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Human herpes virus 6-associated acute limbic encephalitis in a 7-year-old patient with Li-Fraumeni’s syndrome after allogeneic stem cell transplantation

Reitz Mc,L. Porto,S. Bakhtiar,A. Jarisch,E. Rettinger,J. Sörensen,A. Willasch,P. Bader

Published 2021 in Trends in Transplantation

ABSTRACT

Human herpes virus (HHV) 6 is a β-herpesvirus mostly known for causing the common childhood illness exanthema subitum. Nearly all children had contact with HHV 6 at the age of 2 [1] with a peak incidence between 9 and 21 months [2]. Primary infection typically leads to fever for 3 to 5 days with sudden onset of a maculopapular rash upon defervescence, [3] hence the name exanthema subitum. HHV 6 persists in monocytes and salivary glands and can be reactivated when immunity is impaired [4]. As a neurotropic herpes virus, HHV 6 also overcomes the blood-brain barrier and can cause multiple neurological symptoms: primary infection coincides with febrile seizures and accounts for one third of all febrile seizures in children up to the age of two years [3,5]. It can also present as an acute encephalitis in immunocompetent populations [6,7] leading to headaches and seizures [8-11]. As HHV-6-associated encephalitis is described to be located in the limbic system, patients often show altered behavior, e.g. disorientation and confusion [9], drowsiness [8,12], aphasia [13-14], decreased appetite [10] and hallucinations [15]. Furthermore, HHV 6 is able to persist in the central nervous system [16] and reactivation has been associated with encephalitis and encephalopathy [17,18]. It has even been linked to neurodegenerative disorders such as multiple sclerosis [16].

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