Ramsay Hunt syndrome

The strict definition of the Ramsay Hunt syndrome is peripheral facial nerve palsy accompanied by an erythematous vesicular rash on the ear (zoster oticus) or in the mouth. J Ramsay Hunt, who described various clinical presentations of facial paralysis and rash, also recognised other frequent symptoms and signs such as tinnitus, hearing loss, nausea, vomiting, vertigo, and nystagmus. He explained these eighth nerve features by the close proximity of the geniculate ganglion to the vestibulocochlear nerve within the bony facial canal. Hunt's analysis of clinical variations of the syndrome now bearing his name led to his recognition of the general somatic sensory function of the facial nerve and his defining of the geniculate zone of the ear. It is now known that varicella zoster virus (VZV) causes Ramsay Hunt syndrome. Compared with Bell's palsy (facial paralysis without rash), patients with Ramsay Hunt syndrome often have more severe paralysis at onset and are less likely to recover completely. Studies suggest that treatment with prednisone and acyclovir may improve outcome, although a prospective randomised treatment trial remains to be undertaken. In the only prospective study of patients with Ramsay Hunt syndrome, 14% developed vesicles after the onset of facial weakness. Thus, Ramsay Hunt syndrome may initially be indistinguishable from Bell's palsy. Further, Bell's palsy is significantly associated with herpes simplex virus (HSV) infection. In the light of the known safety and effectiveness of antiviral drugs against VZV or HSV, consideration should be given to early treatment of all patients with Ramsay Hunt syndrome or Bell's palsy with a 7-10 day course of famciclovir (500 mg, three times daily) or acyclovir (800 mg, five times daily), as well as oral prednisone (60 mg daily for 3-5 days). Finally, some patients develop peripheral facial paralysis without ear or mouth rash, associated with either a fourfold rise in antibody to VZV or the presence of VZV DNA in auricular skin, blood mononuclear cells, middle ear fluid, or saliva. This indicates that a proportion of patients with "Bell's palsy" have Ramsay Hunt syndrome zoster sine herpete. Treatment of these patients with acyclovir and prednisone within 7 days of onset has been shown to improve the outcome of recovery from facial palsy.     

Early diagnosis of zoster sine herpete and antiviral therapy for the treatment of facial palsy

The effect of antiviral agents on recovery from facial palsy in patients with zoster sine herpete (ZSH; varicella zoster virus reactivation without zoster) has not been evaluated because ZSH is difficult to diagnose early after onset. In this study, all 13 patients who received acyclovir-prednisone treatment within 7 days of onset, as confirmed by a positive PCR result, showed complete recovery. PCR-based early diagnosis of ZSH and antiviral therapy elicited an excellent outcome for recovery from facial palsy due to ZSH.     

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.     

Herpes zoster oticus and facial paralysis (Ramsay Hunt syndrome). Clinico-pathologic study and review of literature

Two patients with herpes zoster oticus and facial paralysis are presented, in 1 of whom the geniculate ganglion showed at autopsy unequivocal pathological changes consistent with previous herpetic inflammation. The other patient showed a large area of sensory loss to pain and touch in the concha and external acoustic meatus during recovery from the acute episode. The clinical and pathological features are discussed in the light of a review of the literature. We conclude that the term “Ramsay Hunt syndrome” should be used only for cases of herpes oticus with facial paralysis and that herpetic geniculate ganglionitis and facial neuritis represent its anatomic substrate.     

Ramsay Hunt syndrome in children

In a retrospective study, 52 children were diagnosed with Ramsay Hunt syndrome. The facial palsy was milder and complete recovery of the function was achieved in 78.6% of patients. Associated cranial neuropathies were less common in children than in adults. The timing of vesicle appearance tended to be delayed in children. In preschool children, Ramsay Hunt syndrome was rare, although the frequency has recently increased. The syndrome is relatively common in older children. This study suggested that vaccination can prevent or reduce the occurrence of Ramsay Hunt syndrome.     

