Ramsay Hunt Syndrome: Difference between revisions

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'''Original Editor '''- [[User:Wendy Walker|Wendy Walker]]  
'''Original Editor '''- [[User:Wendy Walker|Wendy Walker]]  


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== Introduction  ==
== Introduction  ==


Ramsey Hunt Syndrome [RHS] is a peripheral facial nerve palsy caused by an infection with the virus Varicella Zoster virus.  
Ramsay Hunt Syndrome [RHS] is a peripheral facial palsy or cranial nerve (CN) VII and is caused by reactivation of of latent [[Herpes_Zoster|Varicella Zoster]] virus<ref name=":0">Kanerva M, Jones S, Pitkaranta A. [https://link.springer.com/content/pdf/10.1007/s00405-020-05817-y.pdf?pdf=button%20sticky 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.</ref>.  It is generally accompanied by herpes blisters in the neck and head areas, often in the ear and mouth acvity<ref name=":0" /> however, occasionally there will be no rash visible. 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<ref name=":0" />. About 12% of peripheral facial palsy cases are associated with RHS<ref name=":1">Psillas G, Dova S, Ieridou F, Kyrgidis A, Constantinidis J. [http://www.b-ent.be/Content/files/sayilar/2/2019-15-4-297-Psillas.pdf Ramsay Hunt syndrome: clinical presentation and prognostic factors.] B-ENT. 2019 Jan 1;15(4):297-302.</ref>.


It is generally accompanied by a rash (which can be painful) in the area of the ear and/or the mouth.  
The syndrome is named after Dr J. Ramsey Hunt, the physician who first described the syndrome at a meeting of the American Neurological Association in 1906. He subsequently published his article on the subject in 2007<ref>J. Ramsay Hunt, [https://ia600708.us.archive.org/view_archive.php?archive=/22/items/crossref-pre-1909-scholarly-works/10.1097%252F00005053-190601000-00019.zip&file=10.1097%252F00005053-190702000-00001.pdf 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</ref>.


The auditory nerve may also be involved, leading to hearing and balance effects.  
When compared with Bell's Palsy, RHS has a higher incidence of incomplete recovery with longstanding sequelae<ref>Cai Z1, Li H, Wang X, Niu X, Ni P, Zhang W, Shao B.


The syndrome is named after Dr J. Ramsey Hunt, the physician who first described the syndrome at a meeting of the American Neurological Association in 1906. He subsequently published his article on the subject in 2007<ref>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</ref>.
[https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5266197/ Prognostic factors of Bell's palsy and Ramsay Hunt syndrome].  Medicine (Baltimore). 2017 Jan;96(2):e5898.
</ref><ref>Hah YM, Kim SH, Jung J, Kim SS, Byun JY, Park MS, Yeo SG. [https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5266197/ 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.</ref>.


== Mechanism of Injury / Pathological Process  ==
== Mechanism of Injury / Pathological Process  ==


RHS is caused by reactivation of the varicella zoster virus (the virus which causes chicken pox) within the sensory ganglion of the Facial Nerve, the 7th Cranial Nerve. As the 8th Cranial Nerve, known as the Auditory Nerve or the Vetibulocochlear Nerve, lies next to the sensory ganglion of the facial nerve within the facial canal, both nerves are usually involved<ref>C J Sweeney, D H Gilden.  Ramsay Hunt Syndrome J Neurol Neurosurg Psychiatry 2001;71:149-154</ref>.<br>
RHS is caused by reactivation of the varicella-zoster virus (VZV) within the dorsal ganglion of the Facial Nerve, the 7th Cranial Nerve. As the 8th cranial nerve, known as the auditory nerve or the vestibulocochlear nerve, lies next to the sensory ganglion of the facial nerve within the facial canal, both nerves are usually involved<ref>C J Sweeney, D H Gilden.  [https://jnnp.bmj.com/content/jnnp/71/2/149.full.pdf Ramsay Hunt Syndrome] J Neurol Neurosurg Psychiatry 2001;71:149-154</ref>.


[[Image:PP RHS.png|right|300px]]
Risk factors for the reactivation VZV include upper respiratory infections, smoking, diabetes, emotional stress, immunosuppresive therapy, cancer and chronic renal failure<ref name=":1" />.  


