Serum and cerebrospinal fluid neurofilament light chain in patients with central nervous system infections caused by varicella-zoster virus.

Varicella-zoster virus (VZV) is a common cause of viral central nervous system (CNS) infection, and patients may suffer from severe neurological sequelae. The biomarker neurofilament light chain (NFL) is used for assessment of neuronal damage and is normally measured in cerebrospinal fluid (CSF). Novel methods have given the possibility to measure NFL in serum instead, which could be a convenient tool to estimate severity of disease and prognosis in VZV CNS infections. Here, we investigate the correlation of serum and CSF NFL in patients with VZV CNS infection and the association of NFL levels in serum and CSF with different VZV CNS entities. NFL in serum and CSF was measured in 61 patients who were retrospectively identified with neurological symptoms and VZV DNA in CSF detected by PCR. Thirty-three herpes zoster patients and 40 healthy blood donors served as control groups. NFL levels in serum and CSF correlated strongly in the patients with VZV CNS infection. Encephalitis was associated with significantly higher levels of NFL in both serum and CSF compared with meningitis and Ramsay Hunt syndrome. Surprisingly, herpes zoster controls had very high serum NFL levels, comparable with those shown in encephalitis patients. We show that analysis of serum NFL can be used instead of CSF NFL for estimation of neuronal injury in patients with VZV CNS infection. However, high levels of serum NFL also in patients with herpes zoster, without signs of CNS involvement, may complicate the interpretation.

Ramsay Hunt syndrome associated with solitary nucleus, spinal trigeminal nucleus and tract, and vestibular nucleus involvement on sequential magnetic resonance imaging.

Ramsay Hunt syndrome (RHS) is an acute peripheral facial nerve paralysis typically accompanied by erythematous vesicular lesions of the auricular skin. The etiology is considered to be geniculate ganglionitis due to reactivation of varicella-zoster virus (VZV). Encephalitis is a rare but serious complication of VZV reactivation. Clarifying the regional and temporal evolution of the lesions on magnetic resonance imaging (MRI) would help with understanding the pathology of the lesion, but this information is lacking in encephalitis with RHS. Therefore, here, we reviewed sequential MR images in three RHS cases complicated by brainstem lesions. All the regions of the lesions represent specific neuronal structures-the ipsilateral solitary nucleus (SN) and spinal trigeminal nucleus and tract (STNT) in case 1; bilateral SN, ipsilateral STNT, and vestibular nucleus in case 2; ipsilateral SN and vestibular nucleus in case 3-and this seems to account for the persistent robust symptoms. Case 1 initially showed no abnormalities on MRI and cases 2 and 3 showed weak signals on the first MRI which subsequently plateaued. These observations suggest the timeframe within which it becomes possible to detect regional and temporal evolution, namely, that the distribution of the affected regions expands between weeks 2 and 5 after onset of facial paralysis. These observations and the findings of a literature review indicate that the SN, STNT, and vestibular nucleus are relatively prone to developing encephalitis after RHS.

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

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.

Cerebrospinal fluid viral load and biomarkers of neuronal and glial cells in Ramsay Hunt syndrome.

Reactivation of varicella zoster virus (VZV) can manifest with facial palsy diagnosed as Ramsay Hunt Syndrome (RHS) or Ramsay Hunt Syndrome zoster sine herpete (RHS-ZSH). These syndromes are associated with poor prognosis despite treatment with antivirals and corticosteroids. Concentrations of biomarkers such as neurofilament protein (NFL), S-100ß protein and glial fibrillary acidic protein (GFAp) have previously been measured in cerebrospinal fluid (CSF) to assess neuronal damage and glial pathology. We employed immunochemical methods to measure concentrations of NFL, S-100ß protein and GFAp in CSF from patients with RHS (n = 15) and RHS-ZSH (n = 13) diagnosed by detection of VZV DNA in the CSF by quantitative PCR, and compared with a control group (n = 52). The biomarker concentrations were correlated with CSF viral load and outcome measured by House-Brackmann score. NFL and GFAp concentrations were increased compared with controls (P = 0.008 and P = 0.04), while S-100ß levels were decreased. This pattern was more pronounced in patients with RHS compared to the patients with RHS-ZSH (NS and P = 0.028). The amount of viral DNA in CSF correlated with increased GFAp (P = 0.003) and NFL (P = 0.006). No correlations were found between biomarker concentrations and patient outcome. Patients with facial palsy caused by VZV had biochemical signs of neuronal damage and astrogliosis. High amounts of viral DNA may be associated with the degree of damage on neuronal and astroglial cells. Prospective studies are warranted to elucidate the association of elevated biomarkers in the CSF and outcome assessed by more sensitive tests.

