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J Med Sci 2004;24(1):37-42 http://jms.ndmctsgh.edu.tw/2401037.

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Cheng-Wen Ho, et al.

The Intervention of Rehabilitation Therapy on the Treatment of Ramsay Hunt Syndrome


Cheng-Wen Ho1, and Kao-Chung Tsai1,2* Department of Physical Medicine and Rehabilitation, Tri-Service General Hospital, 2National Defense Medical Center, Taipei, Taiwan, Republic of China

This report presents 2 cases of Ramsay Hunt Syndrome with different levels of severity treated effectively with integrated rehabilitative program. Case one was a 36-year-old female experiencing right earache, auricular eruption, and fuzzy oral pronunciation 5 days before admission. Although combination therapy of acyclovir and prednisone was given immediately after hospitalization, level of facial palsy only improved from grade III to grade II (House-Brackmann facial palsy grading system) after discharge. Case two was a 20-year-old male experiencing grade IV left facial palsy 14 days before admission. He received similar medication as that of case one during 10-day hospitalization, but severity of facial palsy persisted after discharge. Afterwards, both patients recovered without any sequelae after having undergone outpatient rehabilitation therapy including infrared radiation, silver spike point electrical stimulation, and facial neuromuscular exercise, 6 sessions in 3 weeks for case one and 6 sessions in 5 weeks for case two. Both patients were satisfied with the results.
Key words: electrical stimulation, facial neuromuscular exercise, herpes zoster oticus, Ramsay Hunt syndrome

INTRODUCTION
Ramsay Hunt syndrome (RHS), first described by Ramsay Hunt in 19071, is caused by reactivation of the varicella-zoster virus (VZV), and is characterized by classic triad of facial palsy, ear pain, and auricular eruption. Further, due to the close proximity of the geniculate ganglion to the vestibulocochlear nerve within the bony facial canal, the RHS patients usually present with tinnitus, hearing loss, vertigo, and hyperacusis as well2. Although secondary to Bells palsy in terms of the cause of acute atraumatic peripheral facial paralysis3,4, RHS, with incidence ranged from 0.3 to 18 percent, has a worse prognosis5,6. Devriese et al.5 described that 84% of RHS patients easily developed complications or sequelae, such as synkinesis, contracture, dry eye, and hearing loss, if without proper treatment.The most advisable method to treat RHS is the combination therapy with acyclovir and prednisone3,4, but still not promising, and several prerequisites are required

for a better result, e.g., early medication and less severity of disease. We present 2 RHS patients with different levels of facial palsy treated effectively with rehabilitation program, after the termination of the combination therapy of acyclovir and prednisone.

CASES REPORT
Case 1 A 36-year-old female English tutor with previous healthy condition presented to a local clinician because of right earache. Due to her poor response to medications and becoming worse with some fuzzy oral pronunciation 5 days later, she was transferred and admitted to our ENT (ear, nose, & throat) ward under the impressions of Ramsay Hunt syndrome (RHS) with grade III facial palsy of HouseBrackmann grading system7 (Table 1) and auricular perichondritis of the right ear. After her admission to the ENT ward, further physical examination revealed vesicular lesions scattered around the right congested auricle with complaints of earache associated with unclear (masking) hearing of right ear, vertigo, and tinnitus associated with weakness and tingling numbness of right facial muscles. Pure tone audiometry (PTA) showed mild sensoneural hearing loss over right ear with 15 db (decibel) difference between left and right ear. The serum level of anti-VZV IgG titer by immunofluroscence assay (IFA) was 1:320, which signified the current
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Received: April 24, 2003; Revised: June 3, 2003; Accepted: June 5, 2003. * Corresponding author: Kao-Chung Tsai, Department of Physical Medicine and Rehabilitation, Tri-Service General Hospital, 325, Cheng-Kung Road Section 2, Taipei 114, Taiwan, Republic of China. Tel: +886-2-87923311 ext 17068; Fax: +886-2-8792-7162; E-mail: kct1@ms16.hinet.net

Ramsay Hunt syndrome

Table 1 House-Brackmann facial nerve grading system


Grade Category I II Normal Characteristics Normal facial function in all areas

Mild dysfunction Gross: slight weakness noticeable on close inspection, may have very slight synkinesis; at rest: normal symmetry and tone; forehead motion: moderate to good function; eye motion: complete closure with minimum effort; mouth motion: slight asymmetry Gross: obvious but not disfiguring difference between two sides, noticeable but not severe synkinesis, contracture, and/or hemifacial spasm; at rest: normal symmetry and tone; forehead motion: slight to moderate movement; eye motion: complete closure with effort; mouth motion: slightly weak with maximum effort Gross: obvious weakness and/or disfiguring asymmetry; at rest: normal symmetry and tone; forehead motion: none; eye motion: incomplete closure; mouth motion: asymmetry with maximum effort Gross: only barely perceptible motion; at rest: asymmetry; forehead motion: none; eye motion: incomplete closure; mouth motion: slight movement No movement

