Ramsay Hunt Syndrome Associated with Varicella-Zoster Virus Encephalitis in a Child.

Ramsay Hunt syndrome (RHS) is a triad of peri-auricular pain, ipsilateral facial nerve palsy and vesicular rash around the ear pinna. It is caused by reactivation of varicella-zoster virus (VZV) that lies dormant in the geniculate ganglia. It can be complicated by VZV encephalitis rarely. We report the case of an 8-year-old previously healthy boy who presented to a tertiary care hospital in Muscat, Oman in 2021 with fever, progressive left ear pain, vesicular rash around his ear pinna and left-sided facial nerve palsy. His course was complicated by VZV encephalitis where he was managed with intravenous (IV) acyclovir and IV corticosteroids. He improved significantly and was asymptomatic with a normal neurology examination at the 6-months follow-up.

Clinical Analysis of 3D-Fluid Attenuated Inversion Recovery and T1volume interpolated body examination Sequences on Delayed Gadolinium-Enhanced Scanning in Ramsay Hunt Syndrome.

BACKGROUND: Through the clinical analysis of 4 clinically confirmed cases of delayed gadolinium enhancement of Ramsay Hunt syndrome 3D-Fluid Attenated Inversion Recovery'and 'T1volume interpolated body examination (3D-FLAIR and T1VIBE) sequences, the more suitable sequences and pathologically damaged tissue sites of deep tissues of Ramsay Hunt syndrome by magnetic resonance imaging gadolinium enhancement were preliminarily explored. METHODS: From October 2020 to March 2021, 4 clinically confirmed patients with Ramsay Hunt syndrome, 2 males and 2 females, aged 27-63, were continuously collected in the hospital otology clinic. Siemens Avento 1.5T magnetic resonance imaging 3D-FLAIR and T1VIBE sequencedelayed gadolinium enhancement scans and serological laboratory tests were performed, respectively, and corresponding antiviral and antiinflammatory therapy was given. RESULTS: The magnetic resonance imaging gadolinium enhancement of 4 cases of Ramsay Hunt syndrome was as follows: 3D-FLAIR sequence delay of 4.5 hours scanning 4 patients labyrinthine and/or middle ear signal was enhanced at the same time as the healthy side; T1VIBE sequence scanning disease in 3 cases of vestibular nerve development was enhanced than the healthy side, 2 cases of facial nerve development was enhanced than the healthy side, and 2 cases of cochlear nerve development was enhanced than the healthy side. All 4 patients were cured with related treatment. CONCLUSION: Through the comparison of 3D-FAIR and T1VIBE sequence of 4.5 hours delay before intravenous gadolinium injection and 4.5 hours delay after intravenous gadolinium injection in 4 patients with Ramsay Hunt syndrome, it was found that (i) 3D-FLAIR sequence delay of 4.5 hours scan was more likely to show whether the inner ear labyrinth barrier permeability increased and (ii) Ramsay Hunt syndrome deep ear tissue damage can be manifested as labyrinthitis, vestibular cochlear neuritis, facial neuritis, and otitis media.

Multiple cranial neuropathy due to varicella zoster virus reactivation without vesicular rash: a challenging diagnosis.

Ramsay Hunt syndrome is due to reactivation of varicella zoster virus (VZV) dormant in the geniculate ganglion of the facial nerve. The diagnosis is typically based on clinical triad of ipsilateral facial paralysis, otalgia, and vesicles in the auditory canal or the auricle. However, Ramsay Hunt syndrome may occur without skin eruption in up to one third of patients. Moreover, the involvement of other cranial nerves in addition to the facial nerve has been also reported. Herein, we reported a case report of a man who developed a multiple cranial neuropathy caused by VZV reactivation without skin vesicular eruption. The present case underlines a possible diagnostic challenge that clinicians may hit when facing a common disorder such as peripheral facial palsy. Indeed, clinicians must be aware that Ramsay Hunt syndrome may develop without skin vesicular eruption as well it may be complicated by multiple cranial nerve involvement. Antiviral therapy is effective in VZV reactivation for recovery of nerve function.

Two cases of Ramsay-Hunt syndrome following varicella zoster viral meningitis in young immunocompetent men: case reports.

