Ramsay-Hunt Syndrome

Ramsay-Hunt Syndrome or Herpes zoster oticus is a syndrome characterised by acute otalgia, herpetic vesicular rash associated with facial palsy. This is the second most common cause of facial paralysis and is due to reactivation of the varicella-zoster virus that remains dormant/latent in the geniculate ganglion after primary infection with chickenpox. Characteristic presentation is severe acute otalgia with unilateral facial paralysis. Vesicular eruptions if not present initially, may appear usually within three to five days. The vesicles may appear on post-auricular skin, pinna, concha, ear canal, and tympanic membrane. Occasionally it may affect neck, shoulder or oral cavity. Besides facial nerve, it may affect other cranial nerves as well.

Ramsay-Hunt in 1907 first described Herpes zoster oticus in a patient with otalgia associated with cutaneous and mucosal rashes, which was ascribed to geniculate ganglion infection with varicella zoster virus (human herpes virus type 3). This syndrome is also known by the names geniculate neuralgia or nervus intermedius neuralgia.

As compared to Bell’s palsy, the symptoms are more severe and carries worse prognosis in Ramsay Hunt syndrome. The risk and frequency of complete facial nerve degeneration is substantially higher than the Bell’s palsy. Patients with vestibular and auditory dysfunction in addition to facial paralysis generally also have worse prognosis. Patients with diabetes, hypertension and advanced age have poorer prognosis.

Bilateral Ramsay Hunt syndrome has also been reported in diabetic patients or immunocompromised patients.

Other cranial nerve neuropathies e.g. neuropathy of V,VI,VIII,IX, and X, may be associated with Ramsay Hunt syndrome.

 

References

Gross G, Doerr HW. Herpes Zoster- Recent Aspects of Diagnosis and Control. S Karger AG, P.O. Box, CH 4009 Basel (Switzerland) 2006. P 47- 57.

Brackmann Derald E, Shelton Clough, Arriaga Moisés A. Otologic Surgery Third Edition. Saunders, an imprint of Elsevier Inc. 2010. P 338- 346.

Dickson Gretchen, Kellerman Rick. Primary Care: Clinics in Office Practice Volume 41, Number 1- Primary Care ENT. Elsevier Inc. 2014. P 137- 138.

Roos Karen L. Emergency Neurology. Springer Science+Business Media, LLC 2012. P 133- 142.

Adams James G, Barton Erik D, Collings Jamie, DeBlieux Peter M C, Gisondi Michael A, Nadel Eric S. Emergency Medicine Clinical Essentials Second Edition. Saunders, an imprint of Elsevier Inc. 2013. P 824- 827.

Denniston Alastair K O, Murray Philip I. Oxford Handbook of Ophthalmology Third Edition. Oxford University Press 2014. P 707.

Tandon Praksh Narain, Ramamurthi Ravi. Ramamurthi and Tandon’s Textbook of Neurosurgery Third Edition Vol 1. Jaypee Brothers Medical Publishers (P) Ltd 2012. P 831- 832.

Washington Tabitha A, Brown Khalilah M, Fanciullo Gilbert J. Pain. Oxford University Press 2012. P 69- 73.

https://emedicine.medscape.com/article/1166804-overview

http://www.jaypeejournals.com/eJournals/ShowText.aspx?ID=3041&Type=FREE&TYP=TOP&IN=&IID=235&isPDF=YES

Commonly, Ramsay Hunt syndrome is a disease affecting elderly people or someone with immune deficiency. However, it may be seen in younger patients under conditions of stress.

Symptoms may include

  • Paroxysmal pain deep within the ear, which precedes rashes
  • Herpetiform eruptions
  • Impaired hearing
  • Vertigo
  • Facial nerve palsy
  • Loss of sensation of taste in anterior two third of tongue
  • Nystagmus

 

 

Ramsay Hunt syndrome is caused due to reactivation of Varicella zoster virus (VZV) lying dormant in geniculate ganglion.

