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.

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.

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.

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.

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.

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.

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.

An unexpected case of Ramsay Hunt syndrome: case report and literature review.

BACKGROUND: Ramsay Hunt Syndrome (RHS) is a rare, severe complication of varicella zoster virus (VZV) reactivation in the geniculate ganglion. Facial paralysis is one of the features and without treatment, it fully recovers in as little as 20% of cases; this is much improved if treatment is started within 72 hours. This case is noteworthy in that coexistence of facial palsy with cervical dermatome involvement by VZV is not typical of RHS. Yet, it has been reported before. CASE PRESENTATION: A 54 year old Caucasian woman presented with right ear discomfort, difficulty hearing and a vesicular rash along the pinnae, 8 days after the eruption of a similar rash in the right C2-C4 dermatomes. 2 days later, she awoke with a partial right-sided facial paralysis, which improved with treatment (valacyclovir and prednisone). CONCLUSIONS: This case is most pertinent to Family Practice, Otolaryngology and Neurology. It highlights the possible co-existence of RHS with cervical VZV reactivation and encourages physicians to monitor for this complication even before geniculate ganglion reactivation occurs. RHS is a rare disease that can present with vague symptoms. A high index of suspicion and close follow up are essential. Early intervention with antivirals and corticosteroids has shown significantly improved outcomes in these patients.

[Ramsay Hunt syndrome in a 13-year-old boy]. / Ramsay Hunts syndrom hos en 13-årig dreng.

We describe a formerly healthy 13-year-old boy who was admitted with symptoms of epiphora from his left eye over a period of four days and of left-sided peripheral facial palsy for one day. There was no history of trauma or tick bites. Cutaneous vesicles were observed in the ipsilateral ear. Pleocytosis was found and positive polymerase chain reaction for varicella zoster virus was obtained in samples from the vesicles and the cerebrospinal fluid. A magnetic resonance imaging confirmed neuritis of the left facial nerve. The patient was treated with a combination of aciclovir and prednisolone for ten days. On follow-up one week later, full recovery had been obtained.

Contralateral botulinum toxin injection to improve facial asymmetry after acute facial paralysis.

HYPOTHESIS: The application of botulinum toxin to the healthy side of the face in patients with long-standing facial paralysis has been shown to be a minimally invasive technique that improves facial symmetry at rest and during facial motion, but our experience using botulinum toxin therapy for facial sequelae prompted the idea that botulinum toxin might be useful in acute cases of facial paralysis, leading to improve facial asymmetry. BACKGROUND: In cases in which medical or surgical treatment options are limited because of existing medical problems or advanced age, most patients with acute facial palsy are advised to await spontaneous recovery or are informed that no effective intervention exists. The purpose of this study was to evaluate the effect of botulinum toxin treatment for facial asymmetry in 18 patients after acute facial palsy who could not be optimally treated by medical or surgical management because of severe medical or other problems. METHODS: From 2009 to 2011, nine patients with Bell's palsy, 5 with herpes zoster oticus and 4 with traumatic facial palsy (10 men and 8 women; age range, 22-82 yr; mean, 50.8 yr) participated in this study. Botulinum toxin A (Botox; Allergan Incorporated, Irvine, CA, USA) was injected using a tuberculin syringe with a 27-gauge needle. The amount injected per site varied from 2.5 to 3 U, and the total dose used per patient was 32 to 68 U (mean, 47.5 +/- 8.4 U). RESULT: After administration of a single dose of botulinum toxin A on the nonparalyzed side of 18 patients with acute facial paralysis, marked relief of facial asymmetry was observed in 8 patients within 1 month of injection. Decreased facial asymmetry and strengthened facial function on the paralyzed side led to an increased HB and SB grade within 6 months after injection. CONCLUSION: Use of botulinum toxin after acute facial palsy cases is of great value. Such therapy decreases the relative hyperkinesis contralateral to the paralysis, leading to greater symmetric function. Especially in patients with medical problems that limit the medical or surgical treatment options, botulinum toxin therapy represents a useful alternative.

Geniculate neuralgia: clinical, radiologic, and intraoperative correlates.

OBJECTIVE: Geniculate neuralgia is an uncommon pain syndrome that can be severe and disabling and is difficult to diagnose. METHODS: The literature was reviewed for geniculate neuralgia, including anatomy, presentation, and treatment. A case illustration was presented that demonstrates the novel brainstem functional imaging findings for geniculate neuralgia. A 39-year-old man presented with a history of left "deep" ear pain within his ear canal. He noted occasional pain on the left side of his face around the ear. He had been treated with neuropathic pain medications without relief. His wife described suicidal ideations discussed by her husband because of the intense pain. RESULTS: The patient's neurologic examination was normal, and otolaryngologic consultation revealed no underlying structural disorder. Anatomic imaging revealed a tortuous vertebral artery-posterior inferior cerebellar artery complex with the posterior inferior cerebellar artery loop impinging on the root entry zone of the nervus intermedius-vestibulocochlear nerve complex and just inferior to the root entry zone of the facial nerve and a small anterior inferior cerebellar artery loop interposed between the cranial nerve VII-VIII complex and the hypoglossal and glossopharyngeal nerves. A left-sided retromastoid craniotomy was performed, and the nervus intermedius was transected. An arterial loop in contact with the lower cranial nerves at the level of the brainstem was mobilized with a polytetrafluoroethylene implant. CONCLUSIONS: The patient indicated complete relief of his preoperative pain after surgery. He has remained pain-free with intact hearing and balance.