Ramsey Hunt Research Articles

Ramsey Hunt Syndrome: A Review Article

Ramsey Hunt Syndrome: A Review Article

Ramsey Hunt syndrome in the child: case report and treatment approach

Herpes zoster is a manifestation of the reactivation of an infection after chickenpox. One of the manifestations of herpes zoster is Ramsey Hunt syndrome, which develops when the ganglion of the VII pair of cranial nerves is involved in process. Diagnosis of Ramsey Hunt syndrome is often based on a combination of a rash in the outer ear and peripheral paresis of the facial muscles. The article describes a rare clinical case of herpes zoster in a child without clinical signs of immunodeficiency, with the development of unilateral paralysis of the facial muscles, pleocytosis in the cerebrospinal fluid. Therapy was performed with a combination of acyclovir and high doses of methylprednisolone with a good clinical effect. It is first mentioning of deviations in the immune status (decreased concentration of the number of markers of naive T‑lymphocytes (TREC)) were revealed, which could be associated with the development of herpes zoster in a child.

Persistent hiccups as a rare presentation of Ramsey Hunt syndrome in a renal transplant recipient

Ramsay Hunt syndrome type 2, also known as RHS and herpes zoster oticus, is a disorder that is caused by the reactivation of varicella zoster virus in the geniculate ganglion, a nerve cell bundle of the facial nerve. RHS typically presents with inability to move many facial muscles, pain in the ear, taste loss on the front of the tongue, dry eyes and mouth, and a vesicular rash. However, the association of RHS with multiple cranial nerve involvement and persistent hiccups is less well reported.

A Case Report on a Patient With Unrecovered Bell’s Palsy of 2 Years Duration Treated With Non-Interventional Pulsed Radio Frequency Electrical Current

Patents with unrecovered Bell’s palsy or a mono facial paresis from other conditions that affect the facial nerve as in Ramsey Hunt disorder, iatrogenicity or disease, often find that their condition causes long term paresis in the facial muscles with functional, psychological and social impacts upon their lives. Previous treatments to the local inflammation occurring in the facial nerve as it exits the stylomastoid foramen with ultrasound, non-stimulating electrical currents and electrical functional muscle stimulation of the compromised facial muscles, often do not restore full function to the affected side of the face. A non-interventional pulsed radio frequency (NI-PRF) electrical current has been observed to stimulate the facial nerve with fasciculations occurring in the main branch of the VII th nerve and its concomitant branches to the upper, middle and lower areas of the face resulting in improved motor function. This commences immediately within the first treatment in acute Bell’s Palsy and only 4-6 treatments are required to assist most patients. Prolonged conditions that were previously thought unrecoverable (after years) have restored motor control and this usually occurs between 1 – 3 months of treatment. Treatment requisite does not have to be continuous and can be given twice weekly or even once weekly over the period mentioned above. Even if treatment is interrupted the condition continues to improve. This would have both an economic and efficient time saving impact on these patients. The main case history of an unrecovered Bell’s palsy of 2 years duration is discussed in this report and demonstrates marked improvement after only six treatments. Electroacupuncture is often included in the above treatments as an adjunct to increase circulation and improve muscle activity

