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Chorea as a presentation of herpes simplex encephalitis relapse
G G Gascon1, A A al-Jarallah, E Okamoto
1Department of Pediatrics, King Faisal Specialist Hospital & Research Centre, Riyadh, Saudi Arabia.
Insights
Herpes simplex encephalitis (HSE) relapses can manifest as chorea in infants. Extended acyclovir treatment (3 weeks) may improve outcomes, while shorter courses show limited efficacy for this rare neurological complication.
Area of Science:
- Neurology
- Infectious Diseases
- Pediatrics
Background:
- Herpes simplex encephalitis (HSE) is a severe neurological infection.
- Relapses of HSE can present with varied neurological symptoms.
- Chorea as an initial manifestation of HSE relapse is rare in infants.
Observation:
- Three infants presented with chorea as the initial symptom of HSE relapse.
- Treatment responses to acyclovir varied significantly among patients.
- One patient relapsed years after initial treatment, while another showed no improvement.
Findings:
- Patient 3 showed clear improvement after a 3-week course of acyclovir.
- Patients treated with shorter or repeated 10-day courses had less favorable outcomes.
- A fourth patient with chorea, initially suspected as HSE, tested negative for HSV.
Implications:
- A minimum 3-week course of acyclovir at 30-35 mg/kg/day is recommended for HSE relapse presenting with chorea.
- This extended treatment duration may be crucial for managing this specific HSE complication.
- Accurate diagnosis is essential, as chorea can have other etiologies.
Abstract:
Three infants, ages 3 months to 3 years, presented with chorea as the initial manifestation of herpes simplex encephalitis (HSE) relapse. Patient 2, treated with repeated 10 day courses of 30 mg/kg/day of acyclovir, had no clear improvement in neurological status. Patient 1, treated with a repeated 10-day course, improved only to have another HSE relapse 4 years later. Patient 3 clearly improved soon after a 3-week course of acyclovir at conventional dosages. A fourth patient (Patient 4) who relapsed with chorea after what was thought to be HSE, and who did not respond to repeated acyclovir treatment, was negative for herpes simplex virus indicators on brain biopsy and DNA testing. We recommend treating all patients suffering from HSE with a minimum 3-week course of acyclovir at 30-35 mg/kg/day in 3 divided doses.