Successful response of non-recovering Ramsay Hunt syndrome to intravenous high dose methylprednisolone

Ramsay Hunt syndrome (RHS) is a frequent cause of facial palsy. It is a consequence of the infection of geniculate ganglion by herpes zoster or herpes simplex virus. In the lack of randomized controlled trials, RHS is empirically treated by a combination therapy of antiviral agents and steroids given orally. However, RHS has, per se, a poorer prognosis than idiopathic facial palsy (Bell's palsy). We describe a case series of two patients with RHS unsuccessfully treated with antiviral drugs and oral corticosteroids, showing an almost complete recovery after late administration of intravenous (i.v.) high dose methylprednisolone. Both patients had all recognized negative prognostic factors including age of onset, a high grade facial weakness, absence of R1 and R2 response at blink reflex test, and in the first case, the involvement of greater superficial petrosal nerve. We propose that i.v. high dose methylprednisolone should be considered, even as a late treatment option, in patients with RHS non recovering after standard antiviral and oral steroid therapy as well as presenting clinical features suggestive of a poor prognosis.     

Electrophysiologic findings and prognosis in Bell's palsy

Electrophysiologic investigations were carried out on 45 patients with Bell's palsy at periodic intervals after the onset of paralysis. It was found that there was a good correlation between prognosis in Bell's palsy and the amplitude of evoked motor response obtained after six or more days of clinical paresis. When the average amplitude of evoked motor response was within normal limits (i.e., 504μV or greater), complete recovery with no residual deficits took place two to six weeks after the onset of facial palsy. When the evoked motor response was absent in all three major branches of the facial nerve, indicating complete nerve degeneration, electromyographic signs of recovery were apparent by the third or fourth month after the onset of paralysis. In these cases, recovery was relatively slow and incomplete, with some degree of residual deficit and synkinesis. Maximal return of voluntary facial movement was established 8 to 12 months after the initial symptom. When the mean amplitude of evoked motor response was below the lower limit of normal (i.e., less than 504μV), electromyographic signs of recovery were noted within 1 to 3 months, depending on the amplitude values. The final outcome of this intermediate group was similar, but not identical, to that of the previous group. The prognosis of facial paralysis in Bell's palsy was thus found to be directly related to the mean amplitude of evoked motor response, regardless of the extent of clinical paralysis.     

Acyclovir responsive brain stem disease after the Ramsay Hunt syndrome

We report an immunocompetent patient with the Ramsay Hunt syndrome (RHS) followed days later by brainstem disease. Extensive virological studies proved that varicella zoster virus (VZV) was the causative agent. Treatment with intravenous acyclovir resulted in prompt resolution of all neurological deficits except peripheral facial palsy. This case demonstrates that after geniculate zoster, brainstem disease may develop even in an immunocompetent individual and effective antiviral therapy can be curative.     

Facial nerve palsy with acute otitis media during the first 2 weeks of life

A full-term male newborn presented to us at the age of 2 weeks with left facial weakness that had started at the age of 4 days and steadily progressed over a 10-day period. Physical examination revealed a complete unilateral left peripheral facial nerve paralysis, with an erythematous dull and bulging left tympanic membrane. Computed tomography and magnetic resonance imaging were normal. The patient underwent left myringotomy and placement of a pneumatic equalization tube within 24 hours of presentation. He also received a 5-day course of prednisone and a 2-week course of antibiotics and acyclovir. Improvement of the facial paralysis was rapid, with near-complete resolution at the time of discharge home and complete resolution 6 weeks later. To our knowledge, this is the only reported case of acute otitis media with associated facial nerve palsy in a newborn during the first 2 weeks of life.     

