The Sydenham chorea

A 6-year-old boy with eye twitching and echolalia.

  1. Transient tic disorder of childhood
  2. Tourette syndrome
  3. Sydenham chorea
  4. Dystonia
  5. Cerebral palsy

Explanation

Tics are commonly seen in a pediatric practice. All have in common the nonrhythmic, spasmodic, involuntary, stereotypical behaviors that involve any muscle group. Transient tic disorder is the most common and is seen more often in boys; a family history is often noted. In this condition, the patient has eye blinking, facial movements, or throat clearing lasting for weeks to about a year. No medications are needed. Chronic motor tics persist throughout life and can incorporate motor movements involving up to three muscle groups.

Gilles de la Tourette syndrome is a lifelong condition that is characterized by motor and vocal tics, obsessive-compulsive behavior, and a high incidence of attention deficit disorder with hyperactivity (ADDH). Therapy for the ADDH is helpful, as is medication to control the motor or vocal tics. Multiple psychosocial problems exist with these children; a multidisciplinary approach is helpful.

Dystonic reactions are sometimes seen in patients receiving phenothiazine medications. They have unusual neck, arm, or leg muscle twitches that are sometimes confused with seizure activity. Diphenhydramine, infused intravenously, usually rapidly reverses this relatively common idiosyncratic drug reaction.

Cerebral palsy is a static condition of movement and posture disorders frequently associated with epilepsy and abnormalities of vision, speech, and intellect. A defect in the developing brain is felt to be the cause. No significant change in the incidence of cerebral palsy has been noted in the past few decades despite drastically improved obstetric and neonatal care. No treatment for cerebral palsy is available; a multidisciplinary approach to manage the many medical problems associated with the condition is helpful.

The term chorea describes involuntary uncoordinated jerks of the arms and legs. Sydenham chorea is the most common acquired chorea of childhood, is seen after infections with group A β-hemolytic streptococci, and is associated with rheumatic heart disease and arthritis. In addition to the motor symptoms, patients may be hypotonic, emotionally labile and have a “milkmaid” grip with sequential grip tightening and relaxing. Other findings include a darting tongue and “spooning” of an extended hand (flexion at the wrist and extension of the fingers).