Ramsay Hunt Syndrome: Difference between revisions

mNo edit summary
(Corrected editors code)
 
(44 intermediate revisions by 7 users not shown)
Line 2: Line 2:
'''Original Editor '''- [[User:Wendy Walker|Wendy Walker]]  
'''Original Editor '''- [[User:Wendy Walker|Wendy Walker]]  


'''Lead Editors''' - {{Special:Contributors/{{FULLPAGENAME}}}}  
'''Top Contributors''' - {{Special:Contributors/{{FULLPAGENAME}}}}  
 
</div>  
</div>  
<br>  
<br>  
Line 8: Line 9:
== Introduction  ==
== Introduction  ==


Ramsay Hunt [RHS] syndrome is defined as an acute peripheral [[Facial Palsy|facial palsy]]/neuropathy associated with erythematous vesicular rash of the skin of the ear canal, auricle (also termed herpes zoster oticus), and/or mucous membrane of the oropharynx<ref>Bhupal HK. Ramsay Hunt syndrome presenting in primary care. Practitioner. 2010 Mar. 254(1727):33-5</ref>. It is commonly asociated with moderate to severe otalgia (earache).<br>  
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>.
 
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>.


This syndrome is also known as geniculate neuralgia or nervus intermedius neuralgia.  
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&nbsp;was first described in 1907 by Dr James Ramsay Hunt, describing a patient who had otalgia (ear pain) associated with cutaneous and mucosal rashes, which he ascribed to infection of the geniculate ganglion by human herpesvirus 3 (ie, varicella-zoster virus [VZV])<ref>Goldani LZ, Ferreira da Silva LF, Dora JM. Ramsay Hunt syndrome in patients infected with human immunodeficiency virus. Clin Exp Dermatol. 2009 Jun 1</ref>. <br>
[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  ==


Ramsay Hunt syndrome is defined as VZV infection of the head and neck that involves the facial nerve = the seventh cranial nerve (CN VII)<ref>Furuta Y, Aizawa H, Ohtani F, et al. Varicella-zoster virus DNA level and facial paralysis in Ramsay Hunt syndrome. Ann Otol Rhinol Laryngol. 2004 Sep. 113(9):700-5</ref>. Other cranial nerves (CN) might be also involved, including CN VIII, IX, V, and VI (in order of frequency). This infection often causes vesiculation and ulceration of the external ear, as well as ipsilateral facial neuropathy (in CN VII), radiculoneuropathy, or geniculate ganglionopathy. In some cases there are also vesicles on the ipsilateral anterior two thirds of the tongue and soft palate.<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>.
 
VZV infection causes 2 distinct clinical syndromes. Primary infection, also known as varicella or chickenpox, is a common pediatric erythematous disease characterized by a highly contagious generalized vesicular rash. The annual incidence of varicella infection has significantly declined after the introduction of mass vaccination programs in most countries of the world<ref>Kleinschmidt-DeMasters BK, Gilden DH. The expanding spectrum of herpesvirus infections of the nervous system. Brain Pathol. 2001 Oct. 11(4):440-51</ref>.  


After chickenpox, VZV remain latent in neurons of cranial nerve and dorsal root ganglia. Subsequent reactivation of latent VZV can result in localized vesicular rash, known as herpes zoster. VZV infection or reactivation involving the geniculate ganglion of CN VII within the temporal bone is the main pathophysiological mechanism of Ramsay Hunt syndrome
Risk factors for the reactivation VZV include upper respiratory infections, smoking, diabetes, emotional stress, immunosuppresive therapy, cancer and chronic renal failure<ref name=":1" />.  
 
[[Image:PP RHS.png|right|300px]]


== 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 presenting feature is often otalgia: pain deep within the ear. A herpetic rash or blisters, which may be on the skin of the ear canal, auricle or both. Facial palsy develops either concurrently or after several days of the otalgia.
 
