Human herpesvirus 6 rhombencephalitis in immunocompetent children.

Abstract

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.

• Authors: John R Crawford, Nadja Kadom, Maria Rita Santi, Brian Mariani, Bennett L Lavenstein
• Journal: Journal of child neurology (J Child Neurol) Vol. 22 Issue 11 Pg. 1260-8 (Nov 2007) ISSN: 0883-0738 [Print] United States
• PMID: 18006954 (Publication Type: Case Reports, Journal Article, Research Support, N.I.H., Extramural)

Topics

• Child, Preschool
• Encephalitis, Herpes Simplex (pathology, virology)
• Female
• Herpesvirus 6, Human (isolation & purification, pathogenicity)
• Humans
• Immunocompetence
• Infant
• Magnetic Resonance Imaging (methods)
• Male
• Rhombencephalon (virology)