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| CASE REPORT |
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| Year : 2016 | Volume
: 22
| Issue : 3 | Page : 217-219 |
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Pediatric Ramsay Hunt syndrome: A rare clinical entity
Natashya Hilda Sima, Poonam K Saidha, V Sreenivas
Department of Otorhinolaryngology - Head and Neck Surgery, St. John's Medical College, Bengaluru, Karnataka, India
| Date of Web Publication | 8-Aug-2016 |
Correspondence Address:
Dr. Natashya Hilda Sima
Department of Otorhinolaryngology - Head and Neck Surgery, St. John's Medical College, Koramangala, Bengaluru - 560 034, Karnataka
India
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/0971-7749.187977
Ramsay Hunt syndrome is a rare cause of facial nerve paralysis in children, caused due to reactivation of latent Varicella–Zoster virus within the geniculate ganglion. In addition to the facial nerve, Ramsay Hunt syndrome may also affect the vestibulocochlear nerve leading to inner ear dysfunction and in severe case may also involve other cranial nerves. We report a case of Ramsay Hunt syndrome in a 15-year-old child. Keywords: Facial nerve palsy, Pediatric facial nerve palsy, Ramsay Hunt syndrome
How to cite this article:
Sima NH, Saidha PK, Sreenivas V. Pediatric Ramsay Hunt syndrome: A rare clinical entity. Indian J Otol 2016;22:217-9 |
| Introduction | |  |
Ramsay Hunt syndrome, first discovered by James Ramsay Hunt, is a syndrome affecting the facial nerve caused due to the reactivation of dormant Varicella–Zoster virus within the geniculate ganglion.[1] It is a disorder which more commonly affects the older age group with children comprising <10% of the cases.[1],[2] As reports on children are limited in literature, the outcome of the disease in children is less understood.
We report a case of a 15-year-old child presenting with peripheral facial nerve palsy diagnosed as Ramsay Hunt syndrome.
| Case Report | | |
A 15-year-old girl presented with history of severe headache and retro-orbital pain causing sleep disturbance of 1 month duration. It was followed by double vision and nonprojectile vomiting and mild deviation of right side of the mouth. There was no history of ear pain or discharge from the ear, taste disturbance, or tingling sensation over the face.
On examination, she had vesicular eruptions over the left nasolabial area [Figure 1] with obvious deviation of the angle of mouth to the right side, decreased closure on the left eye on maximal effort, and absence of creases over the forehead on frowning. She also had left-sided lateral rectus palsy. Ear examination was within normal limits.
Blood investigations included a normal hemoglobin total count and differential count. Pure tone audiogram confirmed a mild sensorineural hearing loss on the left side. Cerebrospinal fluid analysis was within normal limits and was negative for acid-fast bacillus. Fundoscopic examination showed no feature of intracranial hypertension. Magnetic resonance imaging brain revealed mild inflammation in the left petrous apex [Figure 2]. | Figure 2: Magnetic resonance imaging brain showing mild inflammation of left petrous apex
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The child was treated conservatively with intravenous acyclovir five times a day for a duration of 7 days, injection dexamethasone for 10 days, and oral amitriptyline. She also received facial physiotherapy and eye care with taping of the eye at night and sterile eye drops. The child was followed up for a period of 6 months and clinically showed a complete recovery of facial nerve function.
| Discussion | | |
Ramsay Hunt syndrome, also known as geniculate neuralgia or nervous intermediate neuralgia,[3] is the second most common cause of atraumatic facial nerve palsy.[4] It is a disorder affecting the facial nerve and is caused by reactivation of latent Varicella–Zoster virus in the geniculate ganglion, which causes neural inflammation, pressure, and destruction of the facial nerve in the temporal bone.[2]
Ramsay Hunt syndrome usually presents with deep seated ear pain along with facial paralysis, herpetiform lesions,[1],[3] and occasionally inner ear dysfunction with symptoms such as tinnitus, nausea, vomiting, and nystagmus and is caused due to the close proximity of the geniculate ganglion to the vestibulocochlear nucleus within the bony facial canal. In severe cases, there may be involvement of cranial nerves V, IX, X, XI, and XII.[4] The mechanism of involvement of various cranial nerves was described by Hunt where he hypothesized that the gasserian, geniculate, petrous, accessory, plexiform, and second and third dorsal ganglia comprised a chain, the result of which inflammation of one ganglion could extend to and thereby affect other nearby ganglia.[5] Other theories hypothesized the spread of infection through the vasa vasorum, as the blood vessels are vulnerable to herpes zoster virus which thereby produces infarction thus resulting in polyneuritis.[6]
The incidence of Ramsay Hunt syndrome is found to increase with age, and it is also found that the severity of infection increases in older individuals. It is thought to be a result of decline of cellular immunity in older individuals.[2] Children account for <10% of the diagnosed cases with an incidence of 2.7/100,000 in children below the age of 12 years.[1] It has also been found that the appearance of vesicles is delayed in children as compared to adults;[4] however, complete recovery is more common in children as compared to adults.[7]
Diagnosis of Ramsay Hunt syndrome is confirmed by Elisa serum antiherpes zoster virus IgG and IgM antibody titers.[1] PCR helps to distinguish Ramsay Hunt syndrome from Bell's palsy and can be detected in saliva, tears, middle ear fluid, and blood mononuclear cells.[2]
Treatment of Ramsay Hunt syndrome includes antiviral agents such as acyclovir or newer agents such as famciclovir and valacyclovir which prevent the replication of Varicella–Zoster virus. Adjuvant steroid therapy has also been found to be useful as it prevents inflammation and edema.[1],[2]
Recovery of facial nerve function depends on early initiation of treatment, with best results seen if treatment is initiated within 3 days of onset of the disease. Full recovery has been found in up to 75% of the cases when treatment as initiated within 3 days of onset of the disease as compared to complete recovery in 30% of cases when treatment was initiated after 7 days of onset.[8]
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Nil.
Conflicts of interest
There are no conflicts of interest.
| References | | |
| 1. | Derin S, Derin H, Sahan M, Caksen H. A pediatric case of Ramsay Hunt syndrome. Case Rep Otolaryngol 2014;2014:469565.  |
| 2. | Gondivkar S, Parikh V, Parikh R. Herpes zoster oticus: A rare clinical entity. Contemp Clin Dent 2010;1:127-9. [ PUBMED]  |
| 3. | Kathiga Kannan S, Eugenia Sherubin J, Sajesh S, Gopakumar KP. Ramsay Hunt syndrome (Herper Zoster Oticus). J Indian Acad Oral Med Radiol 2012;24:70-2. |
| 4. | Sweeney CJ, Gilden DH. Ramsay Hunt syndrome. J Neurol Neurosurg Psychiatry 2001;71:149-54. |
| 5. | Hunt JR. The symptom-complex of the acute posterior poliomyletis of the geniculate, auditory, glossopharyngeal and pneumogastric ganglia. Arch Intern Med 1910;5:631-75. |
| 6. | Lapresle J, Lasjaunias P. Cranial nerve ischaemic arterial syndromes. A review. Brain 1986;109(Pt 1):207-16. [ PUBMED] |
| 7. | Devriese PP, Moesker WH. The natural history of facial paralysis in herpes zoster. Clin Otolaryngol Allied Sci 1988;13:289-98. |
| 8. | 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;41:353-7. |
[Figure 1], [Figure 2]
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