Clinical spectrum and prognosis of neurological complications of reactivated varicella-zoster infection: the role of immunosuppression.

Immunosuppressed patients are at higher risk for developing herpes zoster (HZ), and neurological complications are frequent in them. However, the influence of immunosuppression (IS) on the severity and prognosis of neurological complications of varicella-zoster virus (VZV) reactivation is unknown. We studied retrospectively patients with neurological complications due to VZV reactivation who attended our hospital between 2004 and 2019. We aimed to assess the clinical spectrum, potential prognostic factors, and the influence of the immune status on the severity of neurological symptoms. A total of 98 patients were included (40% had IS). Fifty-five patients (56%) had cranial neuropathies which included Ramsay-Hunt syndrome (36 patients) and cranial multineuritis (23 patients). Twenty-one patients developed encephalitis (21%). Other diagnosis included radiculopathies, meningitis, vasculitis, or myelitis (15, 10, 6, and 4 patients, respectively). Mortality was low (3%). At follow-up, 24% of patients had persistent symptoms although these were usually mild. IS was associated with severity (defined as a modified Rankin scale greater than 2) (odds ratio, 4.23; 95% confidence interval, 1.74-10.27), but not with prognosis. Shorter latency between HZ and neurologic symptoms was the only factor associated with an unfavorable course (death or sequelae) (odds ratio, 0.82; 95% confidence interval, 0.71-0.95). In conclusion, the clinical spectrum of neurological complications in VZV reactivation is wide. Mortality was low and sequelae were mild. The presence of IS may play a role on the severity of neurological symptoms, and a shorter time between HZ and the onset of neurological symptoms appears to be a negative prognostic factor.

Should acupuncture therapy be used for acute facial paralysis? A protocol for systematic review.

BACKGROUND: Peripheral facial paralysis (PFP) results in functional disorder and social dysfunction, when it is under a severe condition at onset, long-term poor outcomes do occur. Different acupuncture methods have been reported to be potentially effective for shortening the disease course and reducing the occurrence of sequelae when they are applied at an early stage. Neuro edema is a common pathological feature in the acute phase, and many clinical studies have suggested its effect of reducing facial nerve edema. It is of value to estimate the effectiveness and safety of acupuncture treatment at the onset, and to assess the most suitable acupuncture method for the acute period. METHODS AND ANALYSIS: All the RCTs and quasi-RCTs on acupuncture therapy for patients who is during acute stage of PFP will be included. The recovery rate of facial function, the time it takes to restore facial function and the odds of sequelae occurring will be the key parts we focus on. Psychological well-being and quality of life will also be evaluated. Literature searching will be conducted until December 31th, 2022 from eight databases systematically. Two reviewers will screen the literature and extract the data independently. RevMan software will be used for data analysis, and the version 2 of the Cochrane risk-of-bias tool (RoB 2) will be used to assess the certainty of evidence. Forest plots and summary findings will be generated. If data permits, a meta-analysis will be conducted. ETHICS AND DISSEMINATION: Since this study will not involve clinical treatment of patients, ethics approval is not required. The result of this study will be submitted to a peer-reviewed journal for publication and as a proposal for clinical practice and further study on acupuncture treatment at the early stage of PFP. DISCUSSION: This review will summarize the evidence on the different type of acupuncture therapy for acute Bell's palsy and Ramsay-Hunt syndrome. We anticipate that it would be safe and effective when applied to the acute phase of PFP, and some specific suitable acupuncture methods would be found resulting from this review. SYSTEMATIC REVIEW REGISTRATION: International Prospective Register for Systematic Reviews (PROSPERO) number CRD42020205127.

[Case of atypical Ramsay-Hunt syndrome who presented with severe vertigo and vomiting].

We herein present a case of 6-year-old female demonstrating atypical Ramsay-Hunt syndrome. She presented with an earache, severe vertigo, and vomiting at onset, and thereafter, herpes zoster oticus appeared. No facial nerve palsy was seen. She was vaccinated for varicella and had no past history of apparent varicella infection. The patient showed positive IgG and negative IgM serum antibodies for varicella-zoster virus (VZV). An analysis of VZV-DNA from the patient's ear lesion using alleric discrimination real-time PCR identified a wild-type strain of VZV. We diagnosed her to have atypical Ramsay-Hunt syndrome caused by reactivation of the VZV. Aciclovir and prednisolone were administered, and she recovered completely. This case indicates that Ramsay-Hunt syndrome could be caused by a VZV infection after vaccination, even though its frequency is low. Ramsay-Hunt syndrome may thus be considered as one of causes of sudden onset vertigo in children.

Ramsay Hunt syndrome with multiple cranial neuropathies in a liver transplant recipient.

Ramsay Hunt syndrome is an infection of the head and neck caused by varicella zoster virus involving the facial nerve; less commonly, other cranial nerves might be involved. We report a case of Ramsay Hunt syndrome in an immune compromised patient, with classic facial nerve palsy and ipsilateral ear vesicles, which rapidly evolved to involve multiple cranial neuropathies, and improved dramatically with antiviral therapy and corticosteroids. Varicella zoster virus should be considered as a cause of multiple cranial neuropathies in an immune compromised patient, and abrupt treatment with acyclovir should be initiated once this diagnosis is suspected.