== Clinical Presentation  ==
== Clinical Presentation  ==
 
[[Image:PP RHS.png|right|300px]]The most common characteristics are acute facial palsy, otalgia (ear ache) and red vesicular rashes in the external auditory canal and pinna<ref name=":1" />. The otalgia may occur at the same time as the facial palsy, or the palsy may occur a few days after the onset of earaches. Other symptoms such as nystagmus, nausea, vomiting, tinnitus, hearing loss (sensorineural), vertigo and temporal headaches can also be present<ref name=":1" />.  
The most common presenting feature is a moderate to severe earache (otalgia). This may occur at the same time as facial palsy, or the palsy may occur a few days after the onset of earache.
 
A painful red rash with blisters (as in the illustration) commonly appears; the rash can be on the pinna of the ear, within the ear canal, or inside the mouth.
 
In addition the patient may have general signs of a viral infection, including fever, headache and general malaise.<br>
 
A number of patients suffer from ipsilateral hearing loss, and/or tinnitus (ringing/noises in the ears) and/or balance difficulties.


== Diagnostic Procedures  ==
== Diagnostic Procedures  ==


The diagnosis of RHS is a clinical diagnosis, made when the combination of ear pain, a herpetic rash in the ear or mouth and facial palsy occurs; however, virological studies can be used to confirm the diagnosis.  
RHS diagnosis is largely based on history, clinical findings, and neurological examination<ref name=":2">Jeon Y, Lee H. [https://synapse.koreamed.org/articles/1110839 Ramsay hunt syndrome]. Journal of Dental Anesthesia and Pain Medicine. 2018 Dec 1;18(6):333-7.</ref>.Analysis of cerebrospinal fluid and MRI (brain) has limited diagnostic value<ref name=":2" />.
 
MRI scanning can be used to exclude other causes of facial and auditory nerve dysfunction (such as Acoustic Neuroma).  


== Differential Diagnosis  ==
== Differential Diagnosis  ==
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The following conditions can present in with similar symptoms:  
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.  
*[[Bell's_Palsy|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.  
*Postherpetic Neuralgia - not associated with facial palsy  
*Postherpetic Neuralgia - not associated with facial palsy  
*Temporomandibular Disorders - not associated with facial palsy  
*[[Acoustic Neuroma|Acoustic Neuroma]] - MRI scan should be used to exclude this
*Trigeminal Neuralgia - not associated with facial palsy<br>
*[[Temporomandibular Disorders]] - not associated with facial palsy  
*[[Trigeminal Neuralgia|Trigeminal Neuralgia]] - not associated with facial palsy


== Medical Management  ==
== Medical Management  ==


Corticosteroids and antiviral medication are the 1st line treatment for RHS, providing the best results when treatment starts within 72 hours of onset of symptoms.
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<ref>Hato N, Murakami S, Gyo K. Steroid and antiviral treatment for Bell's palsy. Lancet 2008; 371: 1818–20</ref><ref>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.</ref><ref>Daniel P Butler and Adriaan O Grobbelaar.  [https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5626419/pdf/jmdh-10-377.pdf 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
 
</ref>. Over 80% of patients who start antiviral medication within 72 hours have good recovery<ref name=":0" />.  Antiviral agents such as acyclovir reduce acute pain, improve the herpes zoster lesions and prevent postherpetic neuralgia<ref name=":2" />.
Patients who suffer from vertigo may also benefit from vestibular suppressant medication, and analgesia may be required to control the ear pain.


== Physiotherapy Interventions  ==
== Physiotherapy Interventions  ==
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Physiotherapy management of the facial paralysis or weakness is as detailed in the section on [[Facial Palsy|Facial Palsy]].  
Physiotherapy management of the facial paralysis or weakness is as detailed in the section on [[Facial Palsy|Facial Palsy]].  


It is also important to provide information on care of the eye in order to prevent formation of corneal ulcer: see advice page on [[Dry Eye|Dry Eye]]. Referral to an opthalmologist should be considered.
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|Dry Eye]]. Referral to an opthalmologist 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.<br>
RHS patients with long term facial palsy may also start to experience dental problems: see advice page on [[Dental Issues in Facial Palsy|Dental Issues in Facial Palsy]].  
 
