Ramsay Hunt Syndrome (Herpes Zoster Oticus) in A 12 Year Old Child and Review of Literature - A Case Report  

Menon Narayanankutty Sunilkumar , Narendran Gayathrivarma , Vadakut Krishnan Parvathy
Amala Institute of Medical Sciences, Amala Nagar, Kerala, India
Author    Correspondence author
International Journal of Clinical Case Reports, 2015, Vol. 5, No. 12   doi: 10.5376/ijccr.2015.05.0012
Received: 04 Dec., 2014    Accepted: 04 Jan., 2015    Published: 28 Feb., 2015
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This is an open access article published under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
Preferred citation for this article:
Sunilkumar et al., 2015, An Unsuspected Case of a Cervical Degenerative Leiomyoma, from Mumbai, India, International Journal of Clinical Case Reports, Vol.5, No. 12 1-3 (doi: 10.5376/ijccr.2015.05.0012)

Abstract

The enigma of Ramsay Hunt syndrome is that it is an association of an acute peripheral facial neuropathy and herpetic vesicular rash of the skin of the ear canal, auricle (Herpes Zoster Oticus) and/or mucous membrane of the oropharynx. Paediatricians see many children with vesiculous eruptions over the face and the body.  A combination of otalgia and  cutaneous / mucosal eruptions is not very uncommon in these children. Interestingly many treating doctors are not aware of this syndrome and can be missed easily or diagnosed late. Facial palsy can result if a proper history of a varicella zoster infection is not identified, diagnosed with delay and if appropriate measures not taken in such a child. In this case study, we report a12 year-old boy who presented with Herpes Zoster Oticus (HZO) and timely treatment instituted prevented the Ramsay Hunt syndrome (RHS).

Keywords
Ramsay Hunt Syndrome; Herpes Zoster Oticus; Varicella-Zoster Virus; Facial Nerve Palsy; Acyclovir

Introduction
James Ramsay Hunt, an eminent American neurologist in 1907, first described a syndrome of otalgia associated with coetaneous and mucosal rashes in his patient (Louis and Williams, 2003). He postulated that it is a rare complication of latent infection of the geniculate ganglion by human herpesvirus 3 ie Varicella-Zoster Virus (VZV) (Hunt, 1907). This infection gives rise to vesiculation and ulceration of the external ear and ipsilateral anterior two thirds of the tongue and soft palate, as well as ipsilateral facial neuropathy (in 7th cranial nerve). RHS is HZO, or auricular herpes zoster with acute facial palsy and is also known as geniculate neuralgia or nervus intermedius neuralgia. It is a self-limiting disease and the morbidity mainly results from facial weakness (Hunt, 1907; Louis and Williams, 2003). RHS has been reported in a case of varicella infection in utero and presentation in infancy at 3 months age and also in an 82 year old woman (Aframian, 1999; Balatsouras et al., 2007). The incidence of RHS in paediatric age group below 10 years of age is about 3/100.000 and so should be considered as a differential diagnose in any child with atraumatic facial palsy (Bleicher et al., 1996). The importance of being aware of the syndrome is very crucial, both in order to provide the adequate nursing care and to start treatment with antiviral agents immediately to prevent RHS with facial nerve palsy. In this case study, we present 12 year-old boy who presented with HZO.

Case Report
A 12 year old boy was admitted with pain in the right side of the face, toothache, right ear ache of 5 days duration. Vesicles on the right side of face appeared on the previous day evening. He had tingling sensation in the right side of face with pain along with giddiness, vomiting and severe headaches. He was the second sibling of a non-consanguineous couple, had normal milestones of development and was immunized to date. But he has not received vaccination against varicella. Previously, he had history of chicken pox at the age of 7 years. On general physical examination, he was febrile, 1000F, Respiratory rate 28/minute, Heart rate 86/minute, Blood pressure 110/78 mm Hg and very lethargic. He had cervical lymphadenopathy and did not have any pallor, clubbing and jaundice. The skin lesions were tender, grouped vesicular lesions over the right side of the face and lips, dermatomal in distribution, with similar grouped vesicles noticed over the right pinna (Figure1). Some of the lesions gradually were seen to be secondarily infected and features of mild cellulitis started. His oral cavity showed gingivitis, large ulcers over the lateral aspect of the tongue. Central nervous system examination revealed only pain in the area of the vesicles on the right side of the face, there was excessive tears from the right eye, pain and tingling sensation in the right side of the face along the vesicular lesions, mild difficulty in clenching the teeth and fortunately no other severe features of facial nerve palsy such as facial asymmetry and the House-Brackmann facial nerve grading system was found to be grade 1 (ie normal facial function in all areas). There was no dysarthria, gait ataxia or hearing loss. He was conscious and oriented signs of meningeal irritation were absent. Systemic examination was normal.


