Abstract
Purpose
Patients with Ramsay Hunt syndrome have a poorer prognosis than those with Bell palsy despite the use of various treatment modalities. We compared the clinical characteristics, treatment methods, and outcomes in patients with Ramsay Hunt syndrome and Bell palsy.
Materials and Methods
Patients with Ramsay Hunt syndrome were compared with patients with Bell palsy treated using oral steroids and with those treated with both steroids and an antiviral agent. Functional recovery of the facial nerve was scored according to the House-Brackmann grading system. Patients were followed up until recovery or for 3 months. Recovery rates in each group were assessed by age, sex, and initial and last House-Brackmann grade.
Results
Compared with patients with Bell palsy, those with Ramsay Hunt syndrome were generally younger, had initially more severe facial palsy, and a lower recovery rate. Various factors including initial House-Brackmann grade, starting time to treatment, age, comorbid disease, electroneurography, and electromyography showed some correlations with prognosis in all groups. The addition of antiviral agents to an oral steroid regimen did not improve the recovery rate of patients with Bell palsy.
Conclusion
Patients with Ramsay Hunt syndrome have a poorer prognosis than do those with Bell palsy.
1
Introduction
Facial palsy, which occurs annually in 30 of 100 000 individuals in the general population, may have various causes, ranging from head trauma to an idiopathic episode . Early diagnosis and accurate treatment for patients with idiopathic facial palsy (Bell palsy) and Ramsay Hunt syndrome may accelerate recovery and prevent possible complications. Among the agents most frequently used to treat these conditions are steroids, vasodilators, multivitamins, and antiviral agents. Steroid has an anti-inflammatory effect; thus, an enhanced recovery can be achieved in patients with steroid treatment in Bell palsy and Ramsay Hunt syndrome . In addition, the treatment for patients with Ramsay Hunt with antiviral agents may prevent disease progression and improve prognosis by inhibiting the spread of herpes zoster virus . Recently, antiviral agents have also been used to treat patients with Bell palsy because of the mechanism of viral infection of the nervous system; yet, the efficacy of such agents remains unclear.
Previous studies have shown worse disease progression and poorer prognosis in patients with Ramsay Hunt syndrome than in those with Bell palsy. However, in the cited studies , patients were compared under different conditions in that patients with Bell palsy had been treated with steroid, whereas those with Ramsay Hunt syndrome had received both steroid and antiviral agent. To accurately compare the 2 groups of patients, it is necessary to treat the patients in a same setting. We therefore compared clinical characteristics, treatment methods, and outcomes of patients with Ramsay Hunt syndrome and Bell palsy treated with steroid and antiviral agents.
2
Materials and methods
The study population consisted of 115 patients with Ramsay Hunt syndrome and 202 patients with Bell palsy between 2001 and 2009. Patients were diagnosed with Ramsay Hunt syndrome if they had peripheral facial palsy or lesions on the external auditory canal or the vesicles around the ear. Patients were diagnosed with Bell palsy if no other cause of facial palsy could be identified. Data recorded included sex, age, time from first onset to initial treatment, and the presence of any underlying disease such as hypertension and/or diabetes mellitus. The House-Brackmann scale was used to assess initial facial nerve impairment (ie, the worst grade evident after facial palsy had developed) and final facial nerve impairment (measured 3 months after onset). All patients underwent electroneurography (ENoG) after 4 days of palsy onset, and all were explored using electromyography (EMG) 2 weeks after the onset. The extent of nerve function on the affected side was compared with that on the normal side, and recovery was defined as attainment of House-Brackmann grade I or II. Based on the results of ENoG and EMG, 2 patients underwent facial decompression and were therefore excluded from the study because the treatment modalities used differed from those used for other patients.
All patients were admitted for physical and psychologic tests and were given a high-protein and low-salt diet. Adults with Bell palsy were treated with high-dose oral methylprednisolone (80 mg/d for 4 days, 60 mg/d for 2 days, 40 mg/d for 2 days, 20 mg/d for 2 days, and 10 mg/d for 5 days) with or without oral acyclovir (2400 mg/d for 5 days). Children, adolescents, and underweight adults were treated with 1 mg/kg per day methylprednisolone for 4 days and with reduced doses of the drug for the next 6 days. In patients with Ramsay Hunt syndrome, the dose of oral methylprednisolone was the same as that in patients with Bell palsy (80 mg oral methylprednisolone per day for 4 days, 60 mg/d for 2 days, 40 mg/d for 2 days, 20 mg/d for 2 days, and 10 mg/d for 5 days), but the dosage of oral acyclovir was relevant to treat herpes zoster infection (4000 mg/d for 7 days). Because steroids can seriously affect normal glycometabolism and vascular function, patients with diabetes or hypertension were carefully monitored in terms of blood glucose level and blood pressure. Diabetic patients received the same steroid course as prescribed for those without diabetes, under appropriate medical care (ie, monitoring of insulin administration), after consulting an endocrinologist. Data from the 3 patient groups were compared using Kruskal-Wallis, χ 2 , and nonparametric t tests to assess factors associated with complete recovery. All statistical analyses were performed using SPSS (version 12.0; SPSS Inc, Chicago, IL), and P < .05 was considered statistically significant.
