Ramsay hunt syndrome and similar entity: Management dilemma

Background: Varicella-zoster virus (VZV) is a DNA virus which causes primary chickenpox infection via droplet transmission. After a period of latency, its reactivation can cause herpes zoster, with complications including severe meningo-encephalitis and Ramsay Hunt Syndrome (RHS)1. The role of antiviral agents in both conditions remains unclear, leading to discrepancy in treatment decision based on local expert's opinion2. Case Report: A previously well, 49 years old general practitioner presented to emergency department with 3 days history of severe right periauricular headache, vertigo and vomiting. Following normal neurological examination and CT (computed tomography) brain, he was discharged home. Patient represented the next day with ongoing headache and new photophobia. Bedside examination revealed inflamed right outer-ear without appreciable vesicles, along with facial palsy, hearing impairment and numbness in fifth cranial nerve (5-1, 5-2) distribution on the right side. Urgent LP (lumbar puncture) showed leucocytes 53/uL,100% mononuclear cells, protein 0.65 g/L, glucose 3.6mmol/L, no microorganisms seen on culture. Right ear swab was positive for VZV DNA polymerase chain reaction (PCR). Intravenous ceftriaxone, dexamethasone and acyclovir were prescribed to patient while awaiting cerebrospinal fluid (CSF) culture. Patient was then transferred to another hospital, following positive CSF culture for VZV PCR, for management of VZV meningo-encephalitis. After MRI (Magnetic resonance imaging) brain exclusion of focal intracranial lesion, IV acyclovir was ceased in 48 hours and switched to oral valacyclovir for 7 days. Patient made an uneventful recovery in the community. Discussion: A systematic review in 2008 found no evidence that antiviral agents have a beneficial effect on outcomes in RHS2, although some retrospective studies showed more significant recovery in those who received steroid and antiviral agents. The optimal treatment of VZV meningitis is also unknown. Most clinicians would give antiviral agents, yet the route and duration of therapy is widely variable according to expert opinion. As highlighted in our case, it's not been well established if and/or when a good practice is, to switch to oral antiviral agents at early stage of Herpes zoster meningitis with VZV positive CSF. One would argue if intravenous antiviral therapy should have been extended more than 48 hours in our patient with VZV viral meningitis, but there was no sufficient evidence to support or contradict it. Conclusion: Neurological complications of VZV infection, although uncommon, can lead to potentially life threatening sequalae. It's worthwhile discussing different entities of VZV neurological complication such as RHS, VZV meningitis, subclinical meningeal irritation, encephalitis and vasculopathy, along with a clear management guideline.