromes, and phenothiazine-associated tardive dyskinesia.[4] The patient's history is not consistent with these disorders.

Treatment

In addition to control of symptoms, the management of acute rheumatic fever aims to eradicate streptococcal infection and prevent recurrence.[5] Aspirin is effective in treating arthritis and fever. Adults may require 4-8 g per day. Therapy should continue until symptoms disappear and inflammatory markers such as the erythrocyte sedimentation rate normalize. Patients with carditis and congestive heart failure should be treated accordingly. The effectiveness of steroids for cardiac involvement is debatable, in contrast to their use in patients with chorea.

Eradication of streptococcal infection can be achieved with either oral penicillin for 10 days or with depot benzathine penicillin. Erythromycin is an option for those with penicillin allergy. Some authorities feel that prophylactic antibiotics (usually in the form of monthly benzathine penicillin) can be stopped after age 18-20 years if there have been no further attacks. Others believe prophylaxis should continue indefinitely for those with cardiac involvement given an ongoing risk of acute rheumatic fever as late as the sixth decade of life.

The treatment of chorea is potentially more problematic. As with other manifestations of rheumatic fever, attention must be paid to antibiotic therapy and prophylaxis. Choreiform movements typically persist for 3-4 months, although symptoms may rarely continue for years.[6] Corticosteroids appear to shorten the course of the illness and may also be used for recurrent exacerbations. For relapsing or chronic disease, antiepileptics (valproate and carbamazepine) are useful options, as is haloperidol, although this agent may be accompanied by extrapyramidal side effects.

Clinical Course

The current patient had severe, disabling chorea that had prevented oral intake for 3 days. An NG tube was inserted and therapy begun with chlorpromazine 50 mg intramuscularly every 8 hours and prednisolone 1 mg/kg per day. Following 2 days of therapy, the involuntary movements were markedly reduced and the patient was able to eat without a nasogastric tube Video 2. She was discharged home on prednisolone to complete a 2-week course. Instructions were given to report monthly to a local dispensary for injections of benzathine penicillin. At 1 week follow-up she was asymptomatic and had regained the ability to speak; however, she returned after 2 months with a milder recurrence of chorea, controlled with steroids and intermittent chlorpromazine. Three weeks following the second admission, she was again asymptomatic. Eight months later, she had not returned to Kijabe Hospital.

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