III Moderate dysfunction

IV Moderate severe dysfunction

Severe dysfunction

VI Total paralysis

Fig.1 A lateral view of the head and face showing acupuncture points or motor points of corresponding facial muscles and acupuncture Meridians stimulated by silver spike point (SSP) electrical stimulation for patients with facial palsy.

infection of herpes zoster virus (VZV). The MRI of brain showed negative finding. To control both bacterial and VZV infections, intravenous (IV) treatments with prednisone 750mg q8h, gentamycin 80mg q12h, and cefazolin 1gm q8h and oral acyclovir 200mg q4h were used after admission. Meanwhile, because of the persistent grade III facial palsy, rehabilitation therapy was requested 4 days after admission and thus 2 sessions were undertaken during hospitalization. With improved condition, after 10-day hospitalization, she was discharged with the medication of prednisone 1.0 mg bid for 3 days, which was tapered to 0. 5 mg tid for the following 3 days. Besides, she attended 6 more sessions of rehabilitation program in 3 weeks after discharge before a complete recovery of RHS. Case 2 A 20-year-old male student was admitted to our ENT ward with complaints of left earache, numbness of full left face, and difficulties of smiling, closing his left eye, and raising his left eyebrow 14 days before admission. He was diagnosed to have RHS with grade IV left facial palsy. Pure tone audiometry revealed within normal limits. Immediately after admission, he received intravenous prednisone 4mg q8h for 8 days, which was tapered to 6mg qd for 2 days, in addition to IV acyclovir 750mg q8h for 1 week. Rehabilitation evaluation was inquired on the 9th day of hospitalization when grade IV left facial paralysis stayed and was associated with taste deficit, face numbness, vertigo, and mild tinnitus, indicating multiple cranial polyneuropathy.
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The patient was discharged after 10-day hospitalization. His earache alleviated, but grade IV facial palsy stayed. He attended outpatient rehabilitation therapy, in addition to having take-home medication of prednisone 1.5 mg bid for 3 days, which was tapered to 0.5 mg qid for the following 4 days. The rehabilitation program that both patients attended included silver spike point (SSP) electrical stimulation, facial neuromuscular exercise, and infrared radiation (IR). SSP electrical stimulation is functioned via a high voltage galvanic pulsed stimulator system (Super Laus MX4400, Nihon Medix Co., Japan). SSP, works as a surface electrode to focally transport electrical current, has both characteristics of high conductivity and acupuncturelike effect, placed upon the acupuncture point or motor point of individual muscle8 with a total of 6 stimulation points on 6 muscles, including frontalis, orbicularis oculi, zygomatic, masseter, orbicularis oris, and mentalis (Fig. 1). Each motor point has its corresponding acupuncture point of different meridians as frontalis to Gall bladder 14 (GB14), orbicularis oculi to Gall bladder 1 (GB1), zygomatic to Stomach 7 (St7), masseter to Stomach 6 (St6), orbicularis oris to Stomach 4 (St4), and mentalis to Conception vessel 24 (CV24). More detail about these six and other available acupuncture points related to the Western anatomical locations in the face can be obtained in the study by Dr. Dung9. The stimulation parameters are characterized as a bi-directional symmetric waveform with pulse duration of 50(s and triple mixed (TRM) alternating electrical stimulation at 3, 10, and 20 Hz every 4 seconds.

Cheng-Wen Ho, et al.

