Portuguese Version

Year:  2000  Vol. 66   Ed. 5 - (17º)

Relato de Casos

Pages: 536 to 538

Ramsay-Hunt Syndrome: Case Report.

Author(s): Lauro J. L. Alcântara*,
Fabiano Gavaszoni**,
André L. Ataíde**,
Emerson A P. de Carvalho***.

Keywords: herpes zoster oticus, facial paralysis, acyclovir

Abstract:
The Ramsay Hunt syndrome envolves a vesicular eruption on the external, middle and inner ear reported to the reativation of latent varcela-zostervirus (VZV) retained in a dormant state within sensory ganglia of the facial nerve, causing facial paralysis. This syndrome commonly affects elderly, diabetics and immunedeprimed patients. In this article, an younger patient with Ramsay Hunt syndrome is described. This patient was treated with prednisona and acyclovir having a good evolution.

INTRODUCTION

Herpes zoster oticus (HZO) is a viral infection involving external, middle and inner ears resulting from aggravation of varicella - zoster virus, present in a latent state within the sensorial ganglion of facial nerve. Classical clinical presentation includes pain on pinna, followed by vesicular eruption on external canal and pinna. Facial paralysis, if present, characterizes Ramsay-Hunt syndrome, and is simultaneous to the surge of cutaneous blisters. This kind of facial paralysis amounts to 3% to 12% of all types of facial paralysis11,4,15. A variable degree of involvement of the 8th cranial nerve, manifested by auditory and vestibular symptoms (hearing loss and vertigo) are present in approximately 20% of the cases. Involvement of 5th, 9th, 10th, 11th and 12th cranial nerves is less frequent. Other viral infections may also cause similar clinical presentation, such as herpes simplex and cytomegalovirus.

REVIEW

Facial paralysis in Ramsay Hunt syndrome has worse prognosis than Bell's palsy12. Only 10% of patients who have total paralysis will recover the complete function of facial nerve and only 66% of the patients with incomplete paralysis will recover facial function fully.

Evidence of viral etiology has been well documented. Inamura et al. demonstrated high titers against VZV using the technique of complement fixation17 in 21% of patients with HZO and in 100% of patients assessed by Elisa method. Using the technique of temporal bone DNA sequencing by polymerase chain reaction (PCR), Wackym et al. demonstrated the presence of genomic DNA of VZV in geniculate ganglion of 2 patients who had history of Ramsay Hunt syndrome. Enlargement of DNA of geniculate ganglion of controls and specimens with Bell's palsy did not reveal fragments of VZV.

Treatment of HZO had major breakthroughs5,6 as a result of development and application of new virostatic agents, especially acyclovir. Acyclovir is effective in the prevention of replication of a variation of viral particles, including VZV, Epstein Baarvirus and CMV. The drug is phosphorilated in the host cells infected by the viral enzyme thymidinekinase. The resulting acyclovir triphosphate is then incorporated to the neoformed viral DNA, leading to the end of DNA molecular chain. Individual susceptibility to each viral type is not uniform. Since VZV thymidinekinase is less sensitive to acyclovir than herpes simplex, acyclovir is used at higher doses than the usual ones for HZO.

The dose of acyclovir for parenteral administration is 15 mg/Kg/day. Owing to low absorption of acyclovir by gastrointestinal tract5 -15% to 25%, higher doses are suggested for oral administration1.

Dickens et al. reported the results of a study without controls involving 8 patients with HZO treated with intravenous acyclovir. All patients started treatment between 1 and 15 days after the onset of facial paralysis, consistent with the concept that HZO is a central nervous system disseminated viral infection. Four patients had involvement of 9th cranial nerve, one had paresis of 10th nerve and one had involvement of brainstem. Before treatment, 6 patients had complete facial paralysis, and two had paresis. At discharge, after 7 days of 10mg/Kg of acyclovir TID, four out of six patients with facial paralysis had recovery of movement, one out of two patients with facial paresis improved facial symptoms and the other patient progressed to complete facial paralysis. Another treatment series conducted by Stafford and Welch13 obtained similar results. The series reported by Uri et al.16 also reported success in the treatment of HZO with acyclovir.