Features of Audio-Vestibular Deficit and 3D-FLAIR Temporal Bone MRI in Patients with Herpes Zoster Oticus.

Herpes zoster oticus (HZO) is characterized by otalgia and erythematous vesicles in the auricle or external auditory canal. Ramsay Hunt syndrome (RHS) can be diagnosed when facial nerve palsy is accompanied by these symptoms of HZO, and in this case, audio-vestibular symptoms such as hearing loss or dizziness often develop. Recently, 3D-fluid-attenuated inversion recovery sequence (3D-FLAIR) magnetic resonance imaging (MRI) has been introduced in order to evaluate the inner ear structure pathology. The purpose of this study was to investigate the audio-vestibular characteristics in correlation with temporal bone MRI findings in HZO patients. From September 2018 to June 2022, 18 patients with HZO participated in the study. Thirteen patients (77%) showed high-signal intensity in the inner ear structures in 4 h post-contrast 3D-FLAIR images. In a bithermal caloric test, the lateral semicircular canal showed high signal intensity in 4 h post-contrast 3D-FLAIR images in 75% of patients with abnormal canal paresis. While the cochlea showed high signal intensity in 4 h post-contrast 3D-FLAIR images in 75% of patients with hearing loss, the vestibulo-cochlear nerve showed enhancement in post-contrast T1-weighted images in only 33% of patients with hearing loss. The present study demonstrates that audio-vestibular deficits are well-correlated with increased signal intensity of the inner ear endorgans in 4 h post contrast 3D-FLAIR MRI.

Mastoid effusion on temporal bone MRI in patients with Bell's palsy and Ramsay Hunt syndrome.

This study aimed to investigate the incidence of mastoid effusion on temporal bone magnetic resonance imaging (MRI) in patients with Bell's palsy (BP) and Ramsay Hunt syndrome (RHS), and evaluate the usefulness of mastoid effusion in early differential diagnosis between BP and RHS. The incidence of mastoid effusion on 3.0 T-temporal bone MRI, which was conducted within 10 days after the onset of acute facial nerve palsy, was compared between 131 patients with BP and 33 patients with RHS. Findings of mastoid cavity on temporal bone MRI were classified into three groups as normal mastoid, mastoid effusion, and sclerotic change, and the incidence of ipsilesional mastoid effusion was significantly higher in RHS than BP (P < 0.001). Tympanic membrane was normal in 7 of 14 RHS patients with mastoid effusion, and injected without middle ear effusion in 7 patients. This study highlights significantly higher incidence of ipsilesional mastoid effusion in RHS than BP, and suggests that the presence of mastoid effusion may provide additional information for differential diagnosis between RHS and BP.

A severe case of Ramsay Hunt Syndrome treated with acupuncture and related techniques.

Ramsay-Hunt syndrome presents with a vesicular eruption in the ear canal or oral cavity associated with ipsilateral peripheral facial paralysis. The cause is reactivation of the herpes zoster virus in the geniculate ganglion. It is the second most frequent cause of non-traumatic peripheral facial paralysis. Acupuncture is a medical procedure endorsed by the WHO with a wide range of indications. It consists of the application of very fine needles in certain points of the body to relieve pain and relieve certain diseases. We present a case of a young woman with unilateral facial paralysis as part of Ramsay Hunt syndrome. She received conventional treatment with acyclovir, analgesics, corticosteroids and eye protection measures 48 h after the onset of symptoms. At three weeks, due to the lack of improvement of the facial paralysis, manual acupuncture was started along with electroacupuncture, plum blossom hammer for facial stimulation and Chinese herbal medicine. An almost complete improvement was obtained at 14 weeks since the onset of the condition. Acupuncture and related techniques may be an effective intervention for this type of condition, and are associated with very few adverse effects.

Unusual presentation of Ramsay Hunt syndrome in renal transplant patients: case report and literature review.

Ramsay Hunt syndrome (RHS) is a rare manifestation of varicella zoster virus (VZV) infection that accounts for around 12% of all cases of facial paralysis. Although it is more common in immunosuppressed individuals, it has not been yet reported in kidney transplant recipients. We describe the case of a 41-year-old man with a history of renal transplant for whom the diagnosis and treatment of RHS were delayed owing to an unusual presentation. We also review the literature on VZV infection in renal transplant patients.

Ramsay Hunt syndrome in patients infected with human immunodeficiency virus.