Intensity of stimulation is maintained at the level of patients maximal tolerance without concomitant muscle contraction. The therapy time of SSP electrical stimulation is 20 minutes every visit. The facial exercise program was adhered to the previous neuromuscular retraining program10 and composed of (1) relaxation of hyperactive muscles, (2) facial massage exercises, (3) biofeedback training using a mirror to let patient know facial movement, and (4) specific facial exercises like smiling, grimacing, and whistling. Both patients were instructed by the same physical therapist 2 times a week, at least 60 minutes per visit. In addition to the hospital practice, they were encouraged to do methodically the facial exercise themselves in front of a mirror at home. Furthermore, for relief of pain and control of facial muscle spasm, a luminous IR heat lamp was used with a distance ranged from 30 to 60 cm perpendicular to the patients lesion face. The therapeutic time of IR exposure was 20 to 30 minutes every visit. Through the course of illness, in addition to medications with acyclovir-prednisone during hospitalization and prednisone alone for 6 days after discharge, both patients were subsequently treated with rehabilitation program as mentioned above because of persistent RHS with facial palsy. Case 1 was recovered from stage III to stage II facial palsy after discharge, though the combination medication was started within 5 days of onset of facial paralysis. Case 2 still had stage IV facial palsy after completion of the medical therapy as same as that of case 1. For case 1, it took additional 3 weeks of outpatient rehabilitation program for total remission of her facial palsy to occur, in addition to the 1-week inpatient rehabilitation. Case 2 attended only 6 times of outpatient rehabilitation program because of his busy schedule. He also did the home facial exercise twice daily, 40 to 60 minutes per session. He had total facial function recovery 5 weeks after discharge, i.e., 2 more weeks longer than case one. Both patients were treated successfully with subsequent rehabilitation programs without any sequelae or facial asymmetry 2 months after discharge.

DISCUSSION
To our knowledge, this is the first formal report that presents a successful intervention of integrative rehabilitative program on RHS patients with different severities. Although therapy with acyclovir11, an antiviral agent that interferes with the replication of VZV, and prednisone was the most advisable modality for RHS patients2,4,12, Inamura et al.13 reported 4 out of 9 RHS patients were not satisfactorily treated with acyclovir. In addition, the present report

shows little beneficial effect of combination of acyclovir and prednisone, i.e., persistent grade II facial palsy for case 1 and grade IV facial palsy for case 2 after treatment. However, both patients recovered completely without any sequelae later after attending the rehabilitation programs, 3 weeks for case 1 and 5 weeks for case 2. Targan and his colleagues had demonstrated that patients with chronic facial palsy, periods ranged from 1-7 years, were benefited from electrical stimulation. Improvement of clinical residual symptoms and motor recovery were obtained after 3 months of treatment14. Despite its therapeutic effect in facial palsy patients, electrical stimulation had been criticized on account of potentially increased complications as synkinesis and contractures15. Without primary attention to the accuracy of the facial muscle stimulation and recruitment for the attempted voluntary facial movement, facial electrical stimulation would intensify the abnormal facial contraction pattern and complications would possibly ensue. To have effects of both acupuncture and electrical stimulation, SSP electrodes were focally placed on the six acupuncture points (Fig. 1) and connected to a high voltage galvanic pulsed stimulator system and operated cautiously every time by the same physical therapist. By stimulating similar meridian points as those of the presented 2 cases, Mayer had demonstrated an extensive improvement of facial function in 3 weeks on 2 facial palsy patients treated with acupunctures16. To lessen the possibility of abnormal facial contraction, intensity of electrical stimulation was maintained at submotor level so that the patients were able to perceive the pulses without concomitant muscle contraction. The adaptive changes in the central nervous system8 and peripheral reinnervation of facial and trigeminal nerves14,17 had been considered the possible mechanisms of therapeutic effect of electrical stimulation on facial palsy. However, the optimal parameters of electrical stimulation were unable to obtain by the present results of 2 cases or previous reports14 because of erratic conditions of patients and the variable manipulated settings of stimulation, including intensity, duration and frequency. In addition to the electrical stimulation, the facial neuromuscular exercise was suggested to obtain more promising outcome of treatment14. Previous studies had demonstrated the positive results of more functional and symmetric facial contractions in chronic facial paralysis patients after facial exercise program10,17. Shiau et al., however, warned that unsupervised home exercise programs could be detrimental because of patients incorrect practice17. To avoid such a faulty operation, both patients practiced the facial exercise by using a mirror for visual feedback under
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the guidance of the same physical therapist and then did the same exercise methodically at home. Thus, the advantageous effect of facial exercise on facial recovery would be anticipated10, especially on case two, even after only 6session supervised outpatient rehabilitation exercise. Certainly, the most recommended therapy for RHS was the combination of acyclovir and prednisone2,4,12. A recent study of 80 RHS patients with different levels of severity treated with acyclovir-prednisone showed that complete facial recovery, i.e., House grade I, was only seen in 52% (42 out of 80) of patients, no matter what their pretreatment gradings were4. In other words, there were still 48% of patients who retained complications even after combination therapy. For a good prognosis of facial recovery and hearing loss, early combination therapy within 3 days of the onset of facial palsy was critical; chances of grade I recovery was less than 30% if therapy started later than 7 days of onset. Case one received combination therapy within 5 days of onset and hence possibly benefited from medication with improvement of facial palsy from stage III to stage II after discharge. Case 2, however, appeared to get insignificant benefits from medications. He had the persistent grade IV facial palsy after discharge. Probably the late medications, more than 7 days after onset, could account for the ineffectiveness. The roles of combination therapy of acyclovir-prednisone were uncertain. Natural progression of RHS had once been uncovered5. Devriese and Moesker had demonstrated 1-year natural history of 102 facial palsy patients with auricular VZV infection5. In that study, voluntary motor function at the mouth and the eye was used to classify the severity of facial palsy, that was, level A= normal function, B= slight paresis, C= distinct paresis (a level equivalent to that of case 1), D= subtotal loss of function (a level equivalent to that of case 2), and E= total loss of function. Patients with different levels of paralysis, i.e., level B-E, were studied after 1-year without medication. The results showed that most patients had an improvement of 2 or more functional levels. Full facial motor function recovery without sequela, however, was only seen in 6 out of 58 patients (10%) with complete paralysis and in 19 out of 28 patients (66%) with incomplete paralysis. More specifically, the natural recovery rates were 12 of 13 level C patients and 7 of 13 level D patients. It was concluded that facial improvement was clearly related to the maximal degree of loss of function. In conclusion, without early treatment RHS patients are less likely to recover completely. It is not easy for common people or even clinicians to be aware of the early happening of RHS. Thus, it is common to miss the early treatment,
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as seen in the example of case two. Furthermore, the outcome of early medication is not usually promising, such as that of case one. Because of the psychological stress of fear and anguish over the potential appearance changes, RHS patients would long for an early recovery despite knowing the possible natural recovery afterwards. Therefore, the current rehabilitation program emerges as a beneficial, though not a definite solution, for RHS patients with grade III or IV facial palsy, which merits further prospective studies.