神经肌电图对特发性面瘫治疗及预后评估的研究

目的探讨神经肌电图(神经电图electroneurography,ENG和肌电图electromyography,EMG)在特发性面瘫治疗及预后评估中的价值。方法采用丹麦生产的keypointⅣ肌电图仪对96例确诊为特发性面瘫的患者进行患侧与健侧ENG、EMG检测,分别记录患侧面神经颞支、颧支、颊支的运动传导潜伏期、波幅以及所支配的额肌、眼轮匝肌、口轮匝肌的肌电图情况,并与自身健侧作对比。结果特发性面瘫患者患侧面神经运动传导潜伏期延长、M波波幅降低,与自身健侧相比,差异有统计学意义(P<0.05)。波幅下降<70%、NCV(神经传导速度)减慢<20%、EMG大致正常的轻度患者,3个月内面肌完全恢复,治愈率100%;波幅下降70%～90%、NCV减慢20%～50%、EMG呈部分神经源性损害的中度患者,3个月内大部分可完全恢复,治愈率87.8%;波幅下降>90%、NCV减慢>50%、EMG呈部分或完全神经源性损害的中重度患者,3个月内面肌完全恢复者为50%;诱发电位消失、NCV引不出、EMG呈完全神经源性损害的重度患者,3个月内面肌完全恢复者为25%。结论 ENG和EMG检测对特发性面瘫的神经损伤、面肌恢复等预后评估有重要价值,能对临床治疗提供客观依据。     

经皮神经电刺激对急性面瘫患者神经恢复的影响

目的探讨经皮神经电刺激联合药物治疗对急性面瘫患者神经恢复的影响。方法济源市中医院2015-04-2017-06收治的124例急性面瘫患者为研究对象,采用经皮神经电刺激联合药物治疗的66例患者为观察组,常规治疗的58例患者为对照组,治疗2周后评价2组患者的临床症状及面神经功能。结果治疗后观察组额纹消失、鼻唇沟消失及鼓腮漏气患者相比治疗前明显减少(P0.05),对照组仅鼓腮漏气患者较治疗前明显减少(P0.05);组间比较治疗后观察组额纹消失、眼睑闭合露白及鼓腮漏气患者均较对照组明显减少(P0.05);治疗后观察组面神经功能分级人数分布与对照组相比差异具有统计学意义(P0.05)。结论经皮神经电刺激可在常规治疗的基础上有效改善面神经功能的恢复,进而提高其临床治疗效果。     

Facial palsy in Kawasaki syndrome

Facial nerve palsy, a very rare complication of Kawasaki syndrome, has been reported in only 25 patients. We treated a 12-week-old boy with bilateral coronary artery aneurysms due to Kawasaki syndrome who developed marked unilateral peripheral facial nerve palsy on day 36 of illness. None of the 25 previously reported patients with this complication were treated with immunoglobulin; they required 7 to 90 days to recover. In our patient, treatment with this agent was associated with complete resolution of facial nerve palsy within 36 hours. Review of prior cases demonstrates that children with Kawasaki-associated facial nerve palsy have more than twice the risk for coronary artery aneurysm (52% vs <25%) as that of children who do not develop this neurological complication. Unexplained facial nerve paralysis in young children with a prolonged febrile illness should provoke consideration of Kawasaki syndrome and of echocardiography to exclude coronary artery aneurysms. Although facial palsy appears likely to resolve in all patients that survive the acute phase of Kawasaki syndrome, treatment with intravenous immunoglobulin appears to considerably shorten the time to full recovery and provides an important clue to the mechanisms of neurological injury in this illness.     

Prednisolone and valaciclovir in Bell's palsy: a randomised, double-blind, placebo-controlled, multicentre trial