Patients usually present with paroxysmal pain deep within the ear. The pain often radiates outward into the pinna of the ear and may be associated with a more constant, diffuse, and dull background pain. The onset of pain usually precedes the rash by several hours and even days.  
 
Classic Ramsay Hunt syndrome can be associated with the following<ref>Ryu EW, Lee HY, Lee SY, Park MS, Yeo SG. Clinical manifestations and prognosis of patients with Ramsay Hunt syndrome. Am J Otolaryngol. 2011 Nov 8</ref>:  
 
Vesicular rash of the ear or mouth (as many as 80% of cases)<br>The rash might precede the onset of facial paresis/palsy (involvement of the seventh cranial nerve [CN VII])<br>Ipsilateral lower motor neuron facial palsy (CN VII)<br>Vertigo and ipsilateral hearing loss (CN VII)<br>Tinnitus<br>Otalgia<br>Headaches<br>Dysarthria<br>Gait ataxia<br>Fever <br>  


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


In most cases, the diagnosis is made purely on clinical findings: the presence of herpetic blisters in and around the ear, accompanied in most cases by pain in and around the ear and an ipsilateral facial palsy.<br>  
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" />. 


On occasions virological studies, both serological and molecular, can be used to confirm the clinical diagnosis.
== Differential Diagnosis  ==


In particular,&nbsp;VZV antigen detection by direct immunofluorescence assay (DFA) is used, which has sensitivity of 90% and specificity close to 99%<ref>Coffin SE, Hodinka RL. Utility of direct immunofluorescence and virus culture for detection of varicella-zoster virus in skin lesions. J Clin Microbiol. 1995 Oct. 33(10):2792-5</ref>.
The following conditions can present in with similar symptoms:  


Structural lesions (such as [[Acoustic Neuroma|Acoustic Neuroma]]) can be ruled out by MRI or CT scan. <br>
*[[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
*[[Acoustic Neuroma|Acoustic Neuroma]] - MRI scan should be used to exclude this
*[[Temporomandibular Disorders]] - not associated with facial palsy
*[[Trigeminal Neuralgia|Trigeminal Neuralgia]] - not associated with facial palsy


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


Corticosteroids and oral acyclovir are commonly used in the treatment of Ramsay Hunt syndrome. In one review, combined therapy using corticosteroids plus intravenous acyclovir did not show benefit over corticosteroids alone in promoting facial nerve recovery after 6 months. The Cochrane Study on antiviral medication in RHS concludes that more studies are needed<ref>Uscategui T, Doree C, Chamberlain IJ, Burton MJ. Antiviral therapy for Ramsay Hunt syndrome (herpes zoster oticus with facial palsy) in adults. Cochrane Database Syst Rev. 2008 Oct 8. CD006851</ref>.<br>  
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" />. 
Vestibular suppressants may be helpful if vestibular symptoms are severe.  
 
As with Bell's palsy, care must be taken to prevent corneal irritation and injury. Referral should be made to an Opthalmologist. Also, see advice page on [[Dry Eye|Dry Eye]] for further information.  
 
Patients with long term facial palsy may also experience dental problems: see advice page on Dental Issues <br>  
 
== 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.
 
Persistent Idiopathic Facial Pain - this is not associated with facial palsy
 
Postherpetic Neuralgia - not associated with facial palsy
 
Temporomandibular Disorders - not associated with facial palsy
 
Trigeminal Neuralgia - not associated with facial palsy<br>  


== Physiotherapy Interventions  ==
== Physiotherapy Interventions  ==
Line 72: Line 50:
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]]. <br>
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.  
 
== 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
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]].  
 
== 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  ==


Line 92: Line 59:
<br>  
<br>  


[[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, Kim Jackson, Poonam Sepat, Evan Thomas, WikiSysop, Claire Knott, Naomi O'Reilly and Wendy Snyders


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


Retrieved from "https://www.physio-pedia.com/index.php?title=Ramsay_Hunt_Syndrome&oldid=328387"