BACKGROUND: Ramsay-Hunt syndrome (RHS) due to varicella zoster virus (VZV) infection is commonly reported in individuals aged at least 50 years or immunocompromised individuals. VZV infection may invade the central nervous system (CNS) and cause meningitis or encephalitis, which are more likely to occur in patients with chronic diseases such as diabetes and chronic renal failure. However, cases with VZV-induced concurrent RHS and CNS infections are rare. CASE PRESENTATION: Two young male patients, aged 32 and 43 years, with no underlying disease developed VZV meningitis, followed by RHS involving cranial nerves VII and VIII. Both patients presented with symptoms of peripheral facial palsy, and dizziness accompanied by tinnitus and hearing loss, which appeared several days after the onset of fever and headache. These symptoms were documented as facial neuropathy and sensorineural hearing loss in the electrophysiologic studies. Lymphocyte-dominant pleocytosis and VZV positivity were confirmed from cerebrospinal fluid examination and polymerase chain reaction, respectively. The patients were treated with intravenous acyclovir and oral steroids simultaneously. Following the treatment completion, both patients were relieved of their headaches and fever; however, facial palsy, dizziness, and tinnitus persisted. They were followed up at the outpatient clinic. CONCLUSION: These cases confirmed that RHS and CNS infections can co-exist even in young adults with normal immune function and more importantly, that CNS infection can precede RHS. Since early detection and treatment of RHS improve the prognosis, it is critical to closely monitor patients with VZV meningitis or encephalitis considering the possible superimposition of RHS.

Benefits of High-Dose Corticosteroid and Antiviral Agent Combination Therapy in the Treatment of House-Brackman Grade VI Ramsay Hunt Syndrome.

OBJECTIVE: Few large-scale investigations have been conducted on treatment of House-Brackmann grade VI (HB grade VI) Ramsay Hunt syndrome (RHS) patients. We compared recovery rates among patients receiving a normal-dose corticosteroid (prednisolone [PSL] 60 mg/d) or high-dose corticosteroid (PSL 200 mg/d), both with or without an antiviral agents. Recovery rates were also examined based on the order of presentation of herpetic vesicles versus facial palsy. STUDY DESIGN: Retrospective case review. SETTING: Tertiary referral center. PATIENTS: A total of 128 patients with HB grade VI RHS were treated in our department between 1995 and 2017. These patients were divided into four treatment groups based on corticosteroid dosage and use of an antiviral agent. METHODS: We assessed treatment outcomes for HB grade VI patients together with logistic regression analysis to investigate factors that can impact treatment outcomes, that is, sex, age, days to start of treatment, PSL dosage, and antiviral agent administration. RESULTS: Recovery rates were best in the high-dose corticosteroid group with an antiviral agent (71.1%) in comparison with the normal-dose corticosteroid group with an antiviral agent (60.0%) or high-dose corticosteroid alone (57.1%). Significant factors for treatment outcomes were high-dose corticosteroid administration and early initiation of treatment. A better recovery rate was also found when the herpetic vesicles appeared before facial palsy. CONCLUSION: We showed that a combination of a high-dose corticosteroid and antiviral agent produced the best outcomes for patients with HB grade VI RHS. However, our results were not statistically significant because of small sample size.

[Clinical and therapeutic features of herpes zoster oticus: a case report]. / Zona otitique, aspects cliniques et thérapeutiques: à propos d'un cas.

Ear shingles, also called herpes zoster oticus, are a viral infection of the outer, middle and/or inner ear due to varicella zoster virus (VZV). Patients with ear involvement associated with facial palsy are clinically diagnosed with Ramsay-Hunt´s syndrome. We here report the case of a 25-year-old patient with herpes zoster oticus associated with peripheral facial palsy, without other associated signs. This study and literature review investigate the various clinical, para-clinical and evolutionary features of herpes zoster oticus and the therapeutic approaches.

Intratympanic corticosteroid for Bell's palsy and Ramsay Hunt syndrome: Systematic review and meta-analysis.

OBJECTIVE: This study aimed to reveal the efficacy and safety of intratympanic steroid therapy (ITST) in patients with Bell's palsy and Ramsay Hunt syndrome. METHODS: A literature search was conducted in PubMed, Embase, Ichushi-Web, and Cochrane Central Register of Controlled Trials. Published randomized controlled trial and observational studies, which compared the combination of intratympanic corticosteroids with systemic corticosteroid versus systemic corticosteroid for Bell's palsy and Ramsay Hunt syndrome, were included for meta-analysis. The primary outcome was non-recovery at the end of the study follow-up. Data was analyzed using Review Manager software, and pooled odds ratio with 95% CI were calculated. RESULTS: Three randomized controlled trial and two cohort studies met the eligible criteria, and 350 individuals included for meta-analysis. The timing of intratympanic corticosteroid was varied from once or twice a week to consecutive 10-days. Patients treated with combination of systemic corticosteroids and intratympanic corticosteroids were likely to achieve recovery of normal facial function (OR = 0.36 [95% CI 0.12-1.14]). The subgroup analysis of the timing revealed that daily ITST reduced non-recovery (OR = 0.14 [95% CI 0.04-0.50). However, non-daily ITST did not reduced non-recovery (OR 0.93 [95% CI 0.37-2.34]). CONCLUSION: Intratympanic corticosteroid have a potential to reduce non-recovery in patients with Bell's palsy and Ramsay Hunt syndrome. The methods of intratympanic corticosteroid varied among included studies. Further well-designed randomized controlled trial is needed to confirm the effectiveness and to identify an adequate method of intratympanic corticosteroid in these patients.