REFERENCES
1. Hunt JR. On herpetic inflammation of the geniculate ganglion. A new syndrome and its complications. J Nerv Ment Dis 1907;34:73-96. 2. Kuhweide R, Van de Steene V, Vlaminck S, Casselman JW. Ramsay Hunt syndrome: pathophysiology of cochleovestibular symptoms. J Laryngol Otol 2002;116: 844-848. 3. Grose C, Bonthius D, Afifi AK. Chickenpox and the geniculate ganglion facial nerve palsy, Ramsay Hunt syndrome and acyclovir treatment. Pediatr Infect Dis J 2002;21:615-617. 4. Murakami S, Hato N, Horiuchi J, Honda N, Gyo K, Yanagihara N. Treatment of Ramsay Hunt syndrome with acyclovir-prednisone: significance of early diagnosis and treatment. Ann Neurol 1997;41:353-357. 5. Devriese PP, Moesker WH. The natural history of facial paralysis in herpes zoster. Clin Otolaryngol 1998;13:289-298. 6. Robillard RB, Hilsinger RL, Adour KK. Ramsay Hunt facial paralysis: clinical analysis of 185 patients. Otolayngol Head Neck Surg 1986;95:292-297. 7. House JW, Brackmann DE. Facial nerve grading system. Otolaryngol Head Neck Surg 1985;93:146147. 8. Melzack R, Stillwell DM, Fox EJ. Trigger points and acupuncture points for pain: correlations and implications. Pain 1977;3:3-23. 9. Dung HC. Acupuncture points of the cranial nerves. Am J Chinese Med 1984;7:80-92. 10. Segal B, Hunter T, Danys I, Freedman C, Black M. Minimizing synkinesis during rehabilitation of the paralysed face: preliminary assessment of a new smallmovement therapy. J Otolaryngol 1995;24:149-153. 11. Feldman SR, Ford MJ, Briggaman RA. Herpes zoster and facila palsy. Cutis 1988;42:523-524. 12. Sweeney CJ, Gilden DH. Ramsay Hunt syndrome. J Neurol Neurosurg Psychiatry 2001;71:149-154.

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13. Inamura H, Aoyagi M, Tojima H, Koike Y. Effects of acyclovir in Ramsay Hunt syndrome. Acta Otolaryngol (suppl) 1988;446:111-113. 14. Targan RS, Alon G, Kay SL. Effect of long-term electrical stimulation on motor recovery and improvement of clinical residuals in patients with unresolved facial nerve palsy. Otolaryngol Head Neck Surg 2000; 122:246-252.

15. Fitzgerald DC. Role of electrical stimulation therapy for Bells palsy. Am J Otolaryngol 1993;14:413-414. 16. Mayer FJ. Acupuncture in two cases of peripheral nerve paralysis. Am J Chinese Med 1977;5:95-100. 17. Shiau J, Segal B, Danys I, Freedman R, Scott S. Longterm effects of neuromuscular rehabilitation of chronic facial paralysis. J Otolaryngol 1995;24:217-220.

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