Ramsay Hunt syndrome presenting in primary care.

Ramsay Hunt syndrome is a lower motor neurone weakness of the seventh (facial) cranial nerve caused by reactivation of the herpes zoster virus. The virus infects the geniculate ganglion of the nerve causing facial weakness. The onset of a motor neuropathy makes it inherently different from the more typical presentation of shingles, which predominantly causes a sensory neuropathy. Around 20-30% of individuals will be affected by herpes zoster during their lives. Ramsay Hunt syndrome is a rare disease, affecting fewer than 1 in 1500 people in the United States. The syndrome can present with vague and non-specific symptoms, especially during early onset of the disease. A high index of suspicion and frequent follow up are imperative for early diagnosis. A vesicular erythematous rash on, or around, the pinna (zoster oticus) may be present. Advanced age, diabetes mellitus and hypertension appear to be significant poor prognostic features with patients more likely to suffer complications. There is clear evidence to support the use of early antiviral therapy along with oral corticosteroid use. Early and appropriate intervention has resulted in significant improvement in treatment and complication outcomes.

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.

Ramsay Hunt syndrome with pontine lesion: A case report.

RATIONALE: The coexistence of Ramsay Hunt syndrome (RHS) and varicella-zoster virus (VZV) encephalitis is rare. A patient who developed RHS after being infected with VZV, along with a pontine lesion, is reported in the present study. PATIENT CONCERNS: A 41-year-old male patient presented with his mouth askew for 7 days, and dizziness, accompanied by hearing loss for 3 days. DIAGNOSES: The patient was initially diagnosed with RHS. Brainstem encephalitis was confirmed by lumbar puncture and cerebrospinal fluid. Brain magnetic resonance imaging (MRI) and diffusion-weighted imaging (DWI) revealed how VZV entered the intracranial space along the vestibulocochlear nerve and facial nerve in the acute period. INTERVENTIONS: Intravenous acyclovir, IV, immunoglobulins (IVIg) and methylprednisolone were administered. OUTCOMES: The herpes was cleared up and left facial nerve palsy was improved, but hearing loss in the left ear did not improve. LESSONS: An MRI was necessary for some VZV infections limited to the cranial nerve, although there was no evidence of brain stem injury. DWI provided evidence, showing how VZV entered the brain in the early stage. This allowed the doctor to judge the necessity of a lumbar puncture.

[Craniofacial neuralgias]. / Kraniofacijalne neuralgije.

Craniofacial neuralgias are characterized by sudden paroxysmal pain along the distribution of one or more of the cranial or upper cervical spinal nerves. The most significant neuralgia of the craniofacial region is trigeminal neuralgia, while geniculate neuralgia, glossopharyngeal neuralgia and occipital neuralgia are less common. Trigeminal neuralgia may be primary or secondary. Idiopathic trigeminal neuralgia or tic douloureux has been recognized for centuries as an extremely painful disorder most commonly involving the maxillary nerve. Recurrent lancinating, shocklike unilateral pain lasting for seconds to minutes is provoked by non noxious stimulation of the skin at specific sites around the face and less frequently by movement of the tongue. The trigger zones are usually within the same dermatome as the painful sensation. After each episode, there is usually a refractive period during which stimulation of the trigger zone will not induce pain. Idiopathic trigeminal neuralgia occurs somewhat more frequently in women and usually begins in individuals 50 to 70 years of age. There is no pain between attacks, and the frequency of painful episodes can range from several per day to only a few per year. With time, the features may become more atypical, with greater areas of more enduring and dull pain and occasionally bilateral pain, rarely on both sides simultaneously. No sensory or reflex deficit is detectable by routine neurologic testing. Diagnostic local anesthetic blocks will identify the specific nerves involved and the trigger point distribution. Neurologic and neuroradiologic examination is advised in all cases to rule out diseases such as intracranical tumors, vascular malformations or multiple sclerosis.

Typical or Atypical Ramsay-Hunt Syndrome in Delayed Facial Palsy After Stapedectomy?

OBJECTIVES: The aim of this study was to define the typical pattern for varicella zoster virus (VZV) reactivation in delayed facial palsy (DFP) after stapedectomy for otosclerosis. MATERIALS AND METHODS: Review of the relevant literature, personal casistics, and case-report. RESULTS: In total, 48 cases of DFP after stapes surgery have been described so far, including the reported case with exclusive manifestation of atypical Ramsay Hunt syndrome (RH); in the personal series of 1253 stapedectomies, DFP occurred in only one case (0.08%). Complete DFP (House-Brackmann grade VI) rapidly developed 12 days after surgery; RH appeared 2 days later, confirming the role of VZV. The DFP started improving after 8 weeks and completely recovered 6 months later. CONCLUSION: Acute otalgia prior to DFP should raise the suspicion of VZV reactivation. Atypical RH is the most frequent pattern that occurs in DFP after stapedectomy.