Figure 1 1-Child on admission with the characteristic herpetic blisters in the right side of the face-Herpetic Zoster Oticus 

Dermatologist evaluation was done for the child. Tzanck smear showed giant cells. Ear Nose and Throat specialist’s evaluation revealed tympanic membranes intact in both ears and the right ear lobe was erythematic appearance, vesicles extending to external auditory canal and positional vertigo ruled out. Ophthalmology evaluation showed congested right eye and there were no vesicles in the eye and cornea appeared clear. Diagnosis of HZO and prevention of the RHS was made based on the history of varicella infection in the past and the clinical presentation now. Without undue delay on the same day itself appropriate treatment was started. Laboratory investigations such as Hemogram, urine routine, serum electrolytes, Serum Glutamic Pyruvate Transaminase (SGPT), peripheral blood smear, blood and urine culture sensitivity results were normal (Table 1).


Table 1 Laboratory investigations 

Patient was given symptomatic treatment for his fever and pain with paracetamol and treated with Acyclovir (400 mg tablet, 1 tablets 6th hourly), co-amoxyclav intravenously (30 mg /kg/dose) 3 times/day along with metrogyl intravenously (7.5 mg/kg/dose) in 3 times/day for treating secondary infection of the vesicles and the cellulitis, silver sulfadiazine ointment local application over the lesions, proper hydration as he was very much lethargic for the first 3 days. Routine eye care was given like artificial tear drops and antibiotic eye drops. His skin lesions resolved in one week. Patient was discharged after full treatment of 7 days with acyclovir. He was followed-up and is doing well for the last 4 months.

Discussion
The reactivation of VZV (chickenpox) in the geniculate ganglion of the 7th cranial nerve resulting in ipsilateral facial paralysis/paresis along with the vesicular herpetic rash (HZO) and signs such as lethargy, otalgia, tinnitus, hearing loss, nausea, vomiting, vertigo and nystagmus is known as the classic RHS or type 2 RHS (Hunt, 1907; Louis and Williams, 2003). James Ramsay Hunt is also credited with identifying two other neurological syndromes such as RH cerebellar syndrome or the Type 1RHS having an association of seizures, cognitive impairment, myoclonus and progressive ataxia and the RHS Type 3, a neuropathy of the deep palmar branch of the ulnar nerve (Hunt, 1907).

VZV infection initially causes the acute chickenpox which generally occurs in children and young adults as seen in our case 5 years ago and resolves fast. The VZV then remains latent in the nerve cell bodies, the cells of the dorsal root, cranial nerve or autonomic ganglia without causing any symptoms and later on causes herpes zoster or shingles (Hunt, 1907; Bleicher et al., 1996). To cause RHS, VZV breaks out of nerve cell bodies and travel down nerve axons from one or more ganglia along nerves of an affected segment and infect the correspond-ing dermatome causing a painful rash along with the facial palsy. It can affect all ages, including children especially in the age group 6-15 years (Bleicher et al., 1996; Furuta et al., 2005). RHS is distinctive from herpes zoster in that there is a motor component. In RHS, first the visceral efferent motor fibres from the motor nucleus of the 7th cranial nerve that leave the brain stem are affected as they pass through the geniculate ganglion, impairing motor supply of the facial nerve. Also visceral afferent taste fibres from the anterior two thirds of the tongue going to the nucleus of the solitary tract via the nervus intermedius can be affected by local inflammation as they pass the geniculate ganglion. Thirdly decreased lacrimation may result from involvement of these visceral efferent parasympathetic fibres to the lacrimal and salivary glands from the superior salivatory nucleus, again via nervus intermedius as they branch at the level of the geniculate ganglion. Fourth important factor is that the cell bodies of the neurons from Spinal nucleus of 5th cranial nerve receiving general somatic afferent fibres from the geniculate zone of the ear via the chorda tympani lie in the geniculate ganglia and are the site of viral reactivation in classic RHS, causing vesicular eruptions in geniculate zones (Cavoy, 2013).