BACKGROUND: Previous trials of corticosteroid or antiviral treatments for Bell's palsy have been underpowered or have had insufficient follow-up. The aim of this study was to compare the short-term and long-term effects of prednisolone and valaciclovir in the recovery of the affected facial nerve in a large number of patients. METHODS: In this randomised, double-blind, placebo-controlled, multicentre trial, patients aged 18 to 75 years who sought care directly or were referred from emergency departments or general practitioners within 72 h of onset of acute, unilateral, peripheral facial palsy, between May, 2001, and September, 2006, were assessed. Patients were randomly assigned in permuted blocks of eight to receive placebo plus placebo; 60 mg prednisolone per day for 5 days then reduced by 10 mg per day (for a total treatment time of 10 days) plus placebo; 1000 mg valaciclovir three times per day for 7 days plus placebo; or prednisolone (10 days) plus valaciclovir (7 days). Follow-up was for 12 months. The primary outcome event was time to complete recovery of facial function, as assessed with a regional Sunnybrook scale score of 100 points. Analysis was by modified intention to treat. This study is registered with ClinicalTrials.gov, number NCT00510263. FINDINGS: Of 839 patients who were randomly assigned, 829 were included in the modified intention-to-treat analysis: 206 received placebo plus placebo, 210 prednisolone plus placebo, 207 valaciclovir plus placebo, and 206 prednisolone plus valaciclovir. Time to recovery was significantly shorter in the 416 patients who received prednisolone compared with the 413 patients who did not (hazard ratio 1.40, 95% CI 1.18 to 1.64; p<0.0001). There was no difference in time to recovery between the 413 patients treated with valaciclovir and the 416 patients who did not receive valaciclovir (1.01, 0.85 to 1.19; p=0.90). The number of patients with adverse events was similar in all treatment arms. INTERPRETATION: Prednisolone shortened the time to complete recovery in patients with Bell's palsy, whereas valaciclovir did not affect facial recovery.     

面肌诱发电位在巨大听神经瘤术中面神经保护的研究及预后分析

目的 探讨经头皮颅骨刺激面肌诱发电位（TCeMEP）在巨大听神经瘤切除术中的面神经保护作用。方法 对28例巨大听神经瘤术中监测TCeMEP．并比较其变化和术后面肌功能的关系。结果 16例术后TCeMEP下降大于75％，其中9例明显面瘫，7例轻度面瘫；12例术后TCeMEP下降小于75％，其中2例明显面瘫，7例轻度面瘫，3例基本正常。两组明显面瘫率经x^2检验，P〈0．05。结论 TCeMEP可以术中实时监测全部面肌传导通路的功能．为术中保护面神经功能提供可靠的电传导依据。TCeMEP波幅下降75％可以作为术后可能发生明显面瘫的警戒点。     

不同类固醇激素应用方式治疗小儿面神经麻痹的临床研究

目的探讨不同肾上腺皮质激素应用方式治疗儿童面神经麻痹的临床效果差异。方法将50例面神经麻痹患儿随机分为2组,一组给予大剂量甲基强的松龙静脉冲击治疗,另一组给予常规强的松口服治疗作为对照,比较2组患儿治疗后恢复时间、治愈率及不良反应情况。结果治疗组患儿平均恢复时间（14.08±5.9）d,而对照组为（19.48±8.32）d,治疗2周后治疗组患儿治愈率68%,而对照组为40%,两者差异有统计学意义（P〈0.05）。2组不良反应发生率差异无统计学意义。结论大剂量甲基强的松龙冲击治疗儿童面神经麻痹效果明显,症状改善迅速,未见不良反应增加。     

Bell's palsy in children: analysis of clinical findings and course

To evaluate treatment of Bell's palsy (acute idiopathic peripheral facial nerve paralysis) of children, the authors analyzed 38 cases (18 females, 20 males) of Bell's palsy in children aged below 16 years old. The mean age of all cases was 6.8 +/- 6.2 years old. All cases resulted in complete recovery within 6 months. Clinical score of facial motor functions were adapted to 17 patients who were more than 5 years old. They were divided into two groups: early recovery group (clinical symptoms recovered within 3 months; 10 cases) and later recover group (over 3 months; 7 cases). Clinical scores evaluated in the first week from the onset were not significantly different. Steroid therapy was used for 9 patients of early group and 6 patient of later group. All patients of this study were recovered, thus we could not evaluate effect of steroid therapy for Bell's palsy in children. Use of steroid therapy for Bell's palsy needs more concretely administration. We consider how the region locates near to the center is an important prognostic factor.     

