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Human Herpesvirus 6 Rhombencephalitis in Immunocompetent Children

Departments of Neurology Children's National Medical Center, George Washington University, Washington District of Columbia,jcrawfor{at}cnmc.org

Nadja Kadom, MD

Radiology Children's National Medical Center, George Washington University, Washington District of Columbia

Maria Rita Santi, MD

Pathology Children's National Medical Center, George Washington University, Washington District of Columbia

Brian Mariani, PhD

Molecular Infectious Disease Laboratory, Genetics & IVF Institute, Fairfax, Virginia

Bennett L. Lavenstein, MD

Departments of Neurology Children's National Medical Center, George Washington University, Washington District of Columbia

This article describes the clinical presentation, diagnostic workup, and neurologic outcome of 3 immunocompetent pediatric patients diagnosed with human herpesvirus 6 (HHV6) rhombencephalitis. Presentation of HHV6 rhombencephalitis included new onset seizures, ataxia, encephalopathy, and opsoclonus-myoclonus. Neurologic examination revealed cranial neuropathies, cerebellar dysfunction, and extremity weakness. Magnetic resonance imaging abnormalities located in the cerebellum, basal ganglia/thalamus, and cerebral hemispheres were detected in 2 patients. Diagnosis of HHV6 encephalitis was made by real-time and nested polymerase chain reaction of serum and cerebrospinal fluid. The HHV6 variant A was detected in 2 patients by sequence analysis, and HHV6 protein was detected by immunomicroscopy in a patient who underwent biopsy secondary to progressive clinical and neuroradiographic findings. Therapy with intravenous ganciclovir did not correlate with resolution of neurologic symptoms, despite eventual non-detectable HHV6. Human herpesvirus 6 should be considered in the differential diagnosis of unexplained cases of rhombencephalitis in immunocompetent children. Features may be rapidly progressive and include profound encephalopathy, seizures, ataxia, and opsoclonus-myoclonus.

Key Words: Human herpesvirus 6 • HHV6 • encephalitis

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