2
Materials and methods
The study population consisted of 115 patients with Ramsay Hunt syndrome and 202 patients with Bell palsy between 2001 and 2009. Patients were diagnosed with Ramsay Hunt syndrome if they had peripheral facial palsy or lesions on the external auditory canal or the vesicles around the ear. Patients were diagnosed with Bell palsy if no other cause of facial palsy could be identified. Data recorded included sex, age, time from first onset to initial treatment, and the presence of any underlying disease such as hypertension and/or diabetes mellitus. The House-Brackmann scale was used to assess initial facial nerve impairment (ie, the worst grade evident after facial palsy had developed) and final facial nerve impairment (measured 3 months after onset). All patients underwent electroneurography (ENoG) after 4 days of palsy onset, and all were explored using electromyography (EMG) 2 weeks after the onset. The extent of nerve function on the affected side was compared with that on the normal side, and recovery was defined as attainment of House-Brackmann grade I or II. Based on the results of ENoG and EMG, 2 patients underwent facial decompression and were therefore excluded from the study because the treatment modalities used differed from those used for other patients.
All patients were admitted for physical and psychologic tests and were given a high-protein and low-salt diet. Adults with Bell palsy were treated with high-dose oral methylprednisolone (80 mg/d for 4 days, 60 mg/d for 2 days, 40 mg/d for 2 days, 20 mg/d for 2 days, and 10 mg/d for 5 days) with or without oral acyclovir (2400 mg/d for 5 days). Children, adolescents, and underweight adults were treated with 1 mg/kg per day methylprednisolone for 4 days and with reduced doses of the drug for the next 6 days. In patients with Ramsay Hunt syndrome, the dose of oral methylprednisolone was the same as that in patients with Bell palsy (80 mg oral methylprednisolone per day for 4 days, 60 mg/d for 2 days, 40 mg/d for 2 days, 20 mg/d for 2 days, and 10 mg/d for 5 days), but the dosage of oral acyclovir was relevant to treat herpes zoster infection (4000 mg/d for 7 days). Because steroids can seriously affect normal glycometabolism and vascular function, patients with diabetes or hypertension were carefully monitored in terms of blood glucose level and blood pressure. Diabetic patients received the same steroid course as prescribed for those without diabetes, under appropriate medical care (ie, monitoring of insulin administration), after consulting an endocrinologist. Data from the 3 patient groups were compared using Kruskal-Wallis, χ 2 , and nonparametric t tests to assess factors associated with complete recovery. All statistical analyses were performed using SPSS (version 12.0; SPSS Inc, Chicago, IL), and P < .05 was considered statistically significant.
3
Results
Of the 202 patients with Bell palsy, 110 (50 men and 60 women; mean age, 54.2 ± 16.7 years) were treated with both oral steroid and antiviral agent (group A), and 92 (43 men and 49 women; mean age, 53.1 ± 13.5 years) received oral steroid alone (group B). In addition, 155 patients with Ramsay Hunt syndrome (70 men and 85 women; mean age, 47.2 ± 16.5 years) were treated with oral steroid and antiviral agent (group C). Patients with Ramsay Hunt syndrome were significantly younger than those with Bell palsy ( P < .05). Moreover, we found that women were more susceptible than men to both conditions.
The mean initial House-Brackmann grades of groups A and B were 3.75 ± 0.7 and 3.74 ± 0.8, respectively ( P > .05). with Ramsay Hunt syndrome (group C) was 4.35 ± 0.7, significantly lower than that in patients with Bell palsy ( P < .05). Similarly, the final House-Brackmann grade was similar in groups A and B but was significantly lower in group C ( P < .05). Of the 110 patients in group A, 46 (41.8%), 44 (40%), and 20 (18.1%) had initial House-Brackmann grades of III, IV, and V or more, respectively; of the 92 in group B, 48 (52.2%), 20 (21.7%), and 20 (21.7%) had initial House-Brackmann grades of III, IV, and V or more, respectively. Of the 155 patients in group C, 24 (15.5%), 53 (34.2%), and 78 (50.3%) had initial House-Brackmann grades of III, IV, and V or more, respectively.
3.1
Comparison of recovery rate
When we compared recovery rate among the 3 groups, the levels were 86.4% in group A, 80.4% in group B (82.7% of the 202 patients with Bell palsy), and 58.7% in group C. The recovery rate of patients with Ramsay Hunt syndrome was significantly lower than in those with Bell palsy ( P < .05). In contrast, the addition of antiviral agent to the steroid did not affect the recovery rate among patients with Bell palsy ( P > .05) ( Fig. 1 ).
We also found that poorer initial status was associated with lower recovery rate. The recovery rates of patients with Bell palsy and Ramsay Hunt syndrome who had initial House-Brackmann grade V or more were 61.9% and 43.6%, respectively. In patients with initial House-Brackmann grade V or more, however, the addition of antiviral agent to the steroid significantly improved recovery rate ( P < .05) ( Table 1 ).
| Initial H-B grade | Recovery rate (%) | Time to commencement of treatment | Recovery rate (%) | ||||
|---|---|---|---|---|---|---|---|
| Bell palsy | Ramsay Hunt (C) | Bell palsy | Ramsay Hunt (C) | ||||
| Steroid + antiviral agent (A) | Steroid (B) | Steroid + antiviral agent (A) | Steroid (B) | ||||
| III | 40/46 (87) | 43/48 (89.5) | 22/24 (91.7) | <1 wk | 74/84 (88) ⁎⁎ | 44/52 (84.6) † | 56/90 (62.2) |
| IV | 36/44 (81.1) ⁎⁎ | 16/20 (80) † | 35/53 (66) | 1–2 wk | 20/25 (80) ⁎⁎ | 25/33 (75.5) † | 28/54 (51.9) |
| V, VI | 15/20 (75) ⁎ , ⁎⁎ | 15/24 (62.5) | 34/78 (43.6) | >2 wk | 1/1 (100) | 5/7 (71.4) | 7/11 (63.6) |
⁎ Statistically significant between cohorts A and B.
⁎⁎ Statistically significant between cohorts A and C.
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