\documentclass[10pt]{article}

\usepackage{fullpage}
\usepackage{setspace}
\usepackage{parskip}
\usepackage{titlesec}
\usepackage[section]{placeins}
\usepackage{xcolor}
\usepackage{breakcites}
\usepackage{lineno}
\usepackage{hyphenat}





\PassOptionsToPackage{hyphens}{url}
\usepackage[colorlinks = true,
            linkcolor = blue,
            urlcolor  = blue,
            citecolor = blue,
            anchorcolor = blue]{hyperref}
\usepackage{etoolbox}
\makeatletter
\patchcmd\@combinedblfloats{\box\@outputbox}{\unvbox\@outputbox}{}{%
  \errmessage{\noexpand\@combinedblfloats could not be patched}%
}%
\makeatother


\usepackage{natbib}




\renewenvironment{abstract}
  {{\bfseries\noindent{\abstractname}\par\nobreak}\footnotesize}
  {\bigskip}

\titlespacing{\section}{0pt}{*3}{*1}
\titlespacing{\subsection}{0pt}{*2}{*0.5}
\titlespacing{\subsubsection}{0pt}{*1.5}{0pt}


\usepackage{authblk}


\usepackage{graphicx}
\usepackage[space]{grffile}
\usepackage{latexsym}
\usepackage{textcomp}
\usepackage{longtable}
\usepackage{tabulary}
\usepackage{booktabs,array,multirow}
\usepackage{amsfonts,amsmath,amssymb}
\providecommand\citet{\cite}
\providecommand\citep{\cite}
\providecommand\citealt{\cite}
% You can conditionalize code for latexml or normal latex using this.
\newif\iflatexml\latexmlfalse
\providecommand{\tightlist}{\setlength{\itemsep}{0pt}\setlength{\parskip}{0pt}}%

\AtBeginDocument{\DeclareGraphicsExtensions{.pdf,.PDF,.eps,.EPS,.png,.PNG,.tif,.TIF,.jpg,.JPG,.jpeg,.JPEG}}

\usepackage[utf8]{inputenc}
\usepackage[ngerman,english]{babel}



\usepackage{float}








\begin{document}

\title{Ramsay-Hunt syndrome: a case report}



\author[1]{Ana Rocha}%
\author[1]{Filipa Leal}%
\author[2]{Marta Dalila Martins}%
\author[2]{Marina  Mendes}%
\author[1]{Bárbara Silva}%
\author[1]{Luis Nogueira}%
\affil[1]{Centro Hospitalar Tamega e Sousa EPE}%
\affil[2]{Centro Hospitalar Tâmega e Sousa EPE}%


\vspace{-1em}



  
  \date{May 23, 2024}


\begingroup
\let\center\flushleft
\let\endcenter\endflushleft
\maketitle
\endgroup








\sloppy


\textbf{INTRODUCTION:}

Ramsay-Hunt syndrome is a disease caused by reactivation of latent
varicella zoster virus in the geniculate ganglia.~It involves facial
nerve paralysis and it is usually associated with a rash on the auricle
and/or external ear~{[}1{]}.~Sometimes the blisters are not very
noticeable and can be overlooked by the inspector. Other accompanying
symptoms may be ache and fullness of the ear (78\%), vertigo/dizziness
(51\%), hearing loss (45\%), nausea, vomiting, tinnitus and
hyperacusia~{[}2{]}.

The diagnosis is mainly based on the history, clinical findings and
neurological examination. Polymerase chain reaction assays may be useful
by detecting DNA of the virus in exudates from ear scraping or
cerebrospinal fluid (CSF)~{[}3{]}.

It represents 7\% of all peripheral facial paralysis and recovery rates
are lower in this group~{[}4,5{]}. Early treatment with a combination of
acyclovir and prednisone is reported to be effective~{[}6-8{]}.

\textbf{CASE DESCRIPTION:}

A 46-year-old previously healthy woman was admitted to the emergency
department complaining of mouth drooping and vertigo since that morning.
She also had a headache and pain on the right ear since the night
before. She denied fever, hearing loss, hyperacusia, tinnitus, nausea or
vomiting.

On physical examination, she was alert and oriented, with a Glasgow Coma
Scale score of 15, without focal neurologic deficits except for right
peripheral facial palsy {[}Figure 1{]}. Gait was not tested because of
her vertigo but she had no dysmetria on finger-to-nose and heel-to-knee
tests. She was hemodynamically stable and afebrile with no meningeal
signs. She had no visible vesicular eruptions nor any abnormalities on
otoscopy.

