Unilateral facial nerve palsy and vestibulocochlear neuropathy due to herpes zoster are named Ramsay Hunt syndrome (RHS). Rarely, this infection may affect multiple cranial nerves concurrently. Through this report, we are describing a case of RHS, where the VII, VIII, IX, and X cranial nerves were involved simultaneously. This 65-year-old man presented with right lower motor neuron facial nerve palsy, hoarseness, dysphagia, hearing deficit, and vertigo following painful vesicular eruptions on his right ear. Systemic corticosteroids and antiviral agents were administered, subsequent to which the patient improved with the resolution of all the symptoms and signs.
Keywords: Acyclovir, corticosteroid, herpes zoster, Ramsay Hunt syndrome, varicella-zoster virus
| Introduction|| |
The virus responsible for herpes zoster is the varicella-zoster virus (VZV). This enters the cutaneous nerve endings after an episode of chickenpox (Varicella) and lies in a dormant stage in the dorsal root ganglia in the spinal cord. VZV may also remain dormant for decades in cranial nerves such as trigeminal and facial and also autonomic ganglia. Herpes zoster occurs due to the reactivation of this virus. Reactivation may be spontaneous (due to increasing age, physical trauma, and psychological stress) or when host defenses get compromised (following radiotherapy/chemotherapy for malignancy, transplant recipients, high-dose steroids, and HIV infection). When the herpes zoster lesion involves the ear along with facial nerve palsy, it is called Ramsay Hunt syndrome (RHS). Rarely, it may involve multiple cranial nerves such as the vagus causing unilateral vocal cord paralysis. In comparison to the Bell's palsy, where facial nerve paralysis is due to herpes simplex virus, the RHS patients have a higher grade of facial palsy from the start and often have incomplete recovery. We report a rare case of VZV cranial polyneuritis with complete recovery.
| Case Report|| |
A 65-year-old man visited the outpatient department with complaints of pain in the right ear, change in voice, difficulty swallowing, and deviation of mouth to the left side and unable to close his right eye for 10 days. He also complained of hearing loss in the right ear with a spinning sensation in the head and a tendency to fall on walking. On detailed history, he reported that he had fever with some rash over the right ear during the initial 3 days of illness. There was no history of trauma to the head, severe headache, projectile vomiting, high blood pressure, or seizures. The pain was continuous, severe, and radiating to the temporal area. He had taken treatment for the same from a general practitioner (antibiotics and analgesics), with no relief. He also recalled having suffered from chickenpox in early childhood. He did not suffer from any other chronic illness.
On examination, the patient had difficulty walking without support. There was no nystagmus, either spontaneous or gaze induced. The right ear had resolving clustered vesicles in the concha and the external canal was painted with gentian violet [Figure 1]. He had moderately severe dysfunction of the facial nerve that corresponded to grade IV House-Brackmann right lower motor neuron facial paralysis with incomplete right eye closure and deviation of mouth to the left [Figure 2]. On examination of the oropharynx, there was right palatal paralysis with a deviation of palate and uvula on the left side [Figure 3] with absent gag reflex on the right side. On laryngoscopy, there was right vocal cord paralysis [Figure 4]. Pure tone audiometry showed right ear and mild (40 dB) sensorineural hearing loss. A spontaneous nystagmus was present on the gaze test that was grade III, with fast component toward the left ear. The head impulse test was positive with the presence of corrective saccades. The test for skew was negative, thus suggesting peripheral vertigo. No other neurological deficit was evident. The top diagnostic tests for identifying the site of the lesion of the facial nerve were performed. There was decreased lacrimation in the right eye on Schirmer's test, loss of stapedial reflex on tympanometry, and ageusia on the right anterior part of the tongue. These findings suggested a lesion at the geniculate or suprageniculate area. The patient did not consent to viral serology and radiological imaging due to financial constraints. He was started on oral valacyclovir 1000 mg TID, oral prednisolone 60 mg OD, local acyclovir ointment, methylcellulose eye drops, and speech/swallowing therapy. He had good improvement and recovery of function of all 7th, 8th, 9th, and 10th cranial nerves at the 2-month follow-up. The patient was advised HZV vaccine to prevent further recurrences, but he refused due to the high cost.
|Figure 1: Clinical picture of the right-side ear showing resolving vesicular lesion in the concha, coated with gentian violet paint|
Click here to view
|Figure 2: Clinical picture of the patient, showing grade IV right lower motor neuron facial paralysis with an inability to close right eye on maximum effort, deviation of mouth to left, and loss of forehead wrinkles on the right side|
Click here to view
|Figure 3: Clinical picture of oropharynx examination showing soft palate and uvula deviation to left on saying “Aah”|
Click here to view
|Figure 4: Endoscopic picture of the larynx, showing right vocal cord in paramedian position on saying “Ee” with pooling of saliva in right pyriform sinus|
Click here to view
I thought that I had developed stroke and would become paralyzed for life, but I am happy I have recovered.
| Discussion|| |
Herpes zoster has a high incidence in the elderly and involves facial nerves in 1% of cases. RHS occurs when the dormant VZV in the facial nerve geniculate ganglion becomes active. The involvement of the facial nerve, sensory (auricular) branch leads to vesicle formation in the concha and external canal and motor branch involvement causes ipsilateral lower motor neuron facial palsy. Sometimes, the patient of RHS may not have vesicles (zoster sine herpete), as seen in Bell's palsy. Often, herpetic vesicles may appear first and heal by the time neuromuscular symptoms appear, thus missing the diagnosis of herpes zoster on delayed examination.