Herpes zoster oticus. / Herpes zoster oticus.

BACKGROUND: Herpes zoster oticus is a rare neurological disease caused by reactivation of varicella-zoster virus in the facial nerve. CASE PRESENTATION: A woman in her 60 s presented with left-sided seventh and eighth cranial neuropathy. A vesicular rash in her left ear and palate appeared one week after symptom onset. Lumbar puncture revealed cerebrospinal fluid (CSF) pleocytosis, mononuclear cells only, with PCR positive for varicella zoster virus. Inflammation of the cochlear and vestibular systems and along the facial nerve was detected by MRI. Antiviral treatment with valacyclovir in combination with prednisolone was initiated. After one month with outpatient rehabilitation, the vertigo had ceased, but hearing loss and facial paralysis persisted. INTERPRETATION: Early diagnosis and treatment of herpes zoster oticus as a cause of facial palsy when accompanied by ear rash, pain or signs of other cranial nerve involvement may improve overall prognosis.

Zoster Cranial Polyneuropathy in a COVID-19 Patient.

BACKGROUND Ramsay Hunt syndrome is a rare form of herpes zoster caused by the reactivation of the varicella-zoster virus in the geniculate ganglion. The main clinical manifestations are peripheral facial palsy, vesicular rash in the ear, and ipsilateral auricular pain, and sometimes vertigo. COVID-19 is a new multisystemic infectious disease that, in addition to common respiratory manifestations, it is known to affect the immune system, primarily depressing cellular immunity. CASE REPORT A 54-year-old woman was admitted to our hospital with an acute vestibular syndrome and diplopia. She had been diagnosed 3 years prior with interstitial lung disease for which she was taking methylprednisolone. At admission, she tested positive for SARS-CoV-2. In the following days, she developed a sixth nerve palsy on the left side and a right peripheral facial palsy on the right side, followed by a typical zoster rash on the ipsilateral ear. One month later, she developed acute severe hearing loss on the right side. There were no COVID-19 symptoms during her stay in our hospital. The MRI showed Gd enhancement of both facial nerves. Under antiviral and corticoid treatment, the evolution was favorable, with marked improvement at 6 months. CONCLUSIONS COVID-19 increases the risk for herpes zoster infection, probably through induced depression of the cellular immunity. Our case suggests Ramsay Hunt syndrome can be the presenting symptom and sometimes the only symptom of COVID-19. This also seems to be true for other cranial neuropathies, and we recommend testing these patients even if there are no other manifestations.

Unusual presentation of Ramsay-Hunt Syndrome in kidney transplant patient.

Herpes Zoster (HZ) is caused by reactivation of latent varicella zoster virus (VZV) in craniospinal sensory neurons and is characterized by a painful erythematous rash in the affected dermatome. Although kidney transplant recipients who are chronically maintained on immunosuppressive regimens are considered at risk, there are only a few cases described. We report a well-documented case of a 50-year-old male kidney transplant recipient who presented Ramsay-Hunt syndrome with atypical neurological finds.

[Ramsay Hunt syndrome can present with vesicular elements on the tongue, facial nerve palsy and meningitis].

Ramsay Hunt syndrome (RHS) is caused by a reactivation of the varicella zoster virus (VZV). It normally involves the triad of earache, peripheral facial nerve palsy and ipsilateral rash but can present without/with minimal visible rashes. In this case report, a 53-year-old male was admitted to hospital after a week-long headache, right-sided earache and peripheral facial nerve palsy. Vesicular elements were observed on the right side of the tongue. Cerebrospinal fluid (CSF) analysis showed 195 monocytes/mm3, and PCR verified VZV in the CSF. To avoid misdiagnosis, early recognition of the symptoms of RHS and diagnostic workup is advised.

[Herpes zoster duplex associated with Ramsay Hunt syndrome and cervical zoster paresis. A case report].

A 63-year-old Japanese female in an immunocompetent state developed right Ramsay Hunt syndrome and left shoulder pain, and left upper limb motor paresis with herpes zoster (HZ) duplex in the right auricle and left shoulder regions. With her Ramsay Hunt syndrome, neural deafness, tinnitus and vestibular symptoms were observed, and she lacked facial nerve palsy. Cerebrospinal fluid (CSF) findings revealed an increase in lymphocytes (21 cells/µl) and protein content (29 mg/dl), and polymerase chain reaction for varicella-zoster virus DNA in CSF was negative. Cervical root MRI using 3D Nerve VIEW (Philips) imaging showed high-intensity lesions on the C5-C8 spinal roots with contrast enhancements. No abnormalities were observed in the median or ulnar motor sensory nerve conduction velocity conduction studies including the F wave. PubMed search revealed no report of a patient with this profile, and to the best of our knowledge HZ duplex with concomitant neurological impairments has not been reported. We compare our present case with several similar cases from the literature.