The acute vestibular dysfunction in emergency neurology

The aim of this study is to research the structure of acute vestibular disorders at the stage of the emergency room and to determine the frequency of occurrence of life-threatening conditions.
 Material and methods. It was made the analysis of acute vestibular disorders at the stage of emergency room of the Neurology department of Clinical Hospital No. 7, City Clinical Hospital No. 18 and stroke center of Interregional Clinical Diagnostic Center in Kazan, Tatarstan Republic, Russian Federation, for the period 20162020. The diagnosis of vestibular disorders was established on the basis of clinical data. There were additional instrumental researches made in doubtful cases. Statistical data processing was made using Microsoft Excel 10.0 program.
 Results. The study included 106 patients in total with reliably established pathology of the peripheral or central vestibular system. The peripheral or functional nature of vestibular disfunctions were identified in 84% patients. 57% patients had benign paroxysmal positional vertigo. 42% of these patients had repeated benign paroxysmal positional vertigo attack. Vestibular neuronitis and Ramsey Hunt syndrome were detected in 15% patients. Acute cerebrovascular origin was diagnosed in 10% patients (10 ischemic strokes and 1 transient ischemic attack). 1 patient had an ArnoldChiari malformation with the development of occlusive hydrocephalus. So, 12 patients had a life-threatening cause of vestibular pathology, among them only 2 patients had isolated vestibular symptoms. Vestibular migraine was diagnosed in another 5% patients.
 Conclusions. Life-threatening causes of vestibular pathology account for 11% of all cases. Focal neurological symptoms are the main red flags of systemic vertigo. Benign paroxysmal positional vertigo is detected in 57% of cases of vertigo in the emergency room.

Modern approaches to drug treatment for vestibular vertigo

In recent years, some progress has been achieved in elaborating the algorithms and standards for the treatment of many conditions accompanied by vertigo. The current possibilities of treating vestibular vertigo consist of a gradually expanding arsenal of symptomatic and pathogenetic drugs. Among the drugs used for the symptomatic treatment of vestibular vertigo, there are vestibular suppressants (antihistamines, benzodiazepines, and calcium antagonists) and antiemetics (dopamine antagonists and serotonin 5-HT3 receptor antagonists). The paper discusses the possibilities of using betahistine and vitamin D as pathogenetic agents for recurrent benign paroxysmal positional vertigo; diuretics, betahistine (including the new prolonged release formulation Betaserc® Long), glucocorticoids, and gentamicin for Meniere's disease; triptans, beta-blockers, tricyclic antidepressants, and anticonvulsants for vestibular migraine; glucocorticoids, antiviral agents, and drugs that accelerate vestibular compensation for acute unilateral peripheral vestibulopathy (vestibular neuritis and Ramsey Hunt syndrome).The emergence of new drugs, as well as the design of new dosage forms that enhance patient adherence to the prescribed treatment, can improve quality of life in patients suffering from diseases that have recently led to long-term disability or even incapacitation.

Ramsay Hunt Syndrome: A case report and literature review.

Background A case report of Ramsey Hunt Syndrome which is rare in dental clinic, often mis-diagnosed and not extensively documented in literature. Case presentation A case of 60-year-old woman who presented with left sided pain initially diagnosed as toothache with offending tooth extracted. However, two days after patient started having prodromal symptoms of fever, malaise followed with left facial rash, tinnitus, rash in the eternal auditory meatus, palatal and buccal painful ulcers. There was also left facial weakness, patient was treated with antiviral and steroid to good effect after 5 weeks. Conclusion: This case is not commonly seen in dental clinic. It highlights the possible mis-diagnosed cases of herpes zoster infection presenting as RHS. A high index of suspicion and close follow-up is essential.