Primary infection with Varicella zoster virus (VZV), known as varicella or chickenpox is a common erythematous infection of the paediatric age group. VZV remains dormant in cranial nerve neurons and dorsal root ganglia. Subsequent reactivation, more so during periods of stress, may result in prodromal period of severe pain followed by localised vesicular rash, known as herpes zoster infection.

In Ramsay Hunt syndrome, patient develop otalgia associated with mucosal and cutaneous lesions, due to reactivation of infection in geniculate ganglion of the facial nerve with varicella zoster virus (human herpes virus type 3).

Diagnosis of Ramsay Hunt syndrome is based upon the clinical history of otalgia associated with vesicular lesions or eschars, and facial paralysis.

Prodromal symptoms of fatigue for one to two weeks is followed by phase of vesicular eruptions. Erythematous macules/papules develop around ear (commonly affecting concha or external auditory canal). Soon it turns into vesicles which may ulcerate.

The clinical presentation varies in severity from case to case. General features include periauricular pain, herpetiform eruptions and neural dysfunction, involving VII and VIII cranial nerve predominantly.

 

Histology:

There is swelling and inflammation of the ganglion of affected cranial nerve with VZV. Inflammation is predominantly lymphocytic in nature. Some cells of the ganglion show swelling and others may be degenerated.

 

Investigations:

  • Magnetic Resonance Imaging (MRI): Magnetic Resonance Imaging shows enhancement of a large portion of facial nerve. Often the vestibular and cochlear nerve, labyrinth, and dura lining internal auditory canal may also show enhancement.
  • Brainstem auditory evoked response: Brainstem auditory evoked response shows significant increase in latencies of certain waves, and also inter-peak latencies in others.
  • Saliva: VZV DNA level in saliva helps to understand viral reactivation in facial nerve and oropharyngeal epithelium.
  • Cerebrospinal fluid: Real time Polymerase Chain Reaction (PCR) of CSF may be done to diagnose this virus.

Routine imaging is unnecessary. Even electro-diagnostic studies are unreliable in Ramsay Hunt syndrome.

 

Differential diagnosis

Primary initial differentiation is between facial neuropathy and a diagnosis of stroke. Once it is ascertained that the diagnosis is not stroke, than the differentiation is between Bell’s palsy and secondary causes of facial neuropathy. Bell’s palsy accounts for up to 70% of facial neuropathies. Clue to secondary causes include involvement of other cranial nerves or neurologic diseases. Ramsay Hunt syndrome is the second most common cause. The external auditory canal, mouth and tongue should be examined for vesicles to distinguish this condition from Bell’s palsy.

Other conditions to be distinguished are

  • Lyme disease
  • Guillain-Barré syndrome
  • Meningitis
  • HIV infection.

 

 

Management includes

Medical therapy

Combination of steroids and antiviral agents: Use of Combination of steroids and antiviral agents have shown a higher recovery rate. In one study, it was clearly shown that patients treated within three days of the onset showed complete recovery in seventy five present of patients, whereas, only thirty percent showed complete recovery when treated after seven days. Patients show rapid reduction of pain, and occasionally experience return of facial movement.

Due to diffuse neuritis with intervening ‘skip’ regions of facial nerve, surgical decompression of nerve in Ramsay Hunt syndrome is not recommended.

 

Prognosis

With early onset of medical therapy within three days, almost or complete resolution is seen in most of the cases. Cases missing indicative cutaneous symptoms (zoster sine herpete), but showing combination of otalgia, sensorineural hearing loss, vertigo or any kind of facial paresis, raises suspicion of Ramsay Hunt syndrome.

  • PUBLISHED DATE : Mar 05, 2019
  • PUBLISHED BY : NHP Admin
  • CREATED / VALIDATED BY : Dr. S. C. Gupta
  • LAST UPDATED ON : Mar 05, 2019

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