== Resources  ==
 
The charity [http://www.facialpalsy.org.uk/about-facial-palsy/causes-diagnoses/ramsay-hunt-syndrome/55 Facial Palsy UK] have a page on RHS
 
The website [http://ramsayhunt.org/ RamsayHunt.org]&nbsp;offers information and support
 
American organisation [https://rarediseases.org/rare-diseases/ramsay-hunt-syndrome/ National Organization for Rare Disorders] has a useful page on RHS
 
== Recent Related Research (from [http://www.ncbi.nlm.nih.gov/pubmed/ Pubmed])  ==
<div class="researchbox">
<rss>http://www.ncbi.nlm.nih.gov/entrez/eutils/erss.cgi?rss_guid=1PAjVVEin27IaEPoWa7fz5k1_hztXaknK1vTt1wxgmW0R-pQFH|charset=UTF-8|short|max=10</rss>
</div>
== References  ==
== References  ==


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[[Category:Conditions]] [[Category:Neurology]]
[[Category:Conditions]]  
[[Category:Neurological - Conditions]]
[[Category:Facial_Palsy]]
[[Category:Primary Contact]]
[[Category:Syndromes]]

Latest revision as of 10:25, 14 March 2023

Original Editor - Wendy Walker

Top Contributors - Wendy Walker, Poonam Sepat, Kim Jackson, Claire Knott, Evan Thomas, WikiSysop, Wendy Snyders and Naomi O'Reilly


Introduction[edit | edit source]

Ramsay Hunt Syndrome [RHS] is a peripheral facial palsy or cranial nerve (CN) VII and is caused by reactivation of of latent Varicella Zoster virus[1]. It is generally accompanied by herpes blisters in the neck and head areas, often in the ear and mouth acvity[1] however, occasionally there will be no rash visible. 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]. About 12% of peripheral facial palsy cases are associated with RHS[2].

The syndrome is named after Dr J. Ramsey Hunt, the physician who first described the syndrome at a meeting of the American Neurological Association in 1906. He subsequently published his article on the subject in 2007[3].

When compared with Bell's Palsy, RHS has a higher incidence of incomplete recovery with longstanding sequelae[4][5].

Mechanism of Injury / Pathological Process[edit | edit source]

RHS is caused by reactivation of the varicella-zoster virus (VZV) within the dorsal ganglion of the Facial Nerve, the 7th Cranial Nerve. As the 8th cranial nerve, known as the auditory nerve or the vestibulocochlear nerve, lies next to the sensory ganglion of the facial nerve within the facial canal, both nerves are usually involved[6].

Risk factors for the reactivation VZV include upper respiratory infections, smoking, diabetes, emotional stress, immunosuppresive therapy, cancer and chronic renal failure[2].

Clinical Presentation[edit | edit source]

PP RHS.png

The most common characteristics are acute facial palsy, otalgia (ear ache) and red vesicular rashes in the external auditory canal and pinna[2]. The otalgia may occur at the same time as the facial palsy, or the palsy may occur a few days after the onset of earaches. Other symptoms such as nystagmus, nausea, vomiting, tinnitus, hearing loss (sensorineural), vertigo and temporal headaches can also be present[2].

Diagnostic Procedures[edit | edit source]

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[edit | edit source]

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.
  • Postherpetic Neuralgia - not associated with facial palsy
  • Acoustic Neuroma - MRI scan should be used to exclude this
  • Temporomandibular Disorders - not associated with facial palsy
  • Trigeminal Neuralgia - not associated with facial palsy

Medical Management[edit | edit source]

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[edit | edit source]

Physiotherapy management of the facial paralysis or weakness is as detailed in the section on Facial Palsy.

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 opthalmologist 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[edit | edit source]

  1. ↑ 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.
  2. ↑ 2.0 2.1 2.2 2.3 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.
  3. ↑ 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
  4. ↑ 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.
  5. ↑ 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.
  6. ↑ C J Sweeney, D H Gilden. Ramsay Hunt Syndrome J Neurol Neurosurg Psychiatry 2001;71:149-154
  7. ↑ 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.
  8. ↑ Hato N, Murakami S, Gyo K. Steroid and antiviral treatment for Bell's palsy. Lancet 2008; 371: 1818–20
  9. ↑ 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.
  10. ↑ 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


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