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Human herpesvirus 6 rhombencephalitis in immunocompetent children

John R Crawford1, Nadja Kadom, Maria Rita Santi

  • 1Department of Neurology Children's National Medical Center, George Washington University, Washington District of Columbia, USA. jcrawfor@cnmc.org

Journal of Child Neurology
|November 17, 2007
PubMed

Insights

Human herpesvirus 6 (HHV6) rhombencephalitis is a rare pediatric condition presenting with seizures and ataxia. Early diagnosis via PCR is crucial, though ganciclovir may not resolve symptoms in immunocompetent children.

Area of Science:

  • Pediatric Neurology
  • Infectious Diseases
  • Neurovirology

Background:

  • Human herpesvirus 6 (HHV6) rhombencephalitis is an uncommon but severe neurological condition in children.
  • Understanding the clinical spectrum and diagnostic challenges of HHV6 rhombencephalitis is essential for timely intervention.

Observation:

  • Three immunocompetent pediatric patients presented with new-onset seizures, ataxia, encephalopathy, and opsoclonus-myoclonus.
  • Neurologic examination revealed cranial neuropathies, cerebellar dysfunction, and extremity weakness.
  • MRI showed abnormalities in the cerebellum, basal ganglia/thalamus, and cerebral hemispheres in two patients.

Findings:

  • Diagnosis was confirmed by real-time and nested PCR of serum and cerebrospinal fluid, detecting HHV6 variant A in two cases.
  • HHV6 protein was identified via immunomicroscopy in a patient with progressive disease.
  • Intravenous ganciclovir treatment did not correlate with neurologic symptom resolution, despite viral clearance.

Implications:

  • HHV6 rhombencephalitis should be considered in the differential diagnosis of unexplained rhombencephalitis in immunocompetent children.
  • The condition can present with rapidly progressive and profound neurological deficits, including encephalopathy, seizures, ataxia, and opsoclonus-myoclonus.
  • Current therapeutic strategies may not guarantee symptom resolution, highlighting the need for further research into effective treatments.

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