A population-based study of the incidence and complication rates of herpes zoster before zoster vaccine introduction.

OBJECTIVE: To establish accurate, up-to-date, baseline epidemiological data for herpes zoster (HZ) before the introduction of the recently licensed HZ vaccine. METHODS: Using data from January 1, 1996, to October 15, 2005, we conducted a population-based study of adult residents (Greater than or equal to 22 years) of Olmsted County, MN, to determine (by medical record review) the incidence of HZ and the rate of HZ-related complications. Incidence rates were determined by age and sex and adjusted to the US population. RESULTS: A total of 1669 adult residents with a confirmed diagnosis of HZ were identified between January 1, 1996, and December 31, 2001. Most (92%) of these patients were immunocompetent and 60% were women. When adjusted to the US adult population, the incidence of HZ was 3.6 per 1000 person-years (95% confidence interval, 3.4-3.7), with a temporal increase from 3.2 to 4.1 per 1000 person-years from 1996 to 2001. The incidence of HZ and the rate of HZ-associated complications increased with age, with 68% of cases occurring in those aged 50 years and older. Postherpetic neuralgia occurred in 18% of adult patients with HZ and in 33% of those aged 79 years and older. Overall, 10% of all patients with HZ experienced 1 or more nonpain complications. CONCLUSIONS: Our population-based data suggest that HZ primarily affects immunocompetent adults older than 50 years; 1 in 4 experiences some type of HZ-related complication.

Facial palsy after dental procedures - Is viral reactivation responsible?

OBJECTIVES: Herpes labialis viral reactivation has been reported following dental procedures, but the incidence, characteristics and outcomes of delayed peripheral facial nerve palsy following dental work is poorly understood. Herein we describe the unique features of delayed facial paresis following dental procedures. MATERIALS AND METHODS: An institutional retrospective review was performed to identify patients diagnosed with delayed facial nerve palsy within 30 days of dental manipulation. Demographics, prodromal signs and symptoms, initial medical treatment and outcomes were assessed. RESULTS: Of 2471 patients with facial palsy, 16 (0.7%) had delayed facial paresis following ipsilateral dental procedures. Average age at presentation was 44 yrs and 56% (9/16) were female. Clinical evaluation was consistent with Bell's palsy in 14 (88%) and Ramsay-Hunt syndrome in 2 patients (12%). Patients developed facial paresis an average of 3.9 days after the dental procedure, with all individuals developing a flaccid paralysis (House Brackmann (HB) grade VI) during the acute stage. 50% of patients developed persistent facial palsy in the form of non-flaccid facial paralysis (HBIII-IV). CONCLUSION: Facial palsy, like herpes labialis, can occur in the days following dental procedures and may also be related to viral reactivation. In this small cohort, long-term facial outcomes appear worse than for spontaneous Bell's palsy.

Postoperative Ramsay-Hunt syndrome after acoustic neuroma resection. Viral reactivation.

The aim of this paper is to present a patient suffering from acoustic neuroma and operated on with immediate postoperative hearing and facial function preservation who developed delayed Ramsay-Hunt syndrome. To our knowledge, this is the first case in whom a postoperative delayed facial palsy and hearing loss occurred. The patient gave an history of previously diagnosed herpes zoster reactivation limited to chest one-year before. This is undoubtdetly a predisposing factor for development of delayed facial palsy. It must not be underestimated and it obliges to consider a prophylaxis. Theoretically, the prophylactic antiviral therapy might prevent the evolution towards the herpes zoster oticus or reduce the severity of the symptoms allowing the preservation of the hearing function. It would be pointed out that the delayed facial plasy has favourable prognosis, while the hearing impairment may recover with a greater difficulty even after an antiviral treatment as in our case.

Herpes zoster oticus following mandibular block.

Although a few cases of facial palsy following mandibular nerve block and dental surgery have been described, it would appear that herpes zoster oticus following dental surgery has not been documented. It is possible that the latent virus may be activated by the mandibular nerve block and dental surgical interventions. Two cases of herpes zoster oticus, both following inferior alveolar nerve block anaesthesia for dental treatment are presented.

[Four cases of pediatric Ramsay Hunt syndrome].

Four cases of the Ramsay Hunt syndrome were admitted to our hospital during the two years from February 1997 to January 1999. Though one of the 4 patients had been immunized with varicella vaccine, the causative virus was not a vaccine strain but a wild-type strain. These patients were not suffering from underlying diseases. Because the number of pediatric zoster patient without underlying diseases who visited our clinic between 1981 and 1999 was 35 cases, the Ramsay Hunt syndrome turned out not to be extremely rare even among children having no underlying diseases. The prognosis of the Ramsay Hunt syndrome is assumed to be good if the treatment begins at the early stage. To begin the treatment at the early stage, it is necessary to confirm the diagnosis with virological examinations.