Lab work {[}Table~1{]} showed a total leukocyte count of 12.270/uL
(normal range 4.000-12.000/uL). Other than that, blood routine
examination was~normal, including a low level of C-reactive protein
(4mg/L, normal range \textless{}5mg/L).

Contrast-enhanced head CT scan {[}Figure~2{]} showed no signs of acute
vascular, hemorrhagic or edematous events and documented normal
permeability of deep and superficial venous system.

Because of persistent headache, she underwent a lumbar puncture with
drainage of clear CSF. CSF analysis showed normal glycorrhachia, no
pleocytosis and slight proteinorrhachia (84 mg/dL, normal range
15-60mg/dL). Screening for varicella zoster DNA on CSF by polymerase
chain reaction was positive.

A clinical diagnosis of Ramsay-Hunt syndrome was established. The
patient was started on acyclovir 10 mg/kg every 8 hours for 14 days,
prednisolone 1mg/kg for 5 days, humidifying eye drops and right eye
protection. Physiotherapy for the face was initiated and the patient
slowly improved, maintaining at the time of discharge a class III palsy
on the House-Brackmann Facial Nerve Grading System.

\textbf{DISCUSSION:}

Ramsay-Hunt syndrome is perhaps an underdiagnosed form of~peripheral
facial palsy~{[}5{]}, since the diagnosis is mainly based on clinical
findings of facial weakness and a vesicular rash~{[}1{]}. Regarding the
timing of these manifestations, the clinical picture includes three
possibilities. When~the vesicular rash occurs before or simultaneously
with the facial palsy, the diagnosis is usually easy. Sometimes the
blisters develop only after the onset of facial weakness and
occasionally they are not very noticeable and can be overlooked. A
minority of patients, like ours, do not develop them at all. One
retrospective study showed that in 6\% of patients, only the ear canal
or the ear drum were affected, without visible blisters outside. This
can make the diagnosis more difficult and indistinguishable from Bell's
palsy~{[}2{]}.

Our patient also complained of ear pain, headache and vertigo. This
syndrome may also present with acute herpetic neuralgia that can radiate
to the face, ear, head and neck~{[}9{]}. Approximately 50\% of patients
are expected to have an injury of the vestibulocochlear nerve. This can
cause nausea, vomiting, gait insecurity with a tendency to fall to the
affected side, vertigo and~nystagmus~{[}10{]}. Damage of the cochlear
component leads to tinnitus and hearing loss~{[}11,12{]}.

Analysis of cerebrospinal fluid and brain magnetic resonance imaging has
limited diagnostic and prognostic value. However, polymerase chain
reaction assays may be useful to detect herpes zoster virus DNA in
exudates from ear scrapings or CSF and MRI can visualize inflammation of
the cranial nerves~{[}3{]}.

It is important to differentiate Ramsay-Hunt syndrome from other forms
of facial palsy, since the former often has more severe paralysis and
has less chances of recovery~{[}13{]}. Treatment of this condition
involves both glucocorticoids and antiviral therapy, while for Bell's
palsy it remains uncertain whether antiviral therapy adds
benefit~{[}5,8,10{]}. Antiviral agents improve acute pain, recovery rate
and prevent from the occurrence of postherpetic neuralgia~{[}14{]}.
Glucocorticoids have a strong anti-inflammatory action, reducing~edema
in the nerves involved, which can~lead to a hasten recovery~{[}10{]}.
There does not seem to be a difference between the use of oral versus
intravenous antivirals~{[}11{]}.~Starting treatment in the first 48
hours of symptoms, as we did in this case, is crucial as nerve damage
and therefore prognosis depends on the timing at which the combination
therapy is started~{[}5,8,10{]}.

\textbf{CONCLUSIONS}

A high index of suspicion is necessary to diagnose a Ramsay-Hunt
syndrome in a patient presenting with~peripheral facial palsy,
especially in the absence of a typical vesicular rash. Analysis of CSF
has limited value but the detection of varicella zoster DNA may help to
prove the diagnosis.~Furthermore, early diagnosis and adequate treatment
is crucial to improve damaged nerves and maximize the chances of full
recovery.