術前化学療法中にRamsay Hunt症候群を発症し，播種性帯状疱疹・髄膜脳炎へ重症化をきたした進行食道癌の1例

術前化学療法中にRamsay Hunt症候群を発症し，播種性帯状疱疹・髄膜脳炎へ重症化をきたした進行食道癌の1例

Pontine Infarct Camouflaged as Bell’s Palsy

Facial palsy is a neurological disorder triggered by dysfunction of the seventh cranial nerve, categorized as either central, between the cerebral cortex and brainstem nuclei, or peripheral, between the brainstem nuclei and peripheral organs. Central lesions cause impairment of the contralateral lower facial musculature with associated sparing of the forehead and ocular muscles. Conversely, peripheral lesions produce ipsilateral whole-sided facial hemiplegia including both forehead and ocular muscles. Facial palsy is diagnosed clinically, while imaging studies and additional subsidiary testing (e.g. electromyography, nerve conduction studies), serologies, and rarely biopsy can assist in confirming or refuting the working diagnosis. The differential diagnosis comprises Bell’s palsy (idiopathic), HIV infection, Ramsey Hunt syndrome, Lyme, sarcoidosis, amyloidosis, acoustic neuroma, parotid gland tumor, temporal bone biopsy, otitis media, Guillain-Barre syndrome, and brainstem infarct. Facial palsy is branded as Bell’s palsy if one of the aforementioned etiologies is not identified as the root cause. Herein, we report the case of a 58-year-old male, who presented with left facial weakness involving both the upper and lower face, posterior circulation symptoms in the setting of hypertensive emergency. Initial magnetic resonance imaging (MRI) was unrevealing for any acute process, however given lack of improvement repeat imaging was ordered. The patient was ultimately confirmed to have an acute versus subacute pontine infarct where the seventh and eighth cranial nerve exits at the cerebellopontine angle. Ancillary laboratory studies were non-contributory. Subsequently, the patient’s symptoms continued to improve, blood pressure control was achieved, and was consequently discharged on goal-directed medical therapy. J Neurol Res. 2019;9(1-2):14-17 doi: https://doi.org/10.14740/jnr524

Pontine Infarct Camouflaged as Bell’s Palsy

Facial palsy is a neurological disorder triggered by dysfunction of the seventh cranial nerve, categorized as either central, between the cerebral cortex and brainstem nuclei, or peripheral, between the brainstem nuclei and peripheral organs. Central lesions cause impairment of the contralateral lower facial musculature with associated sparing of the forehead and ocular muscles. Conversely, peripheral lesions produce ipsilateral whole-sided facial hemiplegia including both forehead and ocular muscles. Facial palsy is diagnosed clinically, while imaging studies and additional subsidiary testing (e.g. electromyography, nerve conduction studies), serologies, and rarely biopsy can assist in confirming or refuting the working diagnosis. The differential diagnosis comprises Bell’s palsy (idiopathic), HIV infection, Ramsey Hunt syndrome, Lyme, sarcoidosis, amyloidosis, acoustic neuroma, parotid gland tumor, temporal bone biopsy, otitis media, Guillain-Barre syndrome, and brainstem infarct. Facial palsy is branded as Bell’s palsy if one of the aforementioned etiologies is not identified as the root cause. Herein, we report the case of a 58-year-old male, who presented with left facial weakness involving both the upper and lower face, posterior circulation symptoms in the setting of hypertensive emergency. Initial magnetic resonance imaging (MRI) was unrevealing for any acute process, however given lack of improvement repeat imaging was ordered. The patient was ultimately confirmed to have an acute versus subacute pontine infarct where the seventh and eighth cranial nerve exits at the cerebellopontine angle. Ancillary laboratory studies were non-contributory. Subsequently, the patient’s symptoms continued to improve, blood pressure control was achieved, and was consequently discharged on goal-directed medical therapy. J Neurol Res. 2019;9(1-2):14-17 doi: https://doi.org/10.14740/jnr524

Novel pre-therapeutic scoring system using patient and haematological data to predict facial palsy prognosis.

We describe a novel scoring system, the facial Palsy Prognosis Prediction score (PPP score), which we test for reliability in predicting pre-therapeutic prognosis of facial palsy. We aimed to use readily available patient data that all clinicians have access to before starting treatment. Multicenter case series with chart review. Three tertiary care hospitals. We obtained haematological and demographic data from 468 facial palsy patients who were treated between 2010 and 2014 in three tertiary care hospitals. Patients were categorised as having Bell's palsy or Ramsey Hunt's palsy. We compared the data of recovered and unrecovered patients. PPP scores consisted of combinatorial threshold values of continuous patient data (eg platelet count) and categorical variables (eg gender) that best predicted recovery. We created separate PPP scores for Bell's palsy patients (PPP-B) and for Ramsey Hunt's palsy patients (PPP-H). The PPP-B score included age (≥65 years), gender (male) and neutrophil-to-lymphocyte ratio (≥2.9). The PPP-H score included age (≥50 years), monocyte rate (≥6.0%), mean corpuscular volume (≥95 fl) and platelet count (≤200 000 /μL). Patient recovery rate significantly decreased with increasing PPP scores (both PPP-B and PPP-H) in a step-wise manner. PPP scores (ie PPP-B score and PPP-H score) ≥2 were associated with worse than average prognosis. Palsy Prognosis Prediction scores are useful for predicting prognosis of facial palsy before beginning treatment.