Ramsay Hunt syndrome (RHS) is defined as herpes zoster infection of the head and neck that involves the facial nerve. Immunocompromised people, such as those infected with human immunodeficiency virus (HIV), are predisposed to herpes zoster. However, reports of RHS in patients with HIV are rare. We report two cases of RHS in patients with HIV at our hospital, located in southern Brazil. We hope this report will increase the awareness of this condition among doctors caring for patients with HIV.

Atypic geniculate neuralgia: atypic anatomic correlation of cranial nerve roots and AICA.

Geniculate neuralgia is a rare cause of craniofacial pains. The anterior inferior cerebellar artery is the offending vessel which compress nervus intermedius in the patients with typical geniculate neuralgia. We report a patient whose pain was atypical for either geniculate neuralgia and trigeminal neuralgia. At operation the anterior inferior cerebellar artery was coursing with the nerves and was separated. After the decompression the pain resolved immediately.

Nervus intermedius and the surgical management of geniculate neuralgia.

OBJECTIVE: Geniculate neuralgia (GN) is an uncommon craniofacial pain syndrome attributable to nervus intermedius (NI) dysfunction. Diagnosis and treatment can be challenging, due to the complex nature of ear sensory innervation, resulting in clinical overlap with trigeminal neuralgia (TN) and glossopharyngeal neuralgia (GPN). METHODS: A retrospective review of a prospective neurosurgical database at our institution was performed, 2000-2017, with a corresponding systematic literature review. Pain outcomes were dichotomized as unfavorable for unchanged/worsened symptoms versus favorable if improved/resolved. Eight formalin-fixed brains were examined to describe NI at the brainstem. RESULTS: Eleven patients were surgically treated for GN-9 primary, 2 reoperations. The median age was 48, 7 patients were female, and the median follow-up was 11 months (range 3-143). Seven had ≥ 2 probable cranial neuralgias. NI was sectioned in 9 and treated via microvascular decompression (MVD) in 2. Five patients underwent simultaneous treatment for TN (4 MVD; 1 rhizotomy) and 5 for GPN (3 MVD; 2 rhizotomy). Eleven reported symptomatic improvement (100%); 8 initially reported complete resolution (73%). Pain outcomes at last contact were favorable in 8 (73%)-all among the 9 primary operations (89% vs 0%, p = 0.054). Six prior series reported outcomes in 111 patients. CONCLUSIONS: GN is rare, and diagnosis is confounded by symptomatic overlap with TN/GPN. Directed treatment of all possible neuralgias improved pain control in almost all primary operations. Repeat surgery seems a risk factor for an unfavorable outcome. NI is adherent to superomedial VIII at the brainstem; the intermediate/cisternal portion is optimal for visualization and sectioning.

Correlation between MRI and operative findings in Bell's palsy and Ramsay Hunt syndrome.

PURPOSE: To investigate the correlation between gadolinium enhanced magnetic resonance image (MRI) results and surgical findings of facial nerves in Bell's palsy and Ramsay Hunt syndrome. MATERIALS AND METHODS: From 1995 to 2004, MRI was performed on 13 patients with Bell's palsy or Ramsay Hunt syndrome, who were offered with surgical decompression of the facial nerve through the middle cranial fossa approach. Gadolinium enhanced MRI was performed on all patients and the enhancement of the facial nerve was evaluated by radiology specialists. Operative findings including the degree of the facial nerve segment swelling were examined. Furthermore, the time interval from the onset of palsy to surgery was evaluated. RESULTS: Swelling of facial nerve segments was found in patients with enhanced facial nerves from MRI. The swelling of the facial nerve in the labyrinthine segment in particular was identified in all patients with enhanced labyrinthine segments in MRI. The intraoperative swelling of geniculate ganglion of facial nerve was found in 78% of patients with enhanced facial segment in MRI (p=0.01). The intraoperative swelling of tympanic segment was observed from fourth to ninth weeks after the onset of palsy. CONCLUSION: MRI enhancement of facial nerves in Bell's palsy and Ramsay Hunt syndrome is associated with the extent of intratemporal lesions of facial nerves, especially in the labyrinthine segment.

Usefulness of High-Resolution 3D Multi-Sequences for Peripheral Facial Palsy: Differentiation Between Bell's Palsy and Ramsay Hunt Syndrome.

