Slowly progressing varicella zoster brainstem encephalitis complicating Ramsay Hunt syndrome in an immunocompetent patient: case report and review of the literature

Abstract

A 56-year-old immunocompetent male developed brainstem encephalitis complicating Ramsay Hunt syndrome. The disease had a slowly progressing course of months after the triggering infection, much longer than previously reported. Furthermore, magnetic resonance imaging, physical-chemical, and cell count analyses on cerebrospinal fluid were normal, whereas polymerase chain reaction for varicella zoster virus DNA was positive. The simultaneous negativity of both imaging and basic CSF exams is very rare, although possible event which confirms the irreplaceable role of viral screening on CSF. A systematic review of similar reports with highlights on the unusual aspects of our case is also presented.

Brainstem and Cerebellar Involvement in Ramsay Hunt Syndrome

“…To the best of our knowledge, there have been 3 prior cases of RHS complicated exclusively by cerebellitis [1,4,5] and 3 prior cases of RHS complicated exclusively by rhombencephalitis [6][7][8]. While isolated involvement of the brainstem, cerebellum, and spinal cord has been discussed in the literature, no reports to our knowledge have described concurrent brainstem and cerebellar involvement [9].…”

Viral Encephalitis Caused by Herpes Zoster in the Waist and Abdomen: An Unusual Case Report

“…It also mostly occurs in patients with VZV infection in the brain or nerves of the cervical or upper thoracic spinal cord. 2 Viral encephalitis caused by VZV affecting distant body parts, such as herpes zoster of the waist and abdomen, is rare.…”

“…Some patients may have normal MRI findings, although such cases are rarely reported. Ricigliano et al 2 reported a 56-year-old immunocompetent man developed brainstem encephalitis as a complication of Ramsay Hunt syndrome with normal MRI findings, physical examination findings, chemistry parameters, and cell counts in the CSF. Finally, his diagnosis of herpes zoster virus encephalitis was made and confirmed by CSF VZV polymerase chain reaction.…”

Manifestaciones clínicas del síndrome de Ramsay-Hunt en una serie de 20 casos

“…Esta vasculitis herpética multifocal de pequeños vasos puede manifestarse como una encefalitis subaguda [67], incluso con la aparición de hemiparesia o hemihipoestesia [68]. Pero resulta muy infrecuente que un SRH evolucione a una encefalitis [69], presentándose siempre, de días a semanas después de las manifestaciones periféricas de la enfermedad [14]. Sólo la mitad de los afectados tenía una inmunodeficiencia previa, lo que hace difícil comprender cuáles son los mecanismos responsables de esta diseminación, que es un evento potencialmente mortal.…”