Ramsay Hunt Syndrome
Original Editor - Wendy Walker
Top Contributors - Wendy Walker, Kim Jackson, Poonam Sepat, Evan Thomas, WikiSysop, Claire Knott, Alexandra Stead, Naomi O'Reilly and Wendy Snyders
Introduction
Ramsay Hunt Syndrome [RHS], also known as "herpes zoster oticus", is a peripheral facial palsy (cranial nerve VII) caused by reactivation of latent Varicella-zoster virus[VZV][1] in the geniculate ganglion. It is characterised by ipsilateral facial paralysis accompanied by a vesicular rash in the ear canal, auricle or oral cavity though occasionally there will be no rash visible ("zoster sine herpete").[1] All the communicating nerves (cervical nerves C2, C3, and C4) and CNs V, VIII, IX, and X) can also be involved but cases of polyneuropathy including the many communicating nerves are rare.[1]
The syndrome was first described by Dr J. Ramsey Hunt, in 1907[2] RHS accounts for approximately 12% of all peripheral facial palsy cases[3] and has a significantly worse prognosis than Bell's Palsy, with higher rates of long-term sequelae due to incomplete recovery.[4]
When compared with Bell's Palsy, RHS has a higher incidence of incomplete recovery with longstanding sequelae.[5][6]
Mechanism of Injury / Pathological Process
RHS is caused by reactivation of the varicella-zoster virus (VZV) which remains within cranial nerve ganglia (particularly the geniculate ganglion of the Facial Nerve, CN VII) following primary infection ie. Chickenpox. Reactivation occurs due to :
- Immune system decline (due to illness, aging, stress, immunosuppression)
- The viral reactivation causes inflammation and oedema within the confined bony facial canal
- This compression on the nerve in the facial canal leads to facial paralysis
- Concurrent damage to the Auditory/Vestibulocochlear nerve (CNVIII) means that the patient frequently also has loss of hearing, and/or vestibular symptoms.
Risk factors for the reactivation VZV include upper respiratory infections, smoking, diabetes, emotional stress, immunosuppresive therapy, cancer and chronic renal failure.[3]
Clinical Presentation
Cardinal Triad (Classic Presentation)
The classic presentation for the cardinal triad includes:[3]
- Ipsilateral facial paralysis (acute onset)
- Otalgia - severe ear pain, often preceding palsy by hours or even days
- Vesicular rash in the Ramsay Hunt Zone : external auditory canal (most common area); auricle (pinna); tympanic membrane; anterior 2/3 of tongue; Hard/soft palate.
Important: up to 20% of cases have facial palsy WITHOUT a visible rash initially ("zoster sine herpete"); the rash may appear later or not at all.
Associated Symptoms (Frequent)
Cranial Nerve VIII (Vestibulocochlear) Involvement - occurs in approx 40% of cases:[3]
- Hearing loss (most often high-frequency sensorineural)
- Vertigo/dizziness (may be severe)
- Balance problems
- Tinnitus
- Nystagmus
Other Features:
- Hyperacusis (increased sensitivity to sound, due to stapedius muscle paralysis)
- Dry Eye (reduced lacrimation)
- Altered taste (anterior 2/3 tongue - chorda tympani involvement)
Systemic Symptoms:
- Fever, malaise, headache (prodromal viral symptoms)
- Cervical lymphadenopathy
Diagnostic Procedures
RHS diagnosis is largely based on history, clinical findings, and neurological examination.[7] Analysis of cerebrospinal fluid and MRI (brain) has limited diagnostic value.[7]
Differential Diagnosis
The following conditions can present in with similar symptoms:
- Bell's Palsy - this is the most common cause of sudden onset, non-traumatic facial palsy, and can be differentiated from RHS by the absence of severe otalgia (only mild pain in the region of the mastoid usually occurs in Bell's Palsy) and the absence of vesicles and involvement of other cranial nerves.
- Acoustic Neuroma - MRI scan should be used to exclude this
- Lyme Disease - history of tick bite/rash, facial palsy may be bilateral
Medical Management
Corticosteroids and antiviral medication are the 1st line treatment for RHS, providing the best results when treatment starts within 72 hours of t of symptoms.[8][9][10] Over 80% of patients who start antiviral medication within 72 hours have good recovery.[1] Antiviral agents such as acyclovir reduce acute pain, improve the herpes zoster lesions and prevent postherpetic neuralgia.[7]
Physiotherapy Interventions
Physiotherapy management of the facial paralysis or weakness is as detailed in the section on Facial Palsy, for an assessment format see Lower Motor Neuron Facial Palsy Assessment page.
It is also important to provide information on care of the eye in order to prevent the formation of corneal ulcer: see advice page on Dry Eye. Referral to an ophthalmologist should be considered.
RHS patients with long term facial palsy may also start to experience dental problems: see advice page on Dental Issues in Facial Palsy.
References
- ↑ 1.0 1.1 1.2 1.3 Kanerva M, Jones S, Pitkaranta A. Ramsay Hunt syndrome: characteristics and patient self-assessed long-term facial palsy outcome. European Archives of Oto-Rhino-Laryngology. 2020 Apr;277(4):1235-45.
- ↑ J. Ramsay Hunt, On Herpetic Inflammations of the Geniculate Ganglion. A New Syndrome and its Complications. Journal of Nervous and Mental Disease, February 1907, Volume 34, Issue 2, pp 73-96
- ↑ 3.0 3.1 3.2 3.3 Psillas G, Dova S, Ieridou F, Kyrgidis A, Constantinidis J. Ramsay Hunt syndrome: clinical presentation and prognostic factors. B-ENT. 2019 Jan 1;15(4):297-302.
- ↑ C J Sweeney, D H Gilden. Ramsay Hunt Syndrome J Neurol Neurosurg Psychiatry 2001;71:149-154
- ↑ Cai Z1, Li H, Wang X, Niu X, Ni P, Zhang W, Shao B. Prognostic factors of Bell's palsy and Ramsay Hunt syndrome. Medicine (Baltimore). 2017 Jan;96(2):e5898.
- ↑ Hah YM, Kim SH, Jung J, Kim SS, Byun JY, Park MS, Yeo SG. Prognostic value of the blink reflex test in Bell's palsy and Ramsay-Hunt syndrome. Auris Nasus Larynx. 2018 Oct;45(5):966-970. doi: 10.1016/j.anl.2018.01.007. Epub 2018 Feb 3.
- ↑ 7.0 7.1 7.2 Jeon Y, Lee H. Ramsay hunt syndrome. Journal of Dental Anesthesia and Pain Medicine. 2018 Dec 1;18(6):333-7.
- ↑ Hato N, Murakami S, Gyo K. Steroid and antiviral treatment for Bell's palsy. Lancet 2008; 371: 1818–20
- ↑ 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.
- ↑ Daniel P Butler and Adriaan O Grobbelaar. Facial palsy: what can the multidisciplinary team do? J Multidiscip Healthc. 2017; 10: 377–381. Published online 2017 Sep 25. doi: 10.2147/JMDH.S125574