In managing HZO, Adour et al. recommended the use of oral acyclovir, 800mg 5 times a day for 10 days, combined with prednisone administered in decreasing doses for 14 days. Patients should also be well-hydrated and free of renal impairment in order to use acyclovir. The most common side effect is gastrointestinal complication.

CASE REPORT

E. L. T., 17 year-old male, came to the Emergency Room of Hospital de Clínicas, in Curitiba, with history of pain on the right pinna, edema and local fever for three days. There were vesicles on the right pinna and right auditory canal, and with progression, it was also noticed deviation of buccal rime to the left and loss of control of facial muscles on the right. In the exam, he had good general health, no fever, deviation of buccal rime and peripheral facial paresis to the right. Right pinna and right external auditory canal had hyperemia and multiple vesicular lesions, some of them already ulcerated. Tympanic membrane was intact.

The hypothesis of Ramsay Hunt syndrome was made and the patient was referred to the Ambulatory of Otorhinolaryngology of the Hospital for further assessment. The hypothesis was clinically confirmed and treatment with valacyclovir, analgesics and physical therapy was initiated. The patient came back after 6 days with no clinical improvement, motivating a hospital admission. Electroneurography was ordered and the patient was treated with EV 5mg/Kg acyclovir TTD and prednisone 20mg per day. The result of ENG revealed axonal lesion of right facial nerve, with signs of active denervation. The lesion pattern was axonotmesis. The patient was discharged after 5 days with complete clinical improvement. He used PO valacyclovir and prednisone in decreasing doses for one more week.

On the 14th day after the onset of symptoms, blood samples were collected for serology of VZV antibodies and herpes simplex. The results were positive for recent infection of VZV.

After two weeks of evolution, the patient had restored completely his motor facial function.

DISCUSSION

The clinical presentation of otalgia, vesicle eruptions on pinna and external auditory canal, followed by ipsilateral facial paresis/paralysis, is the classical presentation of Ramsay Hunt syndrome. In addition to the clinical presentation5, we may conduct Tzanck smears, obtained from the active lesion, despite the fact that it does not distinguish between herpes zoster and herpes simplex. Direct serology to detect the virus has the advantage of producing quick results after the collection of skin sample. Although viral culture is the gold standard for diagnosis, it normally takes 3 to 7 days to be ready. One alternative is to perform diagnosis using antigens for Elisa test and reaction of complement fixation (CF) and detection of nucleic acid of virus through PCR. Tomita et al. conducted a study with 287 cases of Bell's palsy and 64 cases of Hunt syndrome clinically diagnosed and submitted to exam by complement fixation and Elisa method. In 50 cases clinically diagnosed as Ramsay Hunt syndrome, 74% were positive to CF in the study of VZV antigen and 94% were positive to VZV in Elisa test. A study was conducted to confirm positivity of anti-VZV antibodies and date of material collection - 51.6% of positivity to anti-VZV was found in the first week; 88.9%, in the second week, and 100% in the third week, through Elisa method. In the case reported here, the test conducted on the 14th day after onset of symptoms was positive with Elisa method.

According to Stafford and Welchr13 and Uri, Greenberg and Meyer16, acyclovir has proved to reduce significantly the duration of the disease, pain and ocular complications. Early treatment with acyclovir, famcyclovir and valacyclovir showed to be effective in herpes zoster infections and in preventing complications. The early start of therapy with anti-viral agents is likely to have contributed to the good progression of this case, in addition to the incomplete facial paralysis and the age of the patient.

FINAL COMMENTS

Ramsay Hunt syndrome has variable clinical presentation regarding number and extension of impairment of cranial nerves. Clinical hypothesis, early treatment and lab confirmation of infection enable a favorable progression of the case, preventing disabling nerve motor sequelae.

REFERENCES

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* Ph.D., Professor of the Department of ORL-UFPR;
** Resident Physician of the Department of ORL-UFPR;
*** Undergraduate, School of Medicine UFPR.

Affiliation: Department of Otorhinolaryngology at UFPR.
Address for correspondence: Dr. Fabiano Gavazzoni - Rua Herculano C. F. de Souza, 628 - Apto. 502 - 80240-290 - Água Verde - Curitiba/ PR.
E-mail: fgavazzoni@uol.com.br
Article submitted on September 16, 1999. Article accepted on December 16, 1999.

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