The facial nerve and vestibulocochlear nerve travel in close proximity in the internal auditory meatus, hence simultaneous involvement of the eighth nerve is not uncommon. The clinical presentation additionally has vertigo, tinnitus, sensorineural hearing loss, and nystagmus. Involvement of multiple cranial nerves by VZV is rare, affecting 1.8% of patients with RHS.
Cranial polyneuropathy in VZV may occur due to perineural spread along nerve anastomosis in the skin lining the external auditory canal and blood vessel anastomosis from the infected carotid artery or local meningitis. Other theories of nerve involvement and muscle paralysis in VZV infection are direct nerve cell damage, allergic response of tissues to VZV, and occlusive vasculitis.
Cranial polyneuritis should prompt an evaluation for cerebrovascular disease, meningitis, encephalitis, or malignancy. Contrast magnetic resonance imaging may help in differentiating these by showing enhancement of nerve in the internal auditory canal, intratemporal facial nerve, and membranous labyrinth in patients with RHS. In addition, glossopharyngeal and vagus nerves may show thickening and enhancement. This enhancement occurs due to the breach of the blood–brain–nerve barrier and passage of contrast into endoneurium.
Empirical antiviral treatment should be started early (ideally within 72 h) to contain the duration and severity of illness. It helps by limiting the viral spread and improving VZV vasculopathy. When therapy is started within 3 days versus 4–7 days versus >8 days, paresis resolves in 75%, 48%, and 30%, respectively. Acyclovir (800 mg five times a day) or valacyclovir (1000 mg, three times a day) is continued for 7–10 days. Corticosteroids (prednisolone) should be administered started early (60 mg daily) and tapered slowly. They facilitate resolution by reducing inflammation in affected arteries.
IgM antibody for VZV is useful in confirming a recent infection, although it is not detectable till 8–10 days of appearance of vesicles and also may be negative in some cases. Herpes zoster, if left untreated may have ocular problems (keratitis, uveitis, and retinal necrosis), meningoencephalitis, bacterial superinfection of lesions, and postherpetic neuralgia. Since incidence increases with advancing age, VZV vaccine is often given in the elderly to prevent herpes zoster, although it has 51% 5-year efficacy.
| Conclusion|| |
Herpes zoster should always be considered an etiological differential in a patient with cranial polyneuropathy. The earlier the diagnosis is clinched by thorough clinical examination and treatment is initiated, the better the prognosis and lesser the severe neurological sequelae. Although neuroimaging studies may show nerve thickening and enhancement in some cases, they are not mandatory, especially in settings with limited resources. A good response to treatment is often surrogate evidence of the diagnosis.
Patient informed consent
The patient has provided the authors with written informed consent for publication of his clinical photographs in medical journal for disseminating the knowledge.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given his consent for his images and other clinical information to be reported in the journal. The patient understands that his name and initials will not be published and due efforts will be made to conceal his identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
| References|| |
Zerboni L, Sen N, Oliver SL, Arvin AM. Molecular mechanisms of varicella zoster virus pathogenesis. Nat Rev Microbiol 2014;12:197-210.
Vineet DA, Mithra R, Baskaran P, Mishra S. Oro-facial herpes zoster: A case report with a detailed review of literature. Oral Maxillofac Pathol J 2013;4:346-54.
Shim HJ, Jung H, Park DC, Lee JH, Yeo SG. Ramsay Hunt syndrome with multicranial nerve involvement. Acta Otolaryngol 2011;131:210-5.
Wagner G, Klinge H, Sachse MM. Ramsay Hunt syndrome. J Dtsch Dermatol Ges 2012;10:238-44.
Shinha T, Krishna P. Ramsay Hunt syndrome and zoster laryngitis with multiple cranial nerve involvement. IDCases 2015;2:47-8.
Kim YH, Chang MY, Jung HH, Park YS, Lee SH, Lee JH, et al.
Prognosis of Ramsay Hunt syndrome presenting as cranial polyneuropathy. Laryngoscope 2010;120:2270-6.
Babu K, Kini R, Murthy KR. Unique presentation of 3rd
cranial nerve palsies, nodular scleritis and nummular keratouveitis in an immunocompetent patient following an attack of herpes zoster ophthalmicus. Ocul Immunol Inflamm 2012;20:365-7.
Chiang F, Panyaping T, Tedesqui G, Sossa D, Costa Leite C, Castillo M. Varicella zoster CNS vascular complications. A report of four cases and literature review. Neuroradiol J 2014;27:327-33.
Kim TU, Kim SY, Kim JI, Lee SJ. Ramsay-Hunt syndrome accompanied by dysphagia: A videofluoroscopic swallowing study. Am J Phys Med Rehabil 2013;92:547-50.
Amlie-Lefond C, Jubelt B. Neurologic manifestations of varicella zoster virus infections. Curr Neurol Neurosci Rep 2009;9:430-4.
Choi JH. Two cases of pharyngolaryngeal zoster advanced to multiple cranial neuropathy. Am J Otolaryngol 2013;34:369-72.
Nair P, Gharote H, Singh P, Jain-Choudhary P. Herpes zoster on the face in the elderly. BMJ Case Rep 2014;2014:bcr2013200101.
Hales CM, Harpaz R, Ortega-Sanchez I, Bialek SR, Centers for Disease Control and Prevention (CDC). Update on recommendations for use of herpes zoster vaccine. MMWR Morb Mortal Wkly Rep 2014;63:729-31.
Department of General Medicine, Government Medical College and Hospital, Sector 32, Chandigarh - 160 030
Source of Support: None, Conflict of Interest: None
[Figure 1], [Figure 2], [Figure 3], [Figure 4]