The diagnosis of Ramsay Hunt syndrome is usually made without difficulty when the clinical characteristics are present (Hato et al., 2000). The clinical presenting feature is often pain deep within the ear which is paroxysmal at first but, after a day or two, the pain often radiates outward into the pinna and then becomes constant. The patient also complains of rash or blisters in the distribution of the nervus intermedius which may be on anterior two thirds of the tongue, soft palate, external auditory canal, pinna (HZO) associated with secondary infection and cellulitis as in our case reported. A lower motor neuron pattern of ipsilateral face drop or weakness may be obvious. There may be hyperacusis on that side due to paralysis of the stapedius and tensor tympani .The other presenting features include vertigo and ipsilateral hearing loss, tinnitus facial RHS causes 2-10% of acute peripheral facial paralysis (PFP) cases (Hato et al., 2000; Yawn et al., 2007). The diagnosis RHS can be missed if there is no rash. This condition with only acute PFP is termed “zoster sine herpete” and tends to be misdiagnosed as Bell's palsy (Mori et al., 2002). The House-Brackmann scale is most commonly used to quantify the degree of facial muscle weakness (Gilchrist, 2009). The child in the case study was grade 1 (ie normal facial function in all areas) according to this scale.

Differential diagnosis of RHS if present includes Bell's palsy as it is the most common PFP in children (24-70%) (Kansu and Yilmaz, 2012). But the rash is the characteristic diagnostic feature to differentiate with RHS. Other conditions are viral labyrinthitis, possibly a stroke of the posterior inferior cerebellar artery region, -trigeminal neuralgia, postherpetic neuralgia, persistent idiopathic facial pain and temporomandibular disorders, referred pain (dental abscess) and carcinoma of the nasopharynx. simple otitis (external, media) (Aframian, 1999; Kim and Bhimani, 2008).

Regarding diagnosis virological studies are available but usually the diagnosis of RHS/HZO in children is clinical and a good medical history. VZV isolation in conventional cell culture is considered the definite diagnostic test .It has a specificity of 100% but is not always feasible. Tzanck test was done in our case and has significance to identify the etiology (Durdu et al., 2008). VZV antigen detection by direct immunofluorescence assay has a sensitivity of 90% and specificity 99% (Coffin and Hodinka, 1995). In our case a tzanck smear and timely clinical diagnosis was made and treatment started.

Antiviral agents are effective in reducing the severity and duration of acute herpes zoster and HZO perse when given within 72 hours of rash onset. Moderate pain relief can be achieved with anticonvulsants, tricyclic antidepressants, opioids, and topical treatment modalities such as lidocaine-containing patches and capsaicin cream (Galluzzi, 2007).

Corticosteroids and oral acyclovir are commonly used in the treatment of RHS. Steroids reduce the inflammation of the cranial nerves and help alleviate the pain and neurologic symptoms Retrospective studies have shown earlier administration of steroids along with antivirals within 3 days of symptom onset have 75% rate of full recovery whereas only 30% if combined therapy is started 7 days after onset of symptoms. The typical combined therapy involves a 7- to 10-day course of famciclovir (500 mg, 3 times daily) or acyclovir (60-80 mg/Kg/day, 8th to 6th hourly), along with oral prednisone (60 mg daily for 3 to 5 days) (Murakami et al., 1997). Surgical decompression of the facial nerve has no role in this syndrome (Muecke and Amedee, 1993).

In our case, there was mild difficulty in eating but no evident facial droop .He was started on only acyclovir on day 1 itself for 7 days and there was no facial palsy. Antibiotics were given to treat the secondary infection. He was very closely monitored with all supportive care, and eye/corneal care. He did not develop facial palsy and steroids were not prescribed. The zoster (shingles) vaccine is considered the most effective way to reduce incidence of herpes zoster/HZO and postherpetic neuralgia, and to reduce severity of any outbreak (Muecke and Amedee, 1993; Galluzzi, 2007).

The prognosis for HZO/RHS in children is better than that in adults (Hato et al., 2000). Advanced facial paralysis, audiovestibular findings and starting treatment late result in a bad prognosis (Kansu and Yilmaz, 2012). Hearing loss usually recovers well. Age of the patient and other co-existing illness has poor prognostic features (Yeo et al., 2007).

The prognosis of the facial paralysis in HZO/RHS is worse than that in Bell's palsy, and only 10% of complete facial paralysis in RHS recovers completely (Muecke and Amedee, 1993; Yeo et al., 2006).

HZO caused by VZV reactivation can result in the rare RHS in children. So a child with HZO is vulnerable to progress to RHS, if not diagnosed immediately and timely treatment started. Physician education is vital in detecting HZO/RHS at very early stage as it can be a rare cause of facial palsy in young children and thereby prevent the associated morbidity.

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