Address for correspondence : Hyun Seung Choi, MD, Department of Otolaryngology-Head and Neck Surgery, National Health Insurance Service Ilsan Hospital, 100 Ilsan-ro, Ilsandong-gu, Goyang 410-719, Korea
Tel : +82-31-900-0972, Fax : +82-31-900-0972, E-mail : choihyunseung@gmail.com

Introduction

Primary infection to varicella zoster virus (VZV), a DNA virus of the Herpes viridae family, produces the typical picture of chickenpox. After primo-infection occurs generally during childhood, the virus remains quiescent. The most frequent sites of latency of VZV are dorsal root ganglia of the trigeminal nerve, but any autonomic ganglion or cranial nerve can be involved. Reactivation of this virus during adulthood can produce zoster (shingles), which presents with painful vesicles following a skin segmental distribution.1) Involvement of the ophthalmic division of the trigeminal nerve (herpes zoster ophthalmicus) and involvement of the facial nerve (herpes zoster oticus) are the two most widely known clinical presentations regarding cranial nerves. Involvement of other cranial nerves by VZV is more rarely reported.
We present one case of VZV infection of the external ear (cranial nerve V), pharynx and larynx with neuropathy of four ipsilateral lower cranial nerves (IX, X, XII).

Case

In April 2013, a 53-year-old man presented with the chief complaints of right otalgia for 9 days and hoarseness for 5 days. He also had right shoulder pain and dysphagia but he did not complained of dizziness, facial palsy and hearing loss.
On physical examination, crust and vesicle were observed in right auricle. We could see the drooping of right soft palate and the tongue was deviated to the right side when the patient stuck out his tongue. Endoscopic examination showed multiple ulcerations on the right side of the larynx from epiglottis to arytenoid (Fig. 1). And, right vocal cord was fixated to the paramedian position regardless of breathing and phonation.
There were no abnormal findings on neck computed tomography and brain magnetic resonance imaging but serum immunoglobulin M (IgM) for varicella zoster virus was measured highly.
We gave antiviral agent (Zovirax^® 5 mg/kg, 3 times a day, 5 days, Dong-A Pharmaceutical, Seoul, Korea) and oral steroid (prednisolone 60 mg, 5 days and tapered, Yu-Han Pharmaceutical, Seoul, Korea) to the patient under impression of lower cranial nerve involvement by varicella zoster. After 4 days of hospitalization, the mucosal lesions on the skin were healed but the paretic symptoms took several weeks to disappear. The patient was discharged on the ninth day. When the patient returned 2 weeks from the initial visit, ulceration of the larynx and auricular vesicles were gone and uvula deviation and right vocal cord paralysis were minimal. At 6 weeks, the patient still complained about pain in the right shoulder and ear. Complete recovery of right shoulder function took another one month.

Discussion

The most common presentation of herpes zoster in the head and neck region is called Ramsay Hunt syndrome (RHS), which rarely accompanies multiple cranial neuropathy. Herpes zoster also involves the mucous membrane of the tongue, palate, pharynx, and larynx. Herpes zoster infection of the larynx accompanied by RHS with cranial polyneuropathy is extremely rare, with only few reported cases in the literature.2,3,4,5) In our case, the patient presented with multiple cranial nerve involvements without facial palsy, which may not be suitable for diagnosing RHS, and making this case of VZV infection of the pharynx and larynx more unique.
The mucosal lesion which was confined to one half of the larynx was the key to the diagnosis. Besides mucosal abnormalities, oropharyngeal discomfort and complaints such as dysphagia and hoarseness were more troublesome symptom due to paralysis of the right pharyngeal muscles. These are innervated by the pharyngeal branches of the glossopharyngeal (IX) and the vagus (X) nerve. The vocal cord palsy was indicative of involvement of the right recurrent nerve which is also a branch of the vagus nerve. Laryngeal zoster with palsy of the IXth, Xth, and XIIth cranial nerve is a very rare entity. It has been described by Van Den Bossche, et al.4) in a 25-year-old patient in whom the paralysis was completed recovered. In this case, the author confirmed the clinical diagnosis of zoster by polymerase chain reaction (PCR)-based test of erosional exudate. It was obtained transnasally using a flexible endoscope after light sedation. In our case, PCR was not tested but serologic profiles of VZV IgM antibodies were obtained until week 6. In VZV infection, IgM antibodies against VZV are found 8-10 days after the appearance of visible mucosal or dermatological lesions, while IgG antibodies appear 4-6 days after the rash, reaching the highest level 3 weeks later.5) Only 50% to 84% of cases showed the VZV-IgM response in patients with herpes zoster.6) In our case, the serologic test taken at the initial visit presented positive IgM. IgM was still positive until week 4, although the concentration was decreased compared to the initial visit. At week 6, IgM was no more positive, but the patient still complained about pain in the right shoulder and ear. The full serological profiles of IgM against VZV until week 6 are provided in Fig. 2. Serological determination of IgM is reliable but not very useful for immediate decision-making knowing that IgM antibodies are found 8-10 days after the eruption.5) But in our case, the patient's initial complaint was limited right otalgia for 9 days. So the serologic test alone, which showed positive IgM, was reliable for diagnosis of VZV infection in our case.
In many cases of patients with VZV infection, it is introduced that the detection of VZV DNA by PCR is more sensitive and rapid compared to conventional serologic tests.7) From our case, it shows that with serologic test alone was sufficient for diagnosing VZV infection. Furthermore, VZV infection should be considered whether skin eruptions are present or absent. So if skin eruptions are absent, sample collections for PCR can be limited but it is not for serologic tests.

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2. Chitose SI, Umeno H, Hamakawa S, Nakashima T, Shoji H. Unilateral associated laryngeal paralysis due to varicella-zoster virus: virus antibody testing and videofluoroscopic findings. J Laryngol Otol 2008;122(2):170-6.

3. Nishizaki K, Onoda K, Akagi H, Yuen K, Ogawa T, Masuda Y. Laryngeal zoster with unilateral laryngeal paralysis. ORL J Otorhinolaryngol Relat Spec 1997;59(4):235-7.

4. Van Den Bossche P, Van Den Bossche K, Vanpoucke H. Laryngeal zoster with multiple cranial nerve palsies. Eur Arch Otorhinolaryngol 2008;265(3):365-7.

5. Watelet JB, Evrard AS, Lawson G, Bonte K, Remacle M, Van Cauwenberge P, et al. Herpes zoster laryngitis: case report and serological profile. Eur Arch Otorhinolaryngol 2007;264(5):505-7.

6. van Loon AM, van der Logt JT, Heessen FW, Heeren MC, Zoll J. Antibody-capture enzyme-linked immunosorbent assays that use enzyme-labelled antigen for detection of virus-specific immunoglobulin M, A and G in patients with varicella or herpes zoster. Epidemiol Infect 1992;108(1):165-74.

7. Lin YY, Kao CH, Wang CH. Varicella zoster virus infection of the pharynx and larynx with multiple cranial neuropathies. Laryngoscope 2011;121(8):1627-30.