RASH DECISIONS–DERMATOLOGICAL MANIFESTATIONS PRECEDING A PROGRESSIVE NEUROLOGICAL SYNDROME

We describe the case of a 72 year old woman who presented with sequential cranial nerve palsies preceded by irritation, pain and a vesicular rash in the sensory distributions of...

Ramsey Hunt syndrome — a case report

Case report: An 82-year-old woman with a history of hypertension and diabetes mellitus was presented to the Emergency Room (ER) because of left facial weakness of sudden onset and pain on the mastoid region. She had a history of a recent viral upper airway infection. On examination, she had a peripheral left facial nerve paralysis, with no other abnormalities. She was diagnosed with a Bell's Palsy and discharged from the ER with a prescription of prednisolon and recommendation for eye care measures.

Case presentation of soft tissue parapharyngeal chondroma in a pediatric patient

Soft tissue chondromas are uncommon benign tumors found mostly in the hands and feet and rarely reported in the pediatric population. In this case presentation we describe a 10year old boy who had an MRI for facial paralysis due to Ramsey Hunt Syndrome, which incidentally revealed a parapharyngeal mass. He underwent transoral resection of the mass without complication, and histopathology confirmed the diagnosis of soft tissue chondroma. This case is unique due to the unusual location of the tumor and its presentation in a child.

Varicella-zoster virus meningo-rhombencephalitis presenting as Ramsey Hunt

A 45-year-old woman on etanercept for rheumatoid arthritis presented with an acute right Bell palsy and auricular herpetic rash (Ramsey Hunt syndrome). Despite IV acyclovir, she developed headache, vertigo, severe vomiting, and evidence of a right peripheral audiovestibular loss (figure 1, A and B, video 1 on the Neurology ® Web site at [www.neurology.org][1]). Ongoing symptoms indicated CSF examination, which showed a pleocytosis and positive varicella-zoster PCR, and an MRI that displayed abnormal brainstem signal (figure 2, A and B). Although there was unequivocal peripheral audiovestibular loss,1 the persistence of vertigo beyond 3 days is a red flag suggesting central involvement,2 here presumably resulting from the known effect of etanercept on impairing leukocyte migration3 into the brain. Acknowledgment: The authors thank Luca Seemungal for video editing. The authors report no disclosures relevant to the manuscript. Go to Neurology.org for full disclosures. [1]: http://www.neurology.org

Ramsey Hunt Syndrome with Dysphagia with MRI Evidence of Brain Stem and Upper Cervical Cord Involvement (P02.264)

Ramsey Hunt Syndrome with Dysphagia with MRI Evidence of Brain Stem and Upper Cervical Cord Involvement (P02.264)

Facial Nerve Paralysis in Children: A Report of 29 Cases

We analysed 29 children diagnosed as having facial nerve paralysis at the Departments of Otorhinolaryngology or Pediatrics of Tokyo Medical University Hospital between January 2000 and December 2009. The most common cause was Bell’s palsy (18 cases). The other cases were Ramsey Hunt’s syndrome (5 cases), congenital (4 cases), traumatic (1 case) and otitis media (1 case). Only ten patients were able to undergo electroneurography (ENoG) and those children were all over six years old. The results of ENoG were from 12% to 62.2%. Even when the ENoG score was low, the clinical outcome was good. There was no relationship between the ENoG score and the prognosis in this study. All patients except those with congenital facial palsy were treated with steroids, and the palsy improved in all of them.