Recovery of gait after radiotherapy in paralytic patients with metastatic epidural spinal cord compression

We report on 15 patients with paralysis of the legs caused by metastatic epidural spinal cord compression. After radiation therapy 6 patients regained motor function, 5 of whom recovered ambulatory function, but the recovery was delayed for 3 months or more. The duration of the paralysis prior to treatment varied from 20 hours to 10 days with no significant difference between the group with and the group without recovery. The median time from the initial motor symptoms to total paralysis was longer (45 days) in the patients who recovered compared with those permanently paralyzed (9 days). The response to radiation therapy seems to depend on the rate of loss of spinal cord function rather than the length of total paralysis. We recommend active treatment of patients with paraplegia due to metastatic epidural spinal cord compression, even when the paralysis has been present for over a week.     

Nicolaus A. Friedreich's description of peripheral facial nerve paralysis in 1798.

In 1798, Nicolaus A. Friedreich of Wurzburg published a detailed clinical account of three patients with idopathic peripheral facial nerve paralysis. His astute observations of onset, physical findings, natural course, treatment, and recovery preceded those of Charles Bell by 23 years.     

Androgen induced acceleration of functional recovery after rat sciatic nerve injury

PURPOSE: Testosterone (T) treatment accelerates recovery from facial paralysis after facial nerve crush in hamsters. In this study, we extended those studies to another injury model and asked the following question: Will T treatment accelerate recovery from lower limb paralysis following sciatic nerve crush in the rat? METHODS: Castrated adult male rats received a right side sciatic nerve crush at the level of the sciatic notch, with the left side serving as control. Half the animals received a subcutaneous implant of a propionated form of T (TP), the others were sham-implanted. Weekly testing using the Sciatic Functional Index (SFI), a quantitative measure of locomotion, was done for 7 weeks postoperative (wpo). RESULTS: Between 3 and 5 weeks post-op, the average SFI score of the TP-treated group was higher than controls. This difference was significant at 4 wpo, indicating an accelerated degree of functional recovery. At these timepoints, the differences were attributable to the footprint or paw length and associated with calf muscle reinnervation rather than the toespreading component associated with intrinsic foot muscle rein-nervation. Beyond 5 wpo, there were no differences in the SFI scores. CONCLUSION: The results indicate that, as with facial nerve regeneration in the hamster, testosterone accelerates functional recovery from hind limb paralysis following sciatic nerve injury in the rat. While the responses of spinal motoneurons to injury can differ from those of cranial motoneurons, in this case it appears that they share a similar response to the trophic actions of androgen. This is important in the context of designing therapeutic strategies for dealing with direct trauma to motoneurons resulting from both peripheral and central nervous system trauma, such as spinal cord injury.     

Orbicularis oculi-Reflexe und Summenpotentiale des Orbicularis oris bei idiopathischer Facialislähmung

In 84 patients with idiopathic, clinically complete Bell's palsy the electrically induced blink reflexes with their two components (OOR I and II) were electromyographically recorded on both sides using skin electrodes. In 67 of these patients the evoked responses of the orbicularis oris muscle were also studied. The latencies and amplitudes were measured and related to the clinical outcome of the facial paralysis. The patients were divided into two groups, one with good recovery of the palsy (46 patients), the other with significant residual paresis and/or strong associated movements of the facial musculature (38 patients). In the group with good recovery the following results were obtained: 1. the OOR I remained elicitable or reappeated during the first 12 days after the onset of palsy; 2. the OOR II began to rise during the first 10 days of palsy; 3. the amplitude of the orbicularis oris response did not decrease to below 10%. In the group with poor recovery: 1. both components of the OOR were absent or diminished to below 4% for more than 12 days after the onset of palsy; 2. the latency difference of the OOR I exceeded 8 msec; 3. the amplitude of the orbicularis oris responses decreased to below 10%. Using these criteria it appears to be possible in about 85% of patients to make a prognosis between the 3rd to 5th and the 10th to 12th day after the onset of Bell's palsy.