OBJECTIVE: To investigate the usefulness of magnetic resonance imaging (MRI) including three-dimensional (3D) sequences in the differentiation between Bell's palsy (BP) and Ramsay Hunt syndrome (RHS). STUDY DESIGN: A prospective study. SETTING: Tertiary care center. PATIENTS: Twenty patients: 15 patients with BP and five patients with RHS. INTERVENTION: Diagnostic. MAIN OUTCOME MEASURE: Clinical diagnosis (BP or RHS). RESULTS: The presence of hyperintensity on 3D-fluid-attenuated inversion recovery sequence (3D-FLAIR) and enhancement on gadolinium-enhanced (CE)-3D-FLAIR and CE-3D-T1-weighted image (3D-T1WI) along the internal auditory canal (IAC) wall were significantly associated with RHS (p < 0.05). Hyperintensity in the inner ear was observed on pre- and postcontrast 3D-FLAIR, and enhancement of the cranial nerve (CN)-VIII was observed only on CE-3D-FLAIR. The presence of these findings also showed significant relationships with RHS (p < 0.05). Moreover, thickening of the CN-VII in the fundus of the IAC in 3D-constructive interference on steady state sequence (3D-CISS) also showed a significant association with RHS (p < 0.05). In contrast, the presence of hyperintensity of the CN-VII in the fundus of the IAC on 3D-FLAIR did not demonstrate a significant relationship (p = 0.95), and enhancement in this region was observed in all cases on CE-3D-FLAIR and gadolinium-enhanced-three-dimensional-T1-weighted gradient echo sequence (CE-3D-T1WI). CONCLUSIONS: 3D MRI sequences are useful for differentiating RHS from BP. In particular, the enhancement in the CN-VIII and/or along the IAC wall are valuable findings, and CE-3D-FLAIR is the most useful sequence to evaluate these findings. Thickening of the CN-VII on 3D-CISS is also an important finding.

The lesion site of vestibular dysfunction in Ramsay Hunt syndrome: a study by click and galvanic VEMP.

Ramsay Hunt syndrome (RHS) is characterized by vestibulocochlear dysfunction in addition to facial paralysis and auricular vesicles. The present study investigated the lesion site of vestibular dysfunction in a group of 10 RHS patients. Caloric testing, vestibular evoked myogenic potentials by click sound (cVEMP) and by galvanic stimulation (gVEMP) were used to assess the function of the lateral semicircular canal, saccule, and their afferents. The results of caloric testing (all 10 cases showed canal paresis) mean the existence of lesion sites in lateral semicircular canal and/or superior vestibular nerve (SVN). Abnormal cVEMPs in 7 patients mean the existence of lesions in saccule and/or inferior vestibular nerve (IVN). Four of the 6 patients with absent cVEMP also underwent gVEMP. The results of gVEMP (2 absent and 2 normal) mean that the former 2 have lesions of the vestibular nerve, and the latter 2 have only saccular lesions concerning the pathway of VEMPs. Thus, our study suggested that lesion sites of vestibular symptoms in RHS could be in the vestibular nerve and/or labyrinth, and in SVN and/or IVN. In other words, in the light of vestibular symptoms, there is the diversity of lesion sites.

Prompt contrast enhancement of cerebrospinal fluid space in the fundus of the internal auditory canal: observations in patients with meningeal diseases on 3D-FLAIR images at 3 Tesla.

We speculated that meningeal pathologies might facilitate the permeability of cranial nerves at the fundus of the internal auditory canal (IAC), causing prompt enhancement after administration of Gd-DTPA. Using a 3D- fluid-attenuated inversion recovery (FLAIR) sequence, we evaluated the enhancement of the cerebrospinal fluid (CSF) space in the IAC fundus 10 min after Gd-DTPA administration in patients with meningeal diseases. Twenty patients (aged 22 to 79 years) were divided into 2 groups, a group with meningeal disease comprising 9 patients with meningeal abnormalities (6, tumor dissemination; 3, infection) and a control group of 11 patients with unilateral IAC pathology whose healthy sides were included as controls. Six of the 9 patients in the group with meningeal disease showed bilateral enhancement; one showed unilateral enhancement. None of the control group showed enhancement in the healthy side. One patient with Ramsay-Hunt syndrome showed only ipsilateral enhancement. Enhancement in the IAC fundus was frequently observed in patients with meningeal disease, even just 10 min after administration of contrast agent. This enhancement in the IAC fundus was never visible on T1-weighted 3D-FLASH images.

Ramsay Hunt syndrome associated with brain stem enhancement.

Postcontrast T1-weighted MR images in a patient with Ramsay Hunt syndrome showed an enhancing lesion in the region of the nucleus of the pontine facial nerve and abnormal enhancement of the intrameatal, labyrinthine, and tympanic facial nerve segments and of the geniculate ganglion, as well as enhancement of the vestibulocochlear nerve and parts of the membranous labyrinth. This enhancement most probably resulted from a primary neuritis of the intrameatal nerve trunks of the seventh and eighth cranial nerves.