Facial Nerve Decompression Surgery with Gelatin Containing bFGF

Objective: Basic fibroblast growth factor (bFGF) promotes the regeneration of denervated nerves. Our experimental study suggested that bFGF-impregnated biodegradable gelatin hydrogel facilitates regeneration of the facial nerve in guinea pigs due to the sustained release of bFGF. Clinically, this therapeutic regimen may be useful for facial nerve decompression surgery. Method: Twenty-five patients with severe facial paralysis were treated with the new procedure. The facial nerve was decompressed tympanic and mastoid portions via mastoid. A bFGF-impregnated biodegradable gelatin hydrogel was placed around the exposed nerve. Regeneration of the facial nerve was evaluated once per month by observing the facial movement. Results: Patients were followed until complete recovery occurred, or for more than 6 months in cases with a poor prognosis. All patients with severe Bell’s palsy and Ramsey Hunt syndrome showed early recuperation and milder sequelae compared to conventional decompression surgery. Recovery from the palsy was defined as House-Brackmann grading 1 and 2. The rates of recovered patients with novel surgery and conventional surgery were 92.0% (n = 25) and 70.7% (n = 58), respectively; the former was significantly better than the latter ( P < .05). Conclusion: This is the first study to reveal the efficacy of a bFGF-impregnated biodegradable gelatin hydrogel as a drug-delivery medium in the paralyzed facial nerve surgery. Advantages of this decompression surgery are low risk of hearing impediment and long effective period after onset of paralysis.

Causes and time-course of vertigo in an ear, nose, and throat clinic

The purpose of this study is to review etiologies and identify the time-course of vertigo presenting in an ear, nose, and throat clinic, and serve as a reference guide for other clinics. The study includes retrospective chart review in a tertiary care, university hospital. The patient data with reported ICD-10 codes as causes of vertigo between April 2005 and December 2007 were extracted from the database. At each visit, the main diagnosis as to etiology, characteristics of the vertigo, its time-course, and patient demographic data were recorded. Of 547 cases, 17 diagnoses were made in 73.9%. Diagnostic categories included peripheral vertigo 72.9%, central vertigo 0.8%, psychogenic cause 0.2%, and unknown 26.1%. Common causes of vertigo were benign paroxysmal positional vertigo (BPPV) 52.5%, Meniere's disease 14.6%, and sudden idiopathic hearing loss 2.9%. Less common diagnoses were benign paroxysmal vertigo of childhood 0.7%, labyrinthitis 0.7%, and vestibular schwannoma 0.3%. Rare conditions were delayed endolymphatic hydrops, Ramsey Hunt syndrome, otosyphilis, vestibular neuritis, temporal bone fracture, post-concussion syndrome, cerebellar infarction, epilepsy, cervical vertigo, Streptococcus suis meningitis, and psychogenic vertigo. Ninety-nine cases who reported remission of vertigo during the study period had median onset of the remission at 4 weeks. In the ear, nose, and throat clinic at Chiang Mai University, a tertiary university hospital, peripheral vestibular disorders were the main etiology of vertigo. The three most common causes were BPPV, Meniere's disease, and sudden idiopathic hearing loss. Half of the cases who returned for follow up had remitted symptoms within 4 weeks.

A History of Stroke: Its Recognition and Treatment

In any field, it is difficult to know where you are going unless you know where you have been.<i>A History of Stroke</i>is most timely. Interest, research, and knowledge about cerebrovascular disease and stroke patients have increased exponentially within the past 35 years. The history is brief enough to be reasonably digestible. Most of the contributions occurred within the lifetime and recall of the senior author, Dr Bill Fields, and his long-time associate, collaborator, and coauthor, Dr Noreen Lemak. Dr Fields has himself contributed much to the field of stroke and has been a very active principal, observer, and interpreter of this rapidly changing scene. This is a concise, 200-page account published as a hardbound, compact book that easily fits into a laboratory coat pocket or a handbag. The volume begins with a short chapter summarizing early stroke history from Hippocrates (400 BC) to Ramsey Hunt (1915). Then various