\textbf{AUTHOR CONTRIBUTIONS:}

Author 1: Involved in patient care, data and information collection and
main author of the manuscript.

Author 2: Involved in patient care and manuscript review.

Author 3: Involved in patient care and manuscript review.

Author 4: Involved in patient care and manuscript review.

Author 5: Involved in manuscript review.

Author 6: Involved in manuscript review.

\textbf{ACKNOWLEDMENTS} : None

\textbf{ETHICAL APROVAL} :~The authors have no ethical conflicts to
disclose.

\textbf{CONSENT} :~Informed consent was obtained for publication of this
case report.

\textbf{REFERENCES}

\begin{enumerate}
\tightlist
\item
  Kim SH, Jung J, Jung SY, et. al:~Comparative prognosis in patients
  with Ramsay-Hunt syndrome and Bell's palsy. Eur Arch Otorhinolaryngol.
  2019, 276(4):1011-1016.~10.1007/s00405-019-05300-3
\item
  Kanerva M, Jones S, Pitkaranta A:~Ramsay Hunt syndrome:
  characteristics and patient self-assessed long-term facial palsy
  outcome. Eur Arch Otorhinolaryngol. 2020,
  277(4):1235-1245.~10.1007/s00405-020-05817-y
\item
  Sauerbrei A, Eichhorn U, Schacke M, et. al:~Laboratory diagnosis of
  herpes zoster. J Clin Virol. 1999,
  14(1):31-36.~10.1016/s1386-6532(99)00042-6
\item
  Yeo SW, Lee DH, Jun BC, et. al:~Analysis of prognostic factors in
  Bell's palsy and Ramsay Hunt syndrome. Auris Nasus Larynx. 2007,
  34(2):159-164.~10.1016/j.anl.2006.09.005
\item
  Dorsch JN:~Neurologic syndromes of the head and neck. Prim. Care.
  2014, 41(1):133-149.~10.1016/j.pop.2013.10.012
\item
  Furuta Y, Ohtani F, Mesuda Y, et. al:~Early diagnosis of zoster sine
  herpete and antiviral therapy for the treatment of facial palsy.
  Neurology. 2000, 55(5):708-710.~10.1212/wnl.55.5.708
\item
  Adour KK:~Combination treatment with acyclovir and prednisone for Bell
  palsy. Arch Otolaryngol Head Neck Surg. 1998,
  124(7):824.~10.1001/archotol.124.7.824
\item
  Monsanto RC, Bittencourt AG, Neto NJB, et. al:~Treatment and prognosis
  of facial palsy on Ramsay Hunt syndrome: results based on a review of
  the literature. Arch Otorhinolaryngol. 2016,
  20(4):394-400.~10.1055/s-0036-1584267
\item
  Nanda A, Khan IS:~Nervus intermedius and geniculate neuralgia. World
  Neurosurg. 2013, 79(5-6):651-652.~10.1016/j.wneu.2012.05.002
\item
  Jeon Y, Lee H:~Ramsay Hunt syndrome. J Dent Anesth Pain Med. 2018,
  18(6):333-337.~10.17245/jdapm.2018.18.6.333
\item
  Murakami S, Hato N, Horiuchi J, et. al:~Treatment of Ramsay Hunt
  syndrome with acyclovir-prednisone: significance of early diagnosis
  and treatment. Ann Neurol. 1997, 41(3):353-357.~10.1002/ana.410410310
\item
  Xanthopoulos J, Noussios G, Papaioannides D, et. al:~Ramsay Hunt
  syndrome presenting as a cranial polyneuropathy. Acta Otorhinolaryngol
  Belg. 2002, 56(3):319-323.
\item
  Sweeney C, Gilden D:~Ramsay Hunt syndrome. J Neurol Neurosurg
  Psychiatry. 2001, 71(2):149-154.~10.1136/jnnp.71.2.149
\item
  Kinishi M, Amatsu M, Mohri M, et. al:~Acyclovir improves recovery rate
  of facial nerve palsy in Ramsay Hunt syndrome. Auris Nasus Larynx.
  2001, 28(3):223-226.~10.1016/s0385-8146(01)00055-4
\end{enumerate}

\textbf{Hosted file}

\verb`Figures and Tables.docx`  available at \url{https://authorea.com/users/653662/articles/660502-ramsay-hunt-syndrome-a-case-report}

\selectlanguage{english}